LIQUEN ESTRIADO O NEVIL ?
Nevus Epidérmico Verrugoso Inflamatorio Lineal.
LICHEN STRIATUS OR ILVEN?
Inflammatory Linear Verrucous Epidermal Nevus.
P UBLICADO 2.017 ACTUALIZADO 2023
EDITORIAL ESPAÑOL
==================
Hola amigos de la red DERMAGIC EXPRESS hoy te va hablar de un tema
bastante controversial y discutido hoy día, LA LESIONES
LINEALES en los NIÑOS, específicamente el NEVIL cuyas siglas
significan NEVUS
EPIDERMICO
VERRUGOSO INFLAMATORIO LINEAL, algunos científicos lo denominan LIQUEN ESTRIADO, otros
consideran que son entidades diferentes.
Se trata de una afección dermatológica rara que se presenta
predominantemente en niños de 4 años en adelante tanto en varones como
hembras, pero yo lo he visto aparecer en niños con pocos meses de
nacido. Básicamente la lesión está caracterizada por la
aparición ESPONTANEA de pápulas planas y placas cuyo color puede variar entre
hipocromía (sin color) o rosadas y en algunos casos hiperpigmentadas,
algunas de aspecto verrugoso, las cuales por cambios de temperatura se
"notan" mas.
Estas van extendiéndose en forma "LINEAL" siguiendo una metamera de la piel, denominadas LINEAS DE BLASCHKO, hasta formar una especie de "CAMINO" o trayecto lineal completamente asintomático o levemente pruriginoso,
el cual Puede presentarse en cuello, brazos, piernas, abdomen, área perianal, perivulvar, y en
algunos casos la cara.
La mayoría son totalmente asintomáticas pero en algunos casos el único
síntoma existente es el prurito o comezón y constituyen un verdadero
dolor de cabeza, para los padres de estos niños que manifiestan esta
condición, principalmente por el aspecto estético.
Los padres que van a la consulta creen que se trata de una "CULEBRILLA" por el aspecto lineal de la lesión, y una vez explicado el caso,
quedan TOTALMENTE SORPRENDIDOS de que este tipo de enfermedad exista en la dermatología.
La causa es desconocida, por lo general encuentras como antecedentes,
LESIONES NEVICAS (LUNARES) en los ascendientes del afectado.
Hay varias opciones de tratamiento, que incluyen: laser, esteroides tópicos, tacrolimus, pimecrolimus,
calcipotriol, triamcinolona, tazaroteno, urea y acido salicilico.
EL CLASICO NEVIL: DESAPARECE con un buen tratamiento dermatológico.
Existe una gran controversia con respecto a estas LESIONES LINEALES en
los niños y adultos y voy a tratar de explicártelo lo mejor posible: Las
mas comunes son:
1.) EL LIQUEN ESTRIADO:
=========================
Es exactamente igual en presentación y características al NEVIL, de
hecho hay científicos como lo mencione al principio que consideran ambas
la misma enfermedad con diferente expresión clínica. De hecho tanto el NEVIL como EL LIQUEN ESTRIADO desaparecen con una buena terapia dermatológica, en la mayoría de
los casos.
Mi profesor de histopatología Dr. Marco Tulio Merida Fuentes, gran
Dermatopatologo, quien fuera entrenado por el Dr, Bernard Ackerman en
Nueva York y mi padre dermatólogo decían: NEVIL, Y LIQUEN ESTRIADO, son una misma entidad y el tratamiento
igual: YO OPINO LO MISMO.
LIQUEN ESTRIADO NIÑO DE 5 AÑOS
2.) EL NEVUS EPIDERMICO VERRUGOSO LINEAL: (NO INFLAMATORIO):
=============================================================
Se trata de una lesión NEVICA, tipo lunar en LINEA de aspecto VERRUGOSO, (ver foto) es clínicamente igual al NEVIL, su coloración es pardo
claro o hiperpigmentada. La gran diferencia es que
este NO DESAPARECE con tratamiento tópico convencional y persiste hasta edad
adulta Hay que recurrir a otros métodos como la extirpación
quirúrgica o laser, la mayoría de ellos son congénitos o aparecen
en la infancia.
NEVUS EPIDÉRMICO VERRUGOSOLINEAL DORSO DE MANO
3.) PSORIASIS SEGMENTADA O LINEAL:
==================================
Hay muchos científicos que consideran al NEVIL como una variante
de la PSORIASIS, denominándola PSORIASIS LINEAL,
basados fundamentalmente en los hallazgos HISTOPATOLOGICO
(BIOPSIA) de las lesiones.
Aquí te voy a decir algo basado en MI EXPERIENCIA, EL NEVIL (NEVUS VERRUGOSO INFLAMATORIO LINEAL) cuando desaparece con un buen tratamiento
dermatológico NO VUELVE A APARECER, JAMAS he visto un NEVIL que se curó volver a presentarse.
Por otra parte la PSORIASIS como todos ustedes saben es una
enfermedad multifactorial que da signos y síntomas en otras aéreas
del cuerpo, y que tiende a recaer a menudo, relacionada con
VITILIGO Y DIABETES. La mayoría de los niños con NEVIL que he
tratado NUNCA presentaron SIGNOS O SINTOMAS DE PSORIASIS, ni
recayeron, de modo que...
Con gran respeto a los científicos digo que: EL NEVIL Y LA PSORIASIS LINEAL O SEGMENTADA SON ENTIDADES DIFERENTES, por cierto, NUNCA HE VISTO UN CASO DE "PSORIASIS LINEAL", en
niños ni adultos, quizá exista como entidad, variante de la
PSORIASIS, pero no tiene nada que ver con el clásico NEVIL que se
presenta en niños.
4.) LIQUEN PLANO LINEAL:
===========================
El liquen plano pigmentoso LINEAL es otra entidad que pudiera
confundirse con un NEVIL, pero este por lo general se presenta en
adultos y la coloración del trayecto de las lesiones es de
color VIOLETA o pardo OSCURO, altamente PRURIGINOSO, puede presentarse en cara, (ver EL LIQUEN PLANO aquí), altamente relacionado con el estrés cotidiano. Pero igual que
el NEVIL también desaparece con un buen tratamiento dermatológico.
(Ver foto)
LIQUEN PLANO LINEAL PIGMENTADO DEL CUELLO
5.) NEVUS SEBACEO LINEAL:
=============================
Esta lesión se presenta fundamentalmente en el cuero cabelludo, pero
también se puede presentar ocasionalmente en otras aéreas de la cara
descrito por primera vez por Jadassohn, conocido también como NEVUS SEBACEO DE JADASSOHN, es de aspecto verrugoso color pardo o anaranjado, congénito (está presente al
nacer), y su tratamiento es principalmente quirúrgico. (Ver foto)
NEVUS SEBACEO LINEAL (DE JADASSONHN)
6.) VITILIGO SEGMENTADO:
=========================
Es una variante del clásico vitiligo caracterizado por
maculas ACROMICAS (SIN COLOR) que aparecen en una metamera del cuerpo siguiendo las
líneas de Blaschko, son de mayor tamaño que las lesiones del
clásico NEVIL, puede presentarse en niños como adultos y el
tratamiento en estos casos es mas difícil pues la piel perdió la
coloración a nivel de los MELANOCITOS encargados de producir el pigmento o color de la piel.
CONCLUSION: si tu niño comienza a presentar lesiones dermatológicas en
forma de "LINEA" llévalo a la consulta dermatológica para establecer el
diagnostico adecuado y realizar un buen tratamiento dermatológico.
Si eres adulto igualmente debes consultar a tu dermatólogo.
Espero que esta revisión bibliográfica y fotos te orienten al
respecto.
Saludos a todos.
Dr. José Lapenta
Dr. José M. Lapenta
EDITORIAL ENGLISH
===================
Hello friends of the DERMAGIC EXPRESS network today you I´m going
to talk about a very controversial topic and discussed today, THE LINEAR SKIN LESIONS in CHILDREN, specifically the
ILVEN whose initials stand for INFLAMMATORY LINEAR VERRUCOUS EPIDERMIC NEVUS, some scientists call it LICHEN STRIATUS, and others consider that
they are Different entities.
It is a rare dermatological condition that occurs predominantly in
children before aged 4 years and older in both males and females may
even appear in children with a few months of age. Basically the
lesion is characterized by the SPONTANEOUS appearance of flat
papules and plaques whose color can vary between hypochromic
(without color) or pink and in some cases hyperpigmented, some of
warty appearance, which due to temperature changes are "noticed"
more.
These extend in "LINEAR"
form following the in skin the BLASCHKO LINES, until forming
a kind of "ROAD" or linear
path completely asymptomatic or slightly pruritic, which can present
in
neck, arms, legs, abdomen, Perianal area, perivulvar, and in some
cases the face.
Most are totally asymptomatic but in some cases the only existing
symptom is pruritus or itching and constitutes a
real headache for the parents of these children who manifest this condition,
mainly for the aesthetic aspect.
Parents who go to the clinic believe that it is a "SHINGLES"
because of the linear aspect of the lesion, and once the case is
explained, they are
TOTALLY SURPRISED that this
type of disease exists in dermatology.
The cause is unknown, usually found as antecedents, some types of
"NEVUS" (MOLE) in the ancestors of the affected.
There are several treatment options, including:
laser, topical steroids, tacrolimus, pimecrolimus, calcipotriol,
triamcinolone, tazarotene, retinoic acid, urea and salicylic
acid.
CLASSIC ILVEN:
DISAPPEAR with a good dermatological
treatment.
There is a lot of controversy regarding these LINEAR LESIONS in
children and adults and I will try to explain it to you as best as
possible: The most common are:
1.) THE LICHEN STRIATUS:
=======================
=======================
It is exactly equal in presentation and characteristics to ILVEN, in
fact there are scientists as I mention at the beginning that both
consider the same disease with different clinical expression. In
fact
both ILVEN and LICHEN STRIATUS disappear with good
dermatological therapy, in most cases. (See photo)
My professor of histopathology Marco Tulio Merida Fuentes, a great
Dermatopathologist who was trained by Dr. Bernard Ackerman in New
York and my father dermatologist said:
ILVEN and LICHEN STRIATUS
are the same entity and equal treatment: I OPINION THE SAME.
2.) THE LINEAR VERRUCOUS EPIDERMAL NEVUS:
(NON-INFLAMMATORY):
=================================================================
It is a NEVIC lesion, mole type in VERRUCOUS LINEAR aspect,
(see photo) is clinically equal to ILVEN, its coloration is light
brown or hyperpigmented. The great difference is that this does NOT DISAPPEAR with conventional topical treatment and persists until adulthood.
Other methods such as surgical removal or laser are needed to remove
it; most of them are congenital or appear in childhood.
3.) THE SEGMENTAL PSORIASIS (LINEAR):
====================================
There are many scientists who consider ILVEN as a variant of
PSORIASIS, denominating it LINEAR PSORIASIS, based
fundamentally on the HISTOPATHOLOGICAL (BIOPSY) findings of the
lesions.
Here I am going to tell you something based on
MY EXPERIENCE:
ILVEN (INFLAMMATORY LINEAR VERRUCOUS EPIDERMAL NEVUS) it disappears with a good dermatological treatment and
DOES NOT REPEAT or REPLAPSE,
I have NEVER seen an
ILVEN that was cured to relapse
itself.
On the other hand the PSORIASIS as you all know is a multifactorial
disease that gives signs and symptoms in other areas of the body,
and that tends to relapse often, related to VITILIGO AND
DIABETES.
The majority of the children with ILVEN that I have tried NEVER presented SIGNS OR SYMPTOMS OF PSORIASIS, nor they relapse, so that...
The majority of the children with ILVEN that I have tried NEVER presented SIGNS OR SYMPTOMS OF PSORIASIS, nor they relapse, so that...
With great respect to scientists I say that
ILVEN AND
LINEAR OR SEGMENTAL PSORIASIS ARE DIFFERENT ENTITIES,
by the way, I have NEVER SEEN A CASE OF "LINEAR PSORIASIS", in
children or adults, it may exist as an entity, variant of PSORIASIS,
But it has nothing to do with the classic ILVEN that occurs in
children.
4.) LINEAR LICHEN PLANUS:
=========================
The LINEAR pigmentosus lichen planus is another entity that could be
confused with an ILVEN, but this usually occurs in adults and the
color of the lesion is usually a VIOLET or
BROWN
color,
(View LICHEN PLANUS here) which is highly PRURITIC, may appear on the face, highly related to
everyday stress. But just as ILVEN also disappears with a good
dermatological treatment. (View photo)
5.) SEBACEOUS LINEAR NEVUS:
===========================
This lesion occurs primarily in the scalp, but may also occur
occasionally in other areas of the face first described by
Jadassohn, also known as SEBACEOUS NEVUS OF JADASSOHN, is
congenitally
brown or orange-colored (it is present at birth), and its treatment is primarily surgical.
(View photo)
6.) SEGMENTAL VITILIGO:
=======================
It is a variant of the classic vitiligo characterized by
ACROMIC macules (without COLOR) that appear in the body
following the lines of Blaschko, are of greater size than the
classic ILVEN lesions, can present in children as adults and the
treatment in these cases Is more difficult because the skin lost the
coloration at the level of the MELANOCYTES
responsible for producing the pigment or color of the skin.
CONCLUSION: if your
child begins to present dermatological lesions in the form of
"LINE" take it to the
dermatological consultation to establish the proper diagnosis and to
perform a good dermatological treatment. If you are an adult you
should also consult your dermatologist.
I hope this bibliographic review and photos guide you !
Greetings to all.
Dr. José Lapenta.
Dr. José m. Lapenta.
Dr. José m. Lapenta.
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REFERENCIAS BIBLIOGRAFICAS/ BIBLIOGRAPHICAL REFERENCES
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1.) ILVEN - COMPLETE REMISSION AFTER ADMINISTRATION OF TOPICAL CORTICOSTEROID (CASE REVIEW).
2.) Successful treatment of inflammatory linear verrucous epidermal nevus with tacrolimus and fluocinonide.
3.) Genital/Perigenital Inflammatory Linear Verrucous Epidermal Nevus: A Case Series.
4.) Does inflammatory linear verrucous epidermal nevus represent a segmental type 1/type 2 mosaic of psoriasis?
5.) Immunohistochemical differentiation between inflammatory linear verrucous epidermal nevus (ILVEN) and psoriasis.
6.) Inflammatory linear verrucous epidermal nevus syndrome with its polymorphic presentation - A rare case report.
7.) Vulval and perianal inflammatory linear verrucous epidermal naevus.
8.) Adult onset of inflammatory linear verrucous epidermal nevus.
9.) Inflammatory linear verrucous epidermal nevus (ILVEN).
10.) Histopathologic varieties of epidermal nevus. A study of 160 cases.
11.) Inflammatory linear verrucose epidermal nevus.
12.) Inflammatory linear verrucous epidermal naevus (ILVEN) versus linear psoriasis. A clinical, histological and immunohistochemical study.
13.) Inflammatory linear verrucous epidermal nevus: why a combined laser therapy.14.) Inflammatory linear verrucous epidermal nevus and arthritis: a new association.
15.) [Successful therapy of an ILVEN in a 7-year-old girl with calcipotriol].
16.) Dithranol in the treatment of inflammatory linear verrucous epidermal nevus.
17.) Naevoid Psoriasis and ILVEN: Same Coin, Two Faces?
18.) Carbon dioxide laser treatment of epidermal nevi: response and long-term follow-up.
19.) A case of linear lichen planus pigmentosus.
20.) Lichen planus pigmentosus presenting in zosteriform pattern.
21.) Differential Diagnosis of Linear Eruptions in Children.
22.) Two cases of lichen striatus with prolonged active phase.
23.) Effective topical combination therapy for treatment of lichen striatus in children: a case series and review.
24.) [Lichen striatus with nail abnormality is a self-limiting condition].
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================================================================
1.) ILVEN - COMPLETE REMISSION AFTER ADMINISTRATION OF TOPICAL CORTICOSTEROID (CASE REVIEW).
===============================================================
Georgian Med News. 2017 Feb;(263):10-13.
Wollina U1, Tchernev G1.
Author information
1
Academic Teaching Hospital Dresden-Friedrichstadt, Department of Dermatology and Allergology; Medical Institute of Ministry of Interior (MVR), Department of Dermatology and Dermatologic Surgery, Sofia, Bulgaria.
Abstract
Inflammatory linear verrucous epidermal nevus (ILVEN) is a relatively rare disorder with an onset at early age, consisting of pruritic linear papules and/or plaques and histologic features resembling psoriasis or lichenoid dermatitis. The disease is a version of mosaicism caused by somatic mutations. ILVEN belongs to the heterogeneous group of congenital hamartomas of embryonal ectodermal origin, as a variant of verrucous epidermal nevus, representing approximately 5% of all epidermal nevi, with predominance in females and with general therapeutic resistance. We report on an 18-month-old female patient with ILVEN, who failed to respond to topical tacrolimus, but achieved complete resolution with topical application of momethasone furoate 0.1% under occlusion for 2 weeks, with no signs of recurrence to date. Consequent topical therapy can provide excellent results in young children.
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2.) Successful treatment of inflammatory linear verrucous epidermal nevus with tacrolimus and fluocinonide.
====================================================================
Mutasim DF1.
J Cutan Med Surg. 2006 Jan-Feb;10(1):45-7.
Author information
1
Department of Dermatology, University of Cincinnati College of Medicine, Cincinnati, OH 45267-0592, USA. diya.mutasim@uc.edu
Abstract
BACKGROUND:
Inflammatory linear verrucous epidermal nevus (ILVEN) is a relatively rare disorder that is characterized by an early age at onset; severely pruritic linear papules and plaques; histologic features resembling spongiotic dermatitis, psoriasis, or lichenified dermatitis; and poor response to treatment.
OBJECTIVE:
To report the successful treatment of ILVEN with potent topical steroid and tacrolimus ointments.
METHODS:
An 11-year-old girl presented with a 1-year history of markedly pruritic, progressive linear eruption that extended from the right foot to the right buttock. She had failed treatment with pimecrolimus, calcipotriol, mometasone furoate, triamcinolone, tazarotene, and alpha-hydroxy acid. Histologic examination revealed the findings of spongiotic dermatitis.
RESULTS:
The lesions resolved with fluocinonide ointment and tacrolimus 0.1% ointment.
CONCLUSION:
The combination of two therapeutic agents with different mechanisms of action likely resulted in the successful treatment of this usually resistant condition.
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3.) Genital/Perigenital Inflammatory Linear Verrucous Epidermal Nevus: A Case Series.
====================================================================
Indian J Dermatol. 2015 Nov-Dec;60(6):592-5. doi: 10.4103/0019-5154.169132.
Bandyopadhyay D1, Saha A1.
Author information
1
Department of Dermatology, Venereology and Leprosy, Medical College and Hospitals, Kolkata, West Bengal, India.
Abstract
BACKGROUND:
Inflammatory linear verrucous epidermal nevus (ILVEN) is a distinct variety of keratinocytic epidermal naevus. In contrast to non-inflammatory epidermal naevi, ILVEN are far less common, usually erythematous and intractably pruritic. ILVEN usually appears at birth or early childhood and has a linear distribution following the Blaschko lines. Genital/perigenital involvement is relatively rare.
OBJECTIVES:
To describe the clinical features of 9 children with ILVEN localized to the genital and perigenital areas.
METHOD:
A retrospective study of 9 children with ILVEN presenting to a tertiary care Dermatology Clinic between 2007 and 2014 was undertaken. The clinical and histopathological features were reviewed.
RESULTS:
Nine children (6 females, 3 males) were included in the study based on their characteristic clinicopathological features. The lesions were associated with severe itching in all cases. The mean age at presentation was 4 years (range 1-11 years). Onset of lesions was before 6 months of age in 8 patients. Left sided involvement was twice as common as the right sided one. Male patients had penoscrotal and groin involvement while all the female children had vulvar lesions. None of the children had any extracutaneous abnormalities. The children were treated with topical agents with variable relief or symptoms.
CONCLUSIONS:
The possibility of ILVEN should be considered in every linear genital lesion in children. We have presented the largest series of perigenital ILVEN reported in English literature.
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4.) Does inflammatory linear verrucous epidermal nevus represent a segmental type 1/type 2 mosaic of psoriasis?
====================================================================
Dermatology. 2006;212(2):103-7.
Hofer T1.
Author information
1
Dermatology and Venereology FMH, Winkelriedstrasse 10, CH-5430 Wettingen, Switzerland. thomas.hofer@active.ch
Abstract
BACKGROUND:
A 6-year-old girl with a symmetric linear eruption on both of her legs, clinically and histologically resembling inflammatory linear verrucous epidermal nevus (ILVEN) or linear psoriasis (LP), with concomitant psoriasis of the guttata type and a positive family history of psoriasis is presented. The questions as to whether LP actually exists and ILVEN represents a distinct entity are still under debate.
OBJECTIVE AND METHODS:
The recent literature concerning case reports of ILVEN and LP is reviewed.
RESULTS:
Case reports of ILVEN and LP can be subdivided into four different groups: (1) ILVEN with or without concomitant psoriasis, only in part reacting to antipsoriatic treatment, (2) ILVEN without concomitant psoriasis, (3) LP with concomitant psoriasis vulgaris, with both groups 2 and 3 reacting successfully to antipsoriatic treatment, and (4) LP without concomitant psoriasis vulgaris and with no family history of psoriasis (very rarely reported).
CONCLUSION:
It is hypothesized that inflammatory linear verrucous eruption besides nevoid psoriasis/LP represents a further segmental type 1/type 2 mosaic of psoriasis which, if a (verrucous) epidermal nevus exists, shows a high affinity of occurrence in close context to such a nevus. Heritability is thought to be possible.
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5.) Immunohistochemical differentiation between inflammatory linear verrucous epidermal nevus (ILVEN) and psoriasis.
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Eur J Dermatol. 2004 Jul-Aug;14(4):216-20.
Vissers WH1, Muys L, Erp PE, de Jong EM, van de Kerkhof PC.
Author information
1
Department of Dermatology, University Medical Centre St Radboud, P.O. Box 9101, 6500 HB Nijmegen, The Netherlands. w.vissers@derma.umcn.nl
Abstract
Inflammatory linear verrucous epidermal nevus (ILVEN) is a rare skin disorder with a clinical and histological resemblance to psoriasis. In the past clinical and histological criteria have been defined. However, there remains a discussion as to whether ILVEN is a disease entity distinct from linear psoriasis. Our objective was to compare by quantitative immunohistochemistry the subsets of T-lymphocytes and markers for epidermal growth and keratinisation in biopsies taken from skin lesions of 4 patients with psoriasis and 3 patients with ILVEN: 1. patients with psoriasis (case 1-4) 2. patient with ILVEN cum psoriasis (case 5) 3. patients with ILVEN sine psoriasis (case 6 and 7). Our aim was to delineate ILVEN from psoriasis. Four patients with active psoriasis and three patients with signs and symptoms of ILVEN are described in this case report. Two patients of the ILVEN group had only linear verrucous lesions (ILVEN sine psoriasis), and one patient had linear lesions combined with widespread psoriasis outside the linear verrucous lesion (ILVEN cum psoriasis). The following markers were investigated in skin biopsies taken from the aforementioned patients by quantitative immunohistochemistry: CD2, CD4, CD8, CD25, CD161, CD94, CD45RO, CD45RA, HLA-DR, Keratin-10, Ki-67. In patients with ILVEN (cum and sine psoriasis) the number of Ki-67 positive nuclei, tended to be lower, the number of keratin-10 positive cells and HLA-DR expression higher as compared to psoriasis. In ILVEN sine psoriasis all T-cell subsets and cells expressing NK receptors were reduced as compared to psoriasis, except for CD45RA+ cells, whereas in the patient with ILVEN cum psoriasis the number of these T cell subsets had an intermediary position. In particular the density of CD8+, CD45RO+ and CD2+, CD94 and CD161 showed a marked difference between ILVEN sine psoriasis and psoriasis. In addition to the increased keratin 10 expression in ILVEN sine psoriasis, T cells relevant in the pathogenesis of psoriasis are markedly reduced in ILVEN sine psoriasis as compared to psoriasis. T-cell subsets in ILVEN cum psoriasis had an intermediary position.
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6.) Inflammatory linear verrucous epidermal nevus syndrome with its polymorphic presentation - A rare case report.
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Contemp Clin Dent. 2012 Jan;3(1):119-22. doi: 10.4103/0976-237X.94562.
Kumar CA1, Yeluri G, Raghav N.
Author information
1
Department of Oral Medicine and Radiology, K. D. Dental College and Hospital, Mathura, Uttar Pradesh, India.
Abstract
Epidermal nevi are hamartomatous lesions that are typically present at birth, but can occur anytime during childhood and may rarely appear in adulthood. An estimated one-third of individuals with epidermal nevi have involvement of other organ systems; hence, this condition is considered to be an epidermal nevus syndrome. There are four distinct epidermal nevus syndromes recognizable by the different types of associated epithelial nevi: linear sebaceous nevi, linear nevus comedonicus, linear epidermal nevus, and inflammatory linear verrucous epidermal nevus (ILVEN). Each type may be regarded as a part of a syndrome with other systemic manifestations. We report a rare case of ILVEN syndrome in a 23-year-old female patient with a wide spectrum of mucosal, cutaneous, and skeletal abnormalities, demonstrating the polymorphic presentation of this condition.
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7.) Vulval and perianal inflammatory linear verrucous epidermal naevus.
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Australas J Dermatol. 2009 May;50(2):115-7. doi: 10.1111/j.1440-0960.2009.00518.x.
Le K1, Wong LC, Fischer G.
Author information
1
Department of Dermatology, The Children's Hospital at Westmead, Westmead, New South Wales, Australia. katie.le@unsw.edu.au
Abstract
Inflammatory linear verrucous epidermal naevus (ILVEN) is a rare form of epidermal naevus. It occurs as a linear dermatitic or psoriasiform plaque, with onset usually in the first 5 years of life. Lesions are characteristically intensely itchy. We present a case of ILVEN occurring on the vulva and perianal region of a 6-year-old girl. The lesion was initially thought to be an area of lichenified dermatitis; however, treatment with even super-potent topical corticosteroids did not significantly improve the inflammation. A biopsy was performed and histopathological examination showed characteristic features. ILVEN is frequently refractory to topical treatment and surgical excision of lesions may be an option for relief of symptoms. ILVEN occasionally presents in the inguinogenital region and in this area may, like many vulval naevi, be misdiagnosed as vulvitis, psoriasis, genital warts or sexual abuse.
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8.) Adult onset of inflammatory linear verrucous epidermal nevus.
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J Dermatol. 1999 Sep;26(9):599-602.
Kawaguchi H1, Takeuchi M, Ono H, Nakajima H.
Author information
1
Department of Dermatology, Yokohama City University, School of Medicine, Japan.
Abstract
Adult onset of inflammatory linear verrucous epidermal nevus (ILVEN) is reported in a 44-year-old Japanese man. A mild pruritic eruption appeared one year earlier and extended from the left dorsal foot to the gluteal region. Histologically, acanthosis and papillomatous thickening of epidermis as well as spongiotic edema and exocytosis with lymphocytes and neutrophils were observed. Topical tacalcitol was not effective, but the pruritus as well as the eruption slightly improved with topical corticosteroid and vaseline containing salicylic acid. This adult onset of ILVEN is considered to be a rare case.
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9.) Inflammatory linear verrucous epidermal nevus (ILVEN).
====================================================================
Skoven I.
Abstract
A 12-year-old boy with inflammatory linear verrucous nevus is described. The lesions were pruritic and consisted of small, erythematous, slightly scaling papules coalescing to form linear, lichenified and excoriated plaques. The histological picture was psoriasiform but features of eczema were also seen.
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10.) Histopathologic varieties of epidermal nevus. A study of 160 cases.
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Am J Dermatopathol. 1982 Apr;4(2):161-70.
Su WP.
Abstract
The histopathologic features of 167 biopsy specimens from 160 patients with clinically typical epidermal nevi seen at the Mayo clinic between 1960 and 1978 were reviewed. The most common histopathologic pattern of epidermal nevus was hyperkeratosis, papillomatosis, and acanthosis with elongation of rete ridges. Other histopathologic varieties of epidermal nevus included 1) acrokeratosis verruciformis-like, 2) epidermolytic hyperkeratosis, 3) seborrheic keratosis-like, 4)psoriasiform (inflammatory linear verrucous epidermal nevus), 5) verrucoid, 6) porokeratosis-like, 7) focal acantholytic dyskeratosis, and 8) nevus comedonicus. Correlation of clinical and histopathologic findings is necessary in all lesions suspected of being epidermal nevus.
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11.) Inflammatory linear verrucose epidermal nevus.
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Dermatologica. 1975;150(2):65-9.
Toribio JT, Quicres PA.
Abstract
A typical case of inflammatory linear verrucose epidermal nevus (ILVEN), with quite a psoriasiform histologic pattern, is reported. Intralesionally administered paramethasone acetate caused a temporary suppression of the clinical and histopathologic inflammatory features. The concept of ILVEN is briefly discussed.
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12.) Inflammatory linear verrucous epidermal naevus (ILVEN) versus linear psoriasis. A clinical, histological and immunohistochemical study.
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de Jong E1, Rulo HF, van de Kerkhof PC.
Author information
1
Department of Dermatology, University of Nijmegen, The Netherlands.
Abstract
Inflammatory Linear Verrucous Epidermal Nevus (ILVEN) has been suggested to be a separate disease entity. However, the distinction from linear psoriasis has been discussed in the literature over recent decades. The aim of the present study was to investigate, in addition to the clinical and histological criteria, the immunohistochemical aspects of inflammation, epidermal proliferation and keratinization. From a clinical and histological point of view, ILVEN and psoriasis, according to the established criteria, have been proved to overlap. The immunohistochemical study suggests that the following procedures have an additional diagnostic impact: assessment of elastase-positive cells, assessment of keratin 16 and of keratin 10.
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13.) Inflammatory linear verrucous epidermal nevus: why a combined laser therapy.
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J Cosmet Laser Ther. 2013 Aug;15(4):242-5. doi: 10.3109/14764172.2013.807115. Epub 2013 Jun 21.
Conti R1, Bruscino N, Campolmi P, Bonan P, Cannarozzo G, Moretti S.
Author information
Abstract
Inflammatory linear verrucous epidermal nevus (ILVEN) is a benign cutaneous hamartoma, and more precisely an uncommon variant of the verrucous epidermal nevus. In our case report we describe an ILVEN female patient, resistant to previous treatments but responsive to a combined laser therapy, 10,600-nm CO2 laser and Fractional CO2 laser, with good and longstanding results. A complete resolution of the lesion was observed at the 9-month follow-up. The application of lasers has been reported in literature over recent years for resolving ILVEN lesions. Based on our clinical experience, 10,600-nm CO2 pulsed laser therapy seems to be the best treatment and Fractional CO2 laser treatment can be regarded as a very promising technique to combine with CO2 laser for reducing pigment modifications and endowing a more youthful appearance to the treated areas.
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14.) Inflammatory linear verrucous epidermal nevus and arthritis: a new association.
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J Pediatr. 2001 Apr;138(4):602-4.
Al-Enezi S1, Huber AM, Krafchik BR, Laxer RM.
Author information
1
Division of Rheumatology, Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.
Abstract
Inflammatory linear verrucous epidermal nevus (ILVEN) is a rare, chronic skin condition that begins in early childhood. We present two children with ILVEN and arthritis, a previously undescribed association. We discuss the relevance of this association and suggest appropriate management for this arthritis.
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15.) [Successful therapy of an ILVEN in a 7-year-old girl with calcipotriol].
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Hautarzt. 1999 Nov;50(11):812-4.
[Article in German]
Böhm I1, Bieber T, Bauer R.
Author information
1
Klinik und Poliklinik für Dermatologie der Rheinischen Friedrich-Wilhelms Universität Bonn.
Abstract
A 7-year-old otherwise healthy girl presented with a 2-year history of an ILVEN (inflammatory linear verrucous epidermal nevus) located on the inner part of her right upper arm. The diagnosis was histologically confirmed. Different conservative therapeutic strategies with corticosteroids, antibiotics and antimycotics produced little or no improvement. Because of encouraging reports describing the successful use of 0.005% calcipotriol ointment in patients with ILVEN, we treated our patient with this regimen. After 4 weeks we could recognize a impressive improvement and after 8 weeks the ILVEN had nearly completely disappeared. 25 weeks after withdrawal of calcipotriol, no relapse had occurred. The dramatic response to calcipotriol suggests some pathological links between ILVEN and psoriasis.
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16.) Dithranol in the treatment of inflammatory linear verrucous epidermal nevus.
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Acta Derm Venereol. 1989;69(1):77-80.
de Mare S1, van de Kerkhof PC, Happle R.
Author information
1
Department of Dermatology, University of Nijmegen, The Netherlands.
Abstract
A case of inflammatory linear verrucous epidermal nevus (ILVEN) is reported. Short contact treatment with dithranol resulted in complete relief from itching and a remarkable clearing of all linear lesions except from a small verrucous band on the shin. In patients with ILVEN it is advisable to try dithranol therapy before carrying out surgical procedures such as excision, cryotherapy, electrocautery. The prompt response to dithranol is best explained by the assumption that most of the lesions in this case of ILVEN represented true linear psoriasis.
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17.) Naevoid Psoriasis and ILVEN: Same Coin, Two Faces?
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Indian J Dermatol. 2012 Nov;57(6):489-91. doi: 10.4103/0019-5154.103072.
Sengupta S1, Das JK, Gangopadhyay A.
Author information
1
Department of Dermatology, KPC Medical College and Hospital, Kolkata, India.
Abstract
The true existence of naevoid psoriasis and inflammatory linear verrucous naevus as distinct entities has been a debatable issue. Each has been opined to be a variant of the other. Considerable clinical and histological resemblance is seen between the two conditions. We describe three cases which attempt to throw more light on this issue.
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18.) Carbon dioxide laser treatment of epidermal nevi: response and long-term follow-up.
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Alonso-Castro L1, Boixeda P, Reig I, de Daniel-Rodríguez C, Fleta-Asín B, Jaén-Olasolo P.
Author information
1
Servicio de Dermatología, Hospital Universitario Ramón y Cajal, Madrid, Spain. letticiaac@gmail.com
Abstract
BACKGROUND AND OBJECTIVES:
Epidermal nevi, which are benign skin growths, have been treated using a range of approaches, with varying results. Topical treatments are ineffective and, while surgical excision is a more definitive treatment, it causes scar formation. In recent decades, epidermal nevi have been treated with various types of laser therapy. We describe our experience with the use of carbon dioxide (CO(2)) laser therapy to treat epidermal nevi and inflammatory linear verrucous epidermal nevi (ILVEN).
PATIENTS AND METHODS:
Twenty patients (15 with epidermal nevi and 5 with ILVEN) underwent CO(2) laser treatment at our hospital between 2002 and 2010.
RESULTS:
Response was good (>50% reduction in lesion size) in 50% of cases and excellent (>75% reduction) in 30%. A greater resistance to treatment was observed in patients with ILVEN (only 40% had a good response). Long-term follow-up (at least 18 months) showed a recurrence rate of 30%. The side effects were hypopigmentation (25% of patients) and scarring (20%).
CONCLUSIONS:
We consider CO(2) laser therapy to be the treatment of choice for epidermal nevi as it is well tolerated and has proven to be safe and effective in the long term. While the response in patients with ILVEN was limited, CO(2) laser therapy might be a good option for selected cases or for palliative treatment since no other treatments have yet proven effective in this setting
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19.) A case of linear lichen planus pigmentosus.
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Ann Dermatol. 2010 Aug;22(3):323-5. doi: 10.5021/ad.2010.22.3.323. Epub 2010 Aug 5.
Seo JK1, Lee HJ, Lee D, Choi JH, Sung HS.
Author information
1
Department of Dermatology, Busan Paik Hospital, College of Medicine, Inje University, Busan, Korea.
Abstract
Lichen planus pigmentosus (LPP) is chronic pigmentary disorder that shows diffuse or reticulated hyperpigmented, dark brown macules on the sun-exposed areas such as the face, neck and other flexural folds. Clinically, it is different from classical lichen planus because LPP has a longer clinical course and it manifests with dark brown macules. In case of LPP, involvement of the scalp, nail or mucosal area is rare. The histopathological findings of the lesions show an atrophic epidermis, the presence of melanophages and a vacuolar alteration of the basal cell layer with a sparse lymphohistiocytic lichenoid infiltration. Although there have been a few reports of LPP, there have only 3 cases of linear LPP along the lines of Blaschko in the Korean dermatologic literature. Our patient had lesions on the neck and chin with a linear pattern. In this report, we describe a very rare case of LPP with a linear distribution related to Blaschko's lines on the neck and chin areas.
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20.) Lichen planus pigmentosus presenting in zosteriform pattern.
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Cho S1, Whang KK.
Author information
1
Department of Dermatology, College of Medicine, Ewha Womans University, Seoul, Korea.
Abstract
Lichen planus pigmentosus (LPP) has thus far been described as a condition of unknown etiology which clinically differs from the classical lichen planus (LP) by exhibiting dark brown macules and/or papules mostly in exposed areas and flexural folds and a longer clinical course without pruritus or scalp, nail or mucosal involvement. Histopathologically, LPP shows the typical changes seen in LP, but with thinning of epidermis. We report a case of LPP that developed in a unilateral, zosteriform pattern on the left flank of a 49-year-old man. This case seems to lie in the middle of the spectrum between classical LP and ashy dermatosis, and, to the best of our knowledge, is the first report of LPP presenting in the zosteriform pattern.
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21.) Differential Diagnosis of Linear Eruptions in Children.
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Kruse LL.
Abstract
A 3-year-old girl presented with a linear eruption on her leg for 2 months. She was otherwise healthy and well-appearing. Physical examination showed many small, erythematous, flat-topped papules coalescing into a linear erythematous plaque. At a follow-up visit 9 months later, the eruption had resolved, leaving postinflammatory hypopigmentation.When approaching a cutaneous eruption, appreciating the pattern of the lesions can be instrumental to arriving at the correct diagnosis. For this patient with the acute onset of a plaque on the leg, the differential diagnosis is narrowed by the linear distribution of the skin lesions. The differential diagnosis of linear eruptions in children includes lichen striatus, linear lichen planus, linear psoriasis, inflammatory linear verrucous epidermal nevus, incontinentia pigmenti, phytophotodermatitis, and allergic contact dermatitis. Of note, many of these conditions manifest in a linear manner as a result of cutaneous mosaicism, whereas others are caused by external agents contacting the skin.
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22.) Two cases of lichen striatus with prolonged active phase.
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Pediatr Dermatol. 2014 Mar-Apr;31(2):e67-8. doi: 10.1111/pde.12261. Epub 2014 Jan 24.
Feely MA1, Silverberg NB.
Author information
1
Beth Israel Medical Center, New York, NY; St. Luke's-Roosevelt Hospital Center, New York, NY.
Abstract
Lichen striatus is a localized, eczematous disorder distributed along the lines of Blaschko, primarily affecting children. In the literature, lesions have been described as having an active phase of inflamed lesions for 6 to 12 months followed by flattening and persistent pigmentary alteration. We describe two girls who had prolonged active-phase lesions for 2.5 and 3.5 years, respectively. Practitioners should be aware that lesions of lichen striatus may have a prolonged active phase.
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23.) Effective topical combination therapy for treatment of lichen striatus in children: a case series and review.
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Youssef SM1, Teng JM.
Author information
1
Department of Dermatology, University of Wisconsin-Madison, Madison, WI 53715, USA.
Abstract
Lichen striatus (LS) is an uncommon linear dermatosis that is primarily seen in children from 4 months to 15 years of age. While some of these eruptions are asymptomatic, others can be quite pruritic. In darker-skinned individuals, post-inflammatory hypopigmentation can be significant and may provide a cause for concern for the patients and/or their parents. In our case series of 4 patients, we observed rapid resolution of LS by combining a topical retinoid with a topical steroid. To our knowledge, this is the first report of successful treatment with this kind of combination therapy in the English literature. The patients not only achieved satisfying cosmesis, but also complete resolution of their pruritus. The most common side effect of topical tazarotene is localized irritation at treatment sites, but the patients in this particular series tolerated the treatment well.
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24.) [Lichen striatus with nail abnormality is a self-limiting condition].
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Ugeskr Laeger. 2012 Mar 5;174(10):652-3.
[Article in Danish]
Sandreva T1, Bygum A.
Author information
1
Det Sundhedsvidenskabelige Fakultet, Syddansk Universitet, Campusvej 55, 5230 Odense, Denmark. tsand07@student.sdu.dk
Abstract
A six year-old boy was referred with an asymptomatic linear rash on his left arm and a nail involvement of a fingernail on his left hand. Multiple small erythematous papules coalescing into a linear band extending to the thumb with nail abnormality were noted on the left arm. A diagnosis of lichen striatus was established clinically. The condition has spontaneous remission, although the course of the disease is prolonged when nail involvement exists. The condition is benign and there is no need to perform biopsy.
REFERENCIAS BIBLIOGRAFICAS/ BIBLIOGRAPHICAL REFERENCES
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1.) ILVEN - COMPLETE REMISSION AFTER ADMINISTRATION OF TOPICAL CORTICOSTEROID (CASE REVIEW).
2.) Successful treatment of inflammatory linear verrucous epidermal nevus with tacrolimus and fluocinonide.
3.) Genital/Perigenital Inflammatory Linear Verrucous Epidermal Nevus: A Case Series.
4.) Does inflammatory linear verrucous epidermal nevus represent a segmental type 1/type 2 mosaic of psoriasis?
5.) Immunohistochemical differentiation between inflammatory linear verrucous epidermal nevus (ILVEN) and psoriasis.
6.) Inflammatory linear verrucous epidermal nevus syndrome with its polymorphic presentation - A rare case report.
7.) Vulval and perianal inflammatory linear verrucous epidermal naevus.
8.) Adult onset of inflammatory linear verrucous epidermal nevus.
9.) Inflammatory linear verrucous epidermal nevus (ILVEN).
10.) Histopathologic varieties of epidermal nevus. A study of 160 cases.
11.) Inflammatory linear verrucose epidermal nevus.
12.) Inflammatory linear verrucous epidermal naevus (ILVEN) versus linear psoriasis. A clinical, histological and immunohistochemical study.
13.) Inflammatory linear verrucous epidermal nevus: why a combined laser therapy.14.) Inflammatory linear verrucous epidermal nevus and arthritis: a new association.
15.) [Successful therapy of an ILVEN in a 7-year-old girl with calcipotriol].
16.) Dithranol in the treatment of inflammatory linear verrucous epidermal nevus.
17.) Naevoid Psoriasis and ILVEN: Same Coin, Two Faces?
18.) Carbon dioxide laser treatment of epidermal nevi: response and long-term follow-up.
19.) A case of linear lichen planus pigmentosus.
20.) Lichen planus pigmentosus presenting in zosteriform pattern.
21.) Differential Diagnosis of Linear Eruptions in Children.
22.) Two cases of lichen striatus with prolonged active phase.
23.) Effective topical combination therapy for treatment of lichen striatus in children: a case series and review.
24.) [Lichen striatus with nail abnormality is a self-limiting condition].
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================================================================
1.) ILVEN - COMPLETE REMISSION AFTER ADMINISTRATION OF TOPICAL CORTICOSTEROID (CASE REVIEW).
===============================================================
Georgian Med News. 2017 Feb;(263):10-13.
Wollina U1, Tchernev G1.
Author information
1
Academic Teaching Hospital Dresden-Friedrichstadt, Department of Dermatology and Allergology; Medical Institute of Ministry of Interior (MVR), Department of Dermatology and Dermatologic Surgery, Sofia, Bulgaria.
Abstract
Inflammatory linear verrucous epidermal nevus (ILVEN) is a relatively rare disorder with an onset at early age, consisting of pruritic linear papules and/or plaques and histologic features resembling psoriasis or lichenoid dermatitis. The disease is a version of mosaicism caused by somatic mutations. ILVEN belongs to the heterogeneous group of congenital hamartomas of embryonal ectodermal origin, as a variant of verrucous epidermal nevus, representing approximately 5% of all epidermal nevi, with predominance in females and with general therapeutic resistance. We report on an 18-month-old female patient with ILVEN, who failed to respond to topical tacrolimus, but achieved complete resolution with topical application of momethasone furoate 0.1% under occlusion for 2 weeks, with no signs of recurrence to date. Consequent topical therapy can provide excellent results in young children.
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2.) Successful treatment of inflammatory linear verrucous epidermal nevus with tacrolimus and fluocinonide.
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Mutasim DF1.
J Cutan Med Surg. 2006 Jan-Feb;10(1):45-7.
Author information
1
Department of Dermatology, University of Cincinnati College of Medicine, Cincinnati, OH 45267-0592, USA. diya.mutasim@uc.edu
Abstract
BACKGROUND:
Inflammatory linear verrucous epidermal nevus (ILVEN) is a relatively rare disorder that is characterized by an early age at onset; severely pruritic linear papules and plaques; histologic features resembling spongiotic dermatitis, psoriasis, or lichenified dermatitis; and poor response to treatment.
OBJECTIVE:
To report the successful treatment of ILVEN with potent topical steroid and tacrolimus ointments.
METHODS:
An 11-year-old girl presented with a 1-year history of markedly pruritic, progressive linear eruption that extended from the right foot to the right buttock. She had failed treatment with pimecrolimus, calcipotriol, mometasone furoate, triamcinolone, tazarotene, and alpha-hydroxy acid. Histologic examination revealed the findings of spongiotic dermatitis.
RESULTS:
The lesions resolved with fluocinonide ointment and tacrolimus 0.1% ointment.
CONCLUSION:
The combination of two therapeutic agents with different mechanisms of action likely resulted in the successful treatment of this usually resistant condition.
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3.) Genital/Perigenital Inflammatory Linear Verrucous Epidermal Nevus: A Case Series.
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Indian J Dermatol. 2015 Nov-Dec;60(6):592-5. doi: 10.4103/0019-5154.169132.
Bandyopadhyay D1, Saha A1.
Author information
1
Department of Dermatology, Venereology and Leprosy, Medical College and Hospitals, Kolkata, West Bengal, India.
Abstract
BACKGROUND:
Inflammatory linear verrucous epidermal nevus (ILVEN) is a distinct variety of keratinocytic epidermal naevus. In contrast to non-inflammatory epidermal naevi, ILVEN are far less common, usually erythematous and intractably pruritic. ILVEN usually appears at birth or early childhood and has a linear distribution following the Blaschko lines. Genital/perigenital involvement is relatively rare.
OBJECTIVES:
To describe the clinical features of 9 children with ILVEN localized to the genital and perigenital areas.
METHOD:
A retrospective study of 9 children with ILVEN presenting to a tertiary care Dermatology Clinic between 2007 and 2014 was undertaken. The clinical and histopathological features were reviewed.
RESULTS:
Nine children (6 females, 3 males) were included in the study based on their characteristic clinicopathological features. The lesions were associated with severe itching in all cases. The mean age at presentation was 4 years (range 1-11 years). Onset of lesions was before 6 months of age in 8 patients. Left sided involvement was twice as common as the right sided one. Male patients had penoscrotal and groin involvement while all the female children had vulvar lesions. None of the children had any extracutaneous abnormalities. The children were treated with topical agents with variable relief or symptoms.
CONCLUSIONS:
The possibility of ILVEN should be considered in every linear genital lesion in children. We have presented the largest series of perigenital ILVEN reported in English literature.
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4.) Does inflammatory linear verrucous epidermal nevus represent a segmental type 1/type 2 mosaic of psoriasis?
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Dermatology. 2006;212(2):103-7.
Hofer T1.
Author information
1
Dermatology and Venereology FMH, Winkelriedstrasse 10, CH-5430 Wettingen, Switzerland. thomas.hofer@active.ch
Abstract
BACKGROUND:
A 6-year-old girl with a symmetric linear eruption on both of her legs, clinically and histologically resembling inflammatory linear verrucous epidermal nevus (ILVEN) or linear psoriasis (LP), with concomitant psoriasis of the guttata type and a positive family history of psoriasis is presented. The questions as to whether LP actually exists and ILVEN represents a distinct entity are still under debate.
OBJECTIVE AND METHODS:
The recent literature concerning case reports of ILVEN and LP is reviewed.
RESULTS:
Case reports of ILVEN and LP can be subdivided into four different groups: (1) ILVEN with or without concomitant psoriasis, only in part reacting to antipsoriatic treatment, (2) ILVEN without concomitant psoriasis, (3) LP with concomitant psoriasis vulgaris, with both groups 2 and 3 reacting successfully to antipsoriatic treatment, and (4) LP without concomitant psoriasis vulgaris and with no family history of psoriasis (very rarely reported).
CONCLUSION:
It is hypothesized that inflammatory linear verrucous eruption besides nevoid psoriasis/LP represents a further segmental type 1/type 2 mosaic of psoriasis which, if a (verrucous) epidermal nevus exists, shows a high affinity of occurrence in close context to such a nevus. Heritability is thought to be possible.
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5.) Immunohistochemical differentiation between inflammatory linear verrucous epidermal nevus (ILVEN) and psoriasis.
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Eur J Dermatol. 2004 Jul-Aug;14(4):216-20.
Vissers WH1, Muys L, Erp PE, de Jong EM, van de Kerkhof PC.
Author information
1
Department of Dermatology, University Medical Centre St Radboud, P.O. Box 9101, 6500 HB Nijmegen, The Netherlands. w.vissers@derma.umcn.nl
Abstract
Inflammatory linear verrucous epidermal nevus (ILVEN) is a rare skin disorder with a clinical and histological resemblance to psoriasis. In the past clinical and histological criteria have been defined. However, there remains a discussion as to whether ILVEN is a disease entity distinct from linear psoriasis. Our objective was to compare by quantitative immunohistochemistry the subsets of T-lymphocytes and markers for epidermal growth and keratinisation in biopsies taken from skin lesions of 4 patients with psoriasis and 3 patients with ILVEN: 1. patients with psoriasis (case 1-4) 2. patient with ILVEN cum psoriasis (case 5) 3. patients with ILVEN sine psoriasis (case 6 and 7). Our aim was to delineate ILVEN from psoriasis. Four patients with active psoriasis and three patients with signs and symptoms of ILVEN are described in this case report. Two patients of the ILVEN group had only linear verrucous lesions (ILVEN sine psoriasis), and one patient had linear lesions combined with widespread psoriasis outside the linear verrucous lesion (ILVEN cum psoriasis). The following markers were investigated in skin biopsies taken from the aforementioned patients by quantitative immunohistochemistry: CD2, CD4, CD8, CD25, CD161, CD94, CD45RO, CD45RA, HLA-DR, Keratin-10, Ki-67. In patients with ILVEN (cum and sine psoriasis) the number of Ki-67 positive nuclei, tended to be lower, the number of keratin-10 positive cells and HLA-DR expression higher as compared to psoriasis. In ILVEN sine psoriasis all T-cell subsets and cells expressing NK receptors were reduced as compared to psoriasis, except for CD45RA+ cells, whereas in the patient with ILVEN cum psoriasis the number of these T cell subsets had an intermediary position. In particular the density of CD8+, CD45RO+ and CD2+, CD94 and CD161 showed a marked difference between ILVEN sine psoriasis and psoriasis. In addition to the increased keratin 10 expression in ILVEN sine psoriasis, T cells relevant in the pathogenesis of psoriasis are markedly reduced in ILVEN sine psoriasis as compared to psoriasis. T-cell subsets in ILVEN cum psoriasis had an intermediary position.
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6.) Inflammatory linear verrucous epidermal nevus syndrome with its polymorphic presentation - A rare case report.
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Contemp Clin Dent. 2012 Jan;3(1):119-22. doi: 10.4103/0976-237X.94562.
Kumar CA1, Yeluri G, Raghav N.
Author information
1
Department of Oral Medicine and Radiology, K. D. Dental College and Hospital, Mathura, Uttar Pradesh, India.
Abstract
Epidermal nevi are hamartomatous lesions that are typically present at birth, but can occur anytime during childhood and may rarely appear in adulthood. An estimated one-third of individuals with epidermal nevi have involvement of other organ systems; hence, this condition is considered to be an epidermal nevus syndrome. There are four distinct epidermal nevus syndromes recognizable by the different types of associated epithelial nevi: linear sebaceous nevi, linear nevus comedonicus, linear epidermal nevus, and inflammatory linear verrucous epidermal nevus (ILVEN). Each type may be regarded as a part of a syndrome with other systemic manifestations. We report a rare case of ILVEN syndrome in a 23-year-old female patient with a wide spectrum of mucosal, cutaneous, and skeletal abnormalities, demonstrating the polymorphic presentation of this condition.
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7.) Vulval and perianal inflammatory linear verrucous epidermal naevus.
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Australas J Dermatol. 2009 May;50(2):115-7. doi: 10.1111/j.1440-0960.2009.00518.x.
Le K1, Wong LC, Fischer G.
Author information
1
Department of Dermatology, The Children's Hospital at Westmead, Westmead, New South Wales, Australia. katie.le@unsw.edu.au
Abstract
Inflammatory linear verrucous epidermal naevus (ILVEN) is a rare form of epidermal naevus. It occurs as a linear dermatitic or psoriasiform plaque, with onset usually in the first 5 years of life. Lesions are characteristically intensely itchy. We present a case of ILVEN occurring on the vulva and perianal region of a 6-year-old girl. The lesion was initially thought to be an area of lichenified dermatitis; however, treatment with even super-potent topical corticosteroids did not significantly improve the inflammation. A biopsy was performed and histopathological examination showed characteristic features. ILVEN is frequently refractory to topical treatment and surgical excision of lesions may be an option for relief of symptoms. ILVEN occasionally presents in the inguinogenital region and in this area may, like many vulval naevi, be misdiagnosed as vulvitis, psoriasis, genital warts or sexual abuse.
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8.) Adult onset of inflammatory linear verrucous epidermal nevus.
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J Dermatol. 1999 Sep;26(9):599-602.
Kawaguchi H1, Takeuchi M, Ono H, Nakajima H.
Author information
1
Department of Dermatology, Yokohama City University, School of Medicine, Japan.
Abstract
Adult onset of inflammatory linear verrucous epidermal nevus (ILVEN) is reported in a 44-year-old Japanese man. A mild pruritic eruption appeared one year earlier and extended from the left dorsal foot to the gluteal region. Histologically, acanthosis and papillomatous thickening of epidermis as well as spongiotic edema and exocytosis with lymphocytes and neutrophils were observed. Topical tacalcitol was not effective, but the pruritus as well as the eruption slightly improved with topical corticosteroid and vaseline containing salicylic acid. This adult onset of ILVEN is considered to be a rare case.
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9.) Inflammatory linear verrucous epidermal nevus (ILVEN).
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Skoven I.
Abstract
A 12-year-old boy with inflammatory linear verrucous nevus is described. The lesions were pruritic and consisted of small, erythematous, slightly scaling papules coalescing to form linear, lichenified and excoriated plaques. The histological picture was psoriasiform but features of eczema were also seen.
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10.) Histopathologic varieties of epidermal nevus. A study of 160 cases.
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Am J Dermatopathol. 1982 Apr;4(2):161-70.
Su WP.
Abstract
The histopathologic features of 167 biopsy specimens from 160 patients with clinically typical epidermal nevi seen at the Mayo clinic between 1960 and 1978 were reviewed. The most common histopathologic pattern of epidermal nevus was hyperkeratosis, papillomatosis, and acanthosis with elongation of rete ridges. Other histopathologic varieties of epidermal nevus included 1) acrokeratosis verruciformis-like, 2) epidermolytic hyperkeratosis, 3) seborrheic keratosis-like, 4)psoriasiform (inflammatory linear verrucous epidermal nevus), 5) verrucoid, 6) porokeratosis-like, 7) focal acantholytic dyskeratosis, and 8) nevus comedonicus. Correlation of clinical and histopathologic findings is necessary in all lesions suspected of being epidermal nevus.
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11.) Inflammatory linear verrucose epidermal nevus.
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Dermatologica. 1975;150(2):65-9.
Toribio JT, Quicres PA.
Abstract
A typical case of inflammatory linear verrucose epidermal nevus (ILVEN), with quite a psoriasiform histologic pattern, is reported. Intralesionally administered paramethasone acetate caused a temporary suppression of the clinical and histopathologic inflammatory features. The concept of ILVEN is briefly discussed.
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12.) Inflammatory linear verrucous epidermal naevus (ILVEN) versus linear psoriasis. A clinical, histological and immunohistochemical study.
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de Jong E1, Rulo HF, van de Kerkhof PC.
Author information
1
Department of Dermatology, University of Nijmegen, The Netherlands.
Abstract
Inflammatory Linear Verrucous Epidermal Nevus (ILVEN) has been suggested to be a separate disease entity. However, the distinction from linear psoriasis has been discussed in the literature over recent decades. The aim of the present study was to investigate, in addition to the clinical and histological criteria, the immunohistochemical aspects of inflammation, epidermal proliferation and keratinization. From a clinical and histological point of view, ILVEN and psoriasis, according to the established criteria, have been proved to overlap. The immunohistochemical study suggests that the following procedures have an additional diagnostic impact: assessment of elastase-positive cells, assessment of keratin 16 and of keratin 10.
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13.) Inflammatory linear verrucous epidermal nevus: why a combined laser therapy.
====================================================================
J Cosmet Laser Ther. 2013 Aug;15(4):242-5. doi: 10.3109/14764172.2013.807115. Epub 2013 Jun 21.
Conti R1, Bruscino N, Campolmi P, Bonan P, Cannarozzo G, Moretti S.
Author information
Abstract
Inflammatory linear verrucous epidermal nevus (ILVEN) is a benign cutaneous hamartoma, and more precisely an uncommon variant of the verrucous epidermal nevus. In our case report we describe an ILVEN female patient, resistant to previous treatments but responsive to a combined laser therapy, 10,600-nm CO2 laser and Fractional CO2 laser, with good and longstanding results. A complete resolution of the lesion was observed at the 9-month follow-up. The application of lasers has been reported in literature over recent years for resolving ILVEN lesions. Based on our clinical experience, 10,600-nm CO2 pulsed laser therapy seems to be the best treatment and Fractional CO2 laser treatment can be regarded as a very promising technique to combine with CO2 laser for reducing pigment modifications and endowing a more youthful appearance to the treated areas.
====================================================================
14.) Inflammatory linear verrucous epidermal nevus and arthritis: a new association.
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J Pediatr. 2001 Apr;138(4):602-4.
Al-Enezi S1, Huber AM, Krafchik BR, Laxer RM.
Author information
1
Division of Rheumatology, Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.
Abstract
Inflammatory linear verrucous epidermal nevus (ILVEN) is a rare, chronic skin condition that begins in early childhood. We present two children with ILVEN and arthritis, a previously undescribed association. We discuss the relevance of this association and suggest appropriate management for this arthritis.
====================================================================
15.) [Successful therapy of an ILVEN in a 7-year-old girl with calcipotriol].
====================================================================
Hautarzt. 1999 Nov;50(11):812-4.
[Article in German]
Böhm I1, Bieber T, Bauer R.
Author information
1
Klinik und Poliklinik für Dermatologie der Rheinischen Friedrich-Wilhelms Universität Bonn.
Abstract
A 7-year-old otherwise healthy girl presented with a 2-year history of an ILVEN (inflammatory linear verrucous epidermal nevus) located on the inner part of her right upper arm. The diagnosis was histologically confirmed. Different conservative therapeutic strategies with corticosteroids, antibiotics and antimycotics produced little or no improvement. Because of encouraging reports describing the successful use of 0.005% calcipotriol ointment in patients with ILVEN, we treated our patient with this regimen. After 4 weeks we could recognize a impressive improvement and after 8 weeks the ILVEN had nearly completely disappeared. 25 weeks after withdrawal of calcipotriol, no relapse had occurred. The dramatic response to calcipotriol suggests some pathological links between ILVEN and psoriasis.
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16.) Dithranol in the treatment of inflammatory linear verrucous epidermal nevus.
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Acta Derm Venereol. 1989;69(1):77-80.
de Mare S1, van de Kerkhof PC, Happle R.
Author information
1
Department of Dermatology, University of Nijmegen, The Netherlands.
Abstract
A case of inflammatory linear verrucous epidermal nevus (ILVEN) is reported. Short contact treatment with dithranol resulted in complete relief from itching and a remarkable clearing of all linear lesions except from a small verrucous band on the shin. In patients with ILVEN it is advisable to try dithranol therapy before carrying out surgical procedures such as excision, cryotherapy, electrocautery. The prompt response to dithranol is best explained by the assumption that most of the lesions in this case of ILVEN represented true linear psoriasis.
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17.) Naevoid Psoriasis and ILVEN: Same Coin, Two Faces?
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Indian J Dermatol. 2012 Nov;57(6):489-91. doi: 10.4103/0019-5154.103072.
Sengupta S1, Das JK, Gangopadhyay A.
Author information
1
Department of Dermatology, KPC Medical College and Hospital, Kolkata, India.
Abstract
The true existence of naevoid psoriasis and inflammatory linear verrucous naevus as distinct entities has been a debatable issue. Each has been opined to be a variant of the other. Considerable clinical and histological resemblance is seen between the two conditions. We describe three cases which attempt to throw more light on this issue.
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18.) Carbon dioxide laser treatment of epidermal nevi: response and long-term follow-up.
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Alonso-Castro L1, Boixeda P, Reig I, de Daniel-Rodríguez C, Fleta-Asín B, Jaén-Olasolo P.
Author information
1
Servicio de Dermatología, Hospital Universitario Ramón y Cajal, Madrid, Spain. letticiaac@gmail.com
Abstract
BACKGROUND AND OBJECTIVES:
Epidermal nevi, which are benign skin growths, have been treated using a range of approaches, with varying results. Topical treatments are ineffective and, while surgical excision is a more definitive treatment, it causes scar formation. In recent decades, epidermal nevi have been treated with various types of laser therapy. We describe our experience with the use of carbon dioxide (CO(2)) laser therapy to treat epidermal nevi and inflammatory linear verrucous epidermal nevi (ILVEN).
PATIENTS AND METHODS:
Twenty patients (15 with epidermal nevi and 5 with ILVEN) underwent CO(2) laser treatment at our hospital between 2002 and 2010.
RESULTS:
Response was good (>50% reduction in lesion size) in 50% of cases and excellent (>75% reduction) in 30%. A greater resistance to treatment was observed in patients with ILVEN (only 40% had a good response). Long-term follow-up (at least 18 months) showed a recurrence rate of 30%. The side effects were hypopigmentation (25% of patients) and scarring (20%).
CONCLUSIONS:
We consider CO(2) laser therapy to be the treatment of choice for epidermal nevi as it is well tolerated and has proven to be safe and effective in the long term. While the response in patients with ILVEN was limited, CO(2) laser therapy might be a good option for selected cases or for palliative treatment since no other treatments have yet proven effective in this setting
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19.) A case of linear lichen planus pigmentosus.
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Ann Dermatol. 2010 Aug;22(3):323-5. doi: 10.5021/ad.2010.22.3.323. Epub 2010 Aug 5.
Seo JK1, Lee HJ, Lee D, Choi JH, Sung HS.
Author information
1
Department of Dermatology, Busan Paik Hospital, College of Medicine, Inje University, Busan, Korea.
Abstract
Lichen planus pigmentosus (LPP) is chronic pigmentary disorder that shows diffuse or reticulated hyperpigmented, dark brown macules on the sun-exposed areas such as the face, neck and other flexural folds. Clinically, it is different from classical lichen planus because LPP has a longer clinical course and it manifests with dark brown macules. In case of LPP, involvement of the scalp, nail or mucosal area is rare. The histopathological findings of the lesions show an atrophic epidermis, the presence of melanophages and a vacuolar alteration of the basal cell layer with a sparse lymphohistiocytic lichenoid infiltration. Although there have been a few reports of LPP, there have only 3 cases of linear LPP along the lines of Blaschko in the Korean dermatologic literature. Our patient had lesions on the neck and chin with a linear pattern. In this report, we describe a very rare case of LPP with a linear distribution related to Blaschko's lines on the neck and chin areas.
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20.) Lichen planus pigmentosus presenting in zosteriform pattern.
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Cho S1, Whang KK.
Author information
1
Department of Dermatology, College of Medicine, Ewha Womans University, Seoul, Korea.
Abstract
Lichen planus pigmentosus (LPP) has thus far been described as a condition of unknown etiology which clinically differs from the classical lichen planus (LP) by exhibiting dark brown macules and/or papules mostly in exposed areas and flexural folds and a longer clinical course without pruritus or scalp, nail or mucosal involvement. Histopathologically, LPP shows the typical changes seen in LP, but with thinning of epidermis. We report a case of LPP that developed in a unilateral, zosteriform pattern on the left flank of a 49-year-old man. This case seems to lie in the middle of the spectrum between classical LP and ashy dermatosis, and, to the best of our knowledge, is the first report of LPP presenting in the zosteriform pattern.
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21.) Differential Diagnosis of Linear Eruptions in Children.
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Kruse LL.
Abstract
A 3-year-old girl presented with a linear eruption on her leg for 2 months. She was otherwise healthy and well-appearing. Physical examination showed many small, erythematous, flat-topped papules coalescing into a linear erythematous plaque. At a follow-up visit 9 months later, the eruption had resolved, leaving postinflammatory hypopigmentation.When approaching a cutaneous eruption, appreciating the pattern of the lesions can be instrumental to arriving at the correct diagnosis. For this patient with the acute onset of a plaque on the leg, the differential diagnosis is narrowed by the linear distribution of the skin lesions. The differential diagnosis of linear eruptions in children includes lichen striatus, linear lichen planus, linear psoriasis, inflammatory linear verrucous epidermal nevus, incontinentia pigmenti, phytophotodermatitis, and allergic contact dermatitis. Of note, many of these conditions manifest in a linear manner as a result of cutaneous mosaicism, whereas others are caused by external agents contacting the skin.
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22.) Two cases of lichen striatus with prolonged active phase.
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Pediatr Dermatol. 2014 Mar-Apr;31(2):e67-8. doi: 10.1111/pde.12261. Epub 2014 Jan 24.
Feely MA1, Silverberg NB.
Author information
1
Beth Israel Medical Center, New York, NY; St. Luke's-Roosevelt Hospital Center, New York, NY.
Abstract
Lichen striatus is a localized, eczematous disorder distributed along the lines of Blaschko, primarily affecting children. In the literature, lesions have been described as having an active phase of inflamed lesions for 6 to 12 months followed by flattening and persistent pigmentary alteration. We describe two girls who had prolonged active-phase lesions for 2.5 and 3.5 years, respectively. Practitioners should be aware that lesions of lichen striatus may have a prolonged active phase.
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23.) Effective topical combination therapy for treatment of lichen striatus in children: a case series and review.
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Youssef SM1, Teng JM.
Author information
1
Department of Dermatology, University of Wisconsin-Madison, Madison, WI 53715, USA.
Abstract
Lichen striatus (LS) is an uncommon linear dermatosis that is primarily seen in children from 4 months to 15 years of age. While some of these eruptions are asymptomatic, others can be quite pruritic. In darker-skinned individuals, post-inflammatory hypopigmentation can be significant and may provide a cause for concern for the patients and/or their parents. In our case series of 4 patients, we observed rapid resolution of LS by combining a topical retinoid with a topical steroid. To our knowledge, this is the first report of successful treatment with this kind of combination therapy in the English literature. The patients not only achieved satisfying cosmesis, but also complete resolution of their pruritus. The most common side effect of topical tazarotene is localized irritation at treatment sites, but the patients in this particular series tolerated the treatment well.
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24.) [Lichen striatus with nail abnormality is a self-limiting condition].
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Ugeskr Laeger. 2012 Mar 5;174(10):652-3.
[Article in Danish]
Sandreva T1, Bygum A.
Author information
1
Det Sundhedsvidenskabelige Fakultet, Syddansk Universitet, Campusvej 55, 5230 Odense, Denmark. tsand07@student.sdu.dk
Abstract
A six year-old boy was referred with an asymptomatic linear rash on his left arm and a nail involvement of a fingernail on his left hand. Multiple small erythematous papules coalescing into a linear band extending to the thumb with nail abnormality were noted on the left arm. A diagnosis of lichen striatus was established clinically. The condition has spontaneous remission, although the course of the disease is prolonged when nail involvement exists. The condition is benign and there is no need to perform biopsy.
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