THE ERYTHEMA ANNULARE CENTRIFUGUM, A REVIEW / EL ERITEMA ANULAR CENTRIFUGO, REVISION

 

THE ERYTHEMA ANNULARE CENTRIFUGUM,
LOOKING FOR THE ORIGIN. !!!
 
 EL ERITEMA ANULAR CENTRIFUGO,
BUSCANDO EL ORIGEN. !

ACTUALIZADO 2025

EDITORIAL ESPAÑOL:
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Hola Amigos DERMAGICOS, para hoy seleccione este interesante titulo, de una enfermedad conocida bajo el nombre de ERITEMA ANULAR CENTRÍFUGO (EAC). 

 

1.) HISTORIA:
 

El ERITEMA ANULAR CENTRÍFUGO (EAC) fue descrito originalmente por JEAN DARIER en 1916 como una dermatosis caracterizada por placas anulares con bordes eritematosos e indurados, incluyéndose dentro del capítulo de los ERITEMAS FIGURADOS, donde se pueden describir dos (3) variantes:

A.- NO ASOCIADO A MALIGNIDAD: de múltiples causas.

B. ASOCIADA A MALIGNIDAD INTERNA denominada ERITEMA ANULAR CENTRÍFUGO PARANEOPLÁSICO.

C.) IDIOPÁTICOS: Sin causa aparente.

2.) ETIOLOGÍA:

Entidad Clínica: Eritema figurado.

La etiología del ERITEMA ANULAR CENTRÍFUGO (EAC) no está completamente definida, se le considera generalmente una reacción cutánea de hipersensibilidad inespecífica a diversos estímulos,  en otras publicaciones se habla de una respuesta de hipersensibilidad a antígenos desconocidos, dentro de los factores  causas etiológicas asociadas se describen:

2.a) Asociaciones con el eritema anular CENTRÍFUGO:
 
a.) Comidas:

Tomates, queso azul.

b.) Drogas:

Cimetidina, Antimalaricos, Penicilina, Salicilatos, Estrogenos, Progesterona, K vitamin, piroxicam, ampicilina y otros

c.) Agentes vivos: 

Molusco contagioso, Epstein barr. ascariasis, yersinia, Infecciones ESTREPTOCÓCICAS, MYCOBACTERIUM tuberculosis, Dermatofitos, Candida albicans, herpes zoster, borrelia burgdorferi 

d.) Enfermedades del COLÁGENO: 

Lupus, SÍNDROME de Sjogren, sindrome de CREST (variante de esclerodermia)

e.) Enfermedades ampollares: 

Penfigoide ampollar, PÉNFIGO, Enfermedad por IgA lineal. 

f.) Enfermedades escamosas:  

Psoriasis. 

g.) Enfermedades granulomatosas:
 
Sarcoidosis, Tuberculosis, GRANULOMA ANULAR.

h.) Enfermedades malignas: 

Mieloma MÚLTIPLE, Leucemia, Enfermedad de Hodgkin y otros.

i.) Enfermedades endocrinas: 

Tiroiditis de Hashimoto, enfermedad de Graves. 

j.) Enfermedades EOSINOFÍLICAS: 

Sindrome hipereosinofilico.

k.) Enfermedades intestinal inflamatoria:

Enfermedad de Crohn.

L.) ALTERACIONES HORMONALES REUMATOLÓGICAS O HEPÁTICAS..

M.) MISCELÁNEOS:

IDIOPÁTICO, familiar ESTRÉS.

3.) CARACTERÍSTICAS CLÍNICAS:

El ERITEMA ANULAR CENTRÍFUGO se manifiesta típicamente como placas o pápulas eritematosas que se disponen en forma anular, arqueada o policíclica, con crecimiento centrífugo, es decir hacia la periferia, con un aclaramiento central, y los anillos o placas anulares tienen una característica distintiva: "NO SON CERRADOS", tienen forma de herradura. 

Por lo general las lesiones se presentan en tórax y abdomen pero pueden presentarse en otras áreas, pueden ser únicas y múltiples.

Hay dos variantes principales:

A.- Superficial: borde escamoso periférico, a menudo pruriginoso.

B. - Profunda: borde infiltrado sin descamación, generalmente asintomática.

Las lesiones pueden ser únicas o múltiples, con bordes bien definidos y, con frecuencia, escama interna, ambos tipos incluyen a su vez varios subtipos: Con vesiculación, prurito, y descamación. Las lesiones pueden ser o no migratorias. 

4.) EDAD DE PRESENTACIÓN MÁS FRECUENTE:

El  ERITEMA ANULAR CENTRÍFUGO puede afectar a cualquier edad.  Puede presentarse en adultos y niños, desde la infancia hasta la vida adulta. Es algo más frecuente en adultos, con una edad media de aparición alrededor de los 40 años, aunque puede verse también en niños e incluso en época neonatal de manera esporádica.

5.) EVOLUCIÓN:

La evolución suele ser benigna y crónica, con recurrencias esporádicas, autolimitadas en semanas o meses o curso anual y estacional en algunas variantes. Las lesiones suelen desaparecer espontáneamente en algunos casos, aunque pueden recurrir en localizaciones similares u otras zonas del cuerpo, con una variación de tiempo indefinida.

6.) DIAGNÓSTICO DIFERENCIAL:

Las lesiones de ERITEMA ANULAR CENTRÍFUGO deben diferenciarse de otras enfermedades cutáneas que pueden presentar erupciones anulares o similares, como:

- Tiña corporis

- Eritema marginado reumático

- Urticaria anular

- Lupus eritematoso anular (agudo o discoide)

- Linfoma cutis

- Eritema multiforme

- Eritema gyratum repens

- Granuloma anular

- Sarcoidosis

- Lepra tuberculoide

- Micosis fungoide (linfoma cutaneo de celulas T) 

-  Psoriasis

Para confirmar el diagnóstico es necesario realizar biopsia cutánea.

6.) RELACIÓN CON PARANEOPLASIAS:

El ERITEMA ANULAR CENTRÍFUGO (EAC) es considerado una dermatosis reactiva inespecífica y, aunque la MAYORÍA  de los casos son:

A.) IDIOPÁTICOS, es decir no se le encuentra una causa o asociación especifica, y en un porcentaje menor puede asociarse a:

B.- ENFERMEDADES SISTÉMICAS, incluidas infecciones.

C.- ENFERMEDADES AUTOINMUNES y, ocasionalmente, NEOPLASIAS: como linfoma de Hodgkin, leucemias, mieloma múltiple o carcinomas sólidos. 

Por ello, el ERITEMA ANULAR CENTRÍFUGO (EAC) en el caso de manifestarse como PARANEOPLASIA, debe ser considerado también, un MARCADOR CUTÁNEO DE MALIGNIDAD,  sobre todo en adultos mayores.

7.) TRATAMIENTO:

El tratamiento se orienta a aliviar síntomas y, si se identifica una causa desencadenante (infección, medicamento, neoplasia), tratarla. Entre las opciones de tratamiento se describen:

A.- Corticoides tópicos y ocasionalmente sistémicos para inflamación/prurito.

B.. Formulaciones MAGISTRALES a base de ácido salicílico, urea y ácido láctico

C.- Antihistamínicos (si hay prurito).

D.- Análogos de vitamina D y fototerapia UV en algunos casos refractarios.

E.- Eritromicina oral ha mostrado eficacia en algunas casos y las sulfonamidas también.

Muchos casos remiten espontáneamente y no requieren tratamiento específico, en otros hay que buscar la causa, si la hay, y tratarla.

8.) CONCLUSIONES:

El ERITEMA ANULAR CENTRÍFUGO (EAC), es una patología cutánea, cuya causa específica TODAVÍA, hoy día, no ha sido definida o encontrada. La mayoría de los casos son benignos, pero puede estar asociado a otras entidades, e incluso MALIGNIDAD INTERNA.

De modo que si les presenta esta enfermedad en su práctica diaria, piensen en estas asociaciones y clasificación los cuales les ayudarán a identificar mejor la causa, y pedir los respectivos exámenes de laboratorio para reconocer verdaderamente que lo provocó.

Saludos a Todos...

Dr. José Lapenta.

Dr.  José M. Lapenta

EDITORIAL ENGLISH
===================
Hello Dermalogic Friends, for today, I've selected this interesting title about a disease known as ERYTHEMA ANNULARE CENTRIFUGUM (EAC).

1.) HISTORY:

ERYTHEMA ANNULARE CENTRIFUGUM (EAC) was originally described by JEAN DARIER in 1916 as a dermatosis characterized by annular plaques with erythematous and indurated borders. It is included in the chapter on "Figurate Erythemas", where two (3) variants can be described:

A. NOT ASSOCIATED WITH MALIGNANCY: with multiple causes.

B. ASSOCIATED WITH INTERNAL MALIGNANCY: called PARANEOPLASTIC ERYTHEMA ANNULARE Centrifugal (EAC).

C.) IDIOPATHIC: With no apparent cause.

2.) ETIOLOGY:

Clinical Entity: FIGURATED ERYTHEMA.

The etiology of ERYTHEMA ANNULARE CENTRIFUGUM (EAC) is not fully understood. It is generally considered a nonspecific hypersensitivity cutaneous reaction to various stimuli. Other publications describe a hypersensitivity response to unknown antigens. Among the associated etiological factors, the following are described:

2.A) ASSOCIATIONS WITH ERYTHEMA ANNULARE CENTRIFUGUM:

A.) FOODS:

TOMATOES, BLUE CHEESE.

B.) DRUGS:

CIMITIDINE, ANTIMALARIALS, PENICILLIN, SALICYLATES, ESTROGENS, PROGESTERONE, VITAMIN K, PIROXICAM, AMPICILLIN, AND OTHERS.

C.) LIVE AGENTS:

MOLLUSCUM CONTAGIOSUM, EPSTEIN-BARR VIRUS. ASCARIASIS, YERSINIA, STREPTOCOCCAL INFECTIONS, MYCOBACTERIAL TUBERCULOSIS, DERMATOPHYTES, CANDIDA ALBICANS, HERPES ZOSTER, BORRELIA BURGDORFERI.

D.) COLLAGEN DISEASES:

LUPUS, SJOGREN'S SYNDROME, CREST SYNDROME (A VARIANT OF SCLERODERMA).

E.) BLISTERING DISEASES:

BULLOUS PEMPHIGOID, PEMPHIGUS, LINEAR IGA-INDUCED DISEASE.

F.) SQUAMOUS DISEASES:

PSORIASIS.

G.) GRANULOMATOUS DISEASES:

SARCOIDOSIS, TUBERCULOSIS, GRANULOMA ANNULARE.

H.) MALIGNANT DISEASES:

MULTIPLE MYELOMA, LEUKEMIA, HODGKIN'S DISEASE, AND OTHERS.

I.) ENDOCRINE DISORDERS:

HASHIMOTO'S THYROIDITIS, GRAVES' DISEASE.

J.) EOSINOPHILIC DISORDERS:

HYPEREOSINOPHILIC SYNDROME.

K.) INFLAMMATORY BOWEL DISORDERS:

CROHN'S DISEASE.

L.) RHEUMATOLOGICAL OR LIVER HORMONAL DISORDERS.

M.) MISCELLANEOUS DISORDERS:

IDIOPATHIC, FAMILIAL, STRESS.

 

3.) CLINICAL FEATURES:

ERYTHEMA ANNULARE CENTRIFUGUM typically manifests as erythematous plaques or papules arranged in an annular, arcuate, or polycyclic pattern, with centrifugal growth, i.e., toward the periphery, with central clearing. The annular rings or plaques have a distinctive characteristic: they are not closed; they are horseshoe-shaped.

The lesions usually appear on the chest and abdomen but can occur in other areas. They may be single or multiple.

There are two main variants:

A. Superficial: peripheral scaly border, often pruritic.

B. Deep: infiltrated border without scaling, usually asymptomatic.

The lesions can be single or multiple, with well-defined borders and often internal scaling. Both types include several subtypes: with vesiculation, pruritus, and scaling. The lesions may or may not be migratory.

4.) MOST COMMON AGE OF PRESENTATION:

ERYTHEMA ANNULARE CENTRIFUGUM can affect any age. It can occur in adults and children, from infancy to adulthood. It is somewhat more common in adults, with an average age of onset around 40 years, although it can also be seen sporadically in children and even in neonates.

5.) EVOLUTION:

The course is usually benign and chronic, with sporadic recurrences, self-limiting over weeks or months, or annual and seasonal in some variants. The lesions usually resolve spontaneously in some cases, although they may recur in similar locations or other areas of the body, with an indefinite duration of time.

6.) DIFFERENTIAL DIAGNOSIS:

ERYTHEMA ANNULARE CENTRIFUGUM lesions must be differentiated from other skin diseases that may present with annular or similar rashes, such as:

- Tinea corporis

- Erythema marginatum rheumatoidum

- Annular urticaria

- Annular lupus erythematosus (acute or discoid)

- Lymphoma cutis

- Erythema multiforme

- Erythema gyratum repens

- Granuloma annulare

- Sarcoidosis

- Tuberculoid leprosy

- Mycosis fungoides (cutaneous T-cell lymphoma)

- Psoriasis

A skin biopsy is necessary to confirm the diagnosis.

6.) RELATIONSHIP WITH PARANEOPLASMS:

ERYTHEMA ANNULARE CENTRIFUGUM (EAC) is considered a nonspecific reactive dermatosis, and although the MAJORITY of cases are:

A.- IDIOPATHIC, meaning no specific cause or association has been found, a smaller percentage may be associated with:

B.- SYSTEMIC DISEASES, including infections.

C.- AUTOIMMUNE DISEASES and, occasionally, NEOPLASIA: such as Hodgkin's lymphoma, leukemia, multiple myeloma, or solid carcinomas.

Therefore, ERYTHEMA ANNULARE CENTRIFUGUMERYTHEMA ANNULARE CENTRIFUGUM (EAC), if it manifests as a PARANEOPLASIA, should also be considered a CUTANEOUS MARKER OF MALIGNANCY, especially in older adults.

7.) TREATMENT:

Treatment is aimed at relieving symptoms and, if a triggering cause (infection, medication, neoplasia) is identified, treating it. Treatment options include:

A. Topical and occasionally systemic corticosteroids for inflammation/itching.

B. MAGISTRAL formulations based on salicylic acid, urea, and lactic acid.

C. Antihistamines (if pruritus is present).

D. Vitamin D analogues and UV phototherapy in some refractory cases.

E. Oral erythromycin has shown efficacy in some cases, also sulfonamides.

Many cases resolve spontaneously and do not require specific treatment. In others, the cause, if present, must be identified and treated.

8.) CONCLUSIONS:

ERYTHEMA ANNULARE CENTRIFUGUM (EAC) is a skin condition whose specific cause has not yet been defined or discovered. Most cases are benign, but it can be associated with other conditions, including INTERNAL MALIGNANCIES.

So, if you experience this condition in your daily practice, consider these associations and classifications, which will help you better identify the cause and order the appropriate laboratory tests to truly identify the cause.

Greetings to all...

Dr. José Lapenta

Dr. José M. Lapenta

 

 

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REFERENCIAS BIBLIOGRÁFICAS / BIBLIOGRAPHICAL REFERENCES
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4.) Erythema annulare centrifugum, angio-oedema in lupus vulgaris.
5.) Erythema annulare centrifugum. A case due to tuberculosis.
6.) [Erythema annulare centrifugum of the legs symptomatic of prostate adenocarcinoma; a specific paraneoplastic syndrome]?
7.) Erythema annulare centrifugum and Hodgkin's disease: association with disease activity.
8.) On erythema annulare centrifugum-type of psoriasis.
9.) Annular psoriasiform eruption with lymphocytic
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10.) Psoriasis: odd varieties in the adult.
11.) [Localized form of psoriasis of the erythema annulare centrifugum type with pustulation]
12.) [Psoriasis and pustular psoriasis of the erythema annulare centrifugum type (3 cases)].
13.) Erythema annulare centrifugum induced by molluscum contagiosum.
14.) Erythema annulare centrifugum and molluscum contagiosum.
15.) [Hypereosinophilic syndrome. Cutaneous picture of "erythema annulare centrifugum"--comparison with ultrastructural study].
16.) [Hypereosinophilic syndrome. Centrifugal annular erythema as an initial manifestation].
17.) Erythema annulare centrifugum as the presenting sign of the hypereosinophilic syndrome: observations on therapy.
18.) [Erythema annulare centrifugum as a symptom in acute myeloid leukemia].
19.) Annular erythemas in infants associated with autoimmune disorders in their mothers. Report on three cases.
20.) Ampicillin induced erythema annulare centrifugum.
21.) [Specific leukemic infiltration with the appearance of erythema annulare centrifugum (Darier) in chronic lymphadenosis].
22.) Unusual cutaneous associations of a malignant carcinoid tumour of the bronchus--erythema annulare centrifugum and white banding of the toe nails.
23.) [The bullous variant of erythema annulare centrifugum Darier in Candida albicans infections].
24.) [Migrant eruptions in lupus erythematosus. Apropos of a case of lupus erythematosis gyratus repens].
25.) Erythema annulare centrifugum coincident with Epstein-Barr virus infection in an infant.
26.) Erythema annulare centrifugum and relapsing polychondritis.
27.) Vulvar disease in children: a clinical audit of 130 cases.
28.) Amitriptyline-induced erythema annulare centrifugum.
29.) Drugs causing fixed eruptions: a study of 450 cases.
30.) Erythema annulare centrifugum in a patient with polyglandular autoimmune disease type 1.
31.) Recurrent ringed lesion of the sole: erythema annulare centrifugum.
32.) Erythema annulare centrifugum associated with pregnancy.
33.) [Annular erythema in childhood--a new eosinophilic dermatosis].
34.) Erythema annulare centrifugum and intestinal Candida albicans infection--coincidence or connection?
35.) Dermacase. Erythema annulare centrifugum.
36.) Psoriasis: changing clinical patterns.
37.) Erythema annulare centrifugum and Escherichia coli urinary infection.
38.) Sarcoidosis presenting as erythema annulare centrifugum.
39.) [Recurrent annular erythema--cutaneous manifestation of Sjogren syndrome with anti SS-A (Ro) and anti SS-B (La) antibodies].
40.) Non-small-cell lung cancer with multiple paraneoplastic syndromes.
41.) [Darier's erythema annulare centrifugum of neonatal onset with a 15 years' follow-up. Efficacy of interferon and role of cytokines].
42.) Erythema annulare centrifugum unresponsive to immunosuppressive therapy.
43.) Annular vasculitis associated with pregnancy.
44.) Inflammatory carcinoma masquerading as erythema annulare centrifugum.
45.) [Darier's erythema annulare centrifugum and primary Vaquez polycythemia]
46.) Recurrent annular erythema with anti-SSA/Ro and anti-SSB/La antibodies localized on palms and fingers in an elderly man.
47.) Clinical and immunological analysis of annular erythema associated with Sjogren syndrome.
48.) Clinical, serological and immunogenetic features of Japanese anti-Ro/SS-A-positive patients with annular erythema.
49.) Persistent annular erythema of infancy.
50.) HLA antigens in anti-Ro(SS-A)-positive patients with recurrent annular erythema.
51.) Urticated annular erythema: a new manifestation of Sjogren's syndrome.
52.) Recurrent annular erythema associated with anti-SS-
53.) Erythema annulare centrifugum associated with gold sodium thiomalate therapy.
54.) Annular erythema. A comparative study of Sjogren syndrome with subacute cutaneous lupus erythematosus.
55.) Annular erythema associated with Sjogren's syndrome: a variant of systemic lupus erythematosus [see comments]
56.) Lymphocyte response to staphylococcal enterotoxin B in patients with annular erythema associated with Sjogren syndrome.
57.) ICAM-1 and LFA-1 expressions in the lesional skin of annular erythema associated with Sjogren syndrome.
58.) Annular erythema associated with primary Sjogren syndrome: analysis of T cell subsets in cutaneous infiltrates [see comments]
59.) Annular erythema in identical twins.
60.) Annular erythema: a possible association with primary Sjogren's syndrome.
61.) Neonatal lupus syndrome and microtubular structure.
62.) Erythema annulare of infancy.
63.) An annular erythema of infancy.
64.)Reactive annular erythema after intramuscular vitamin K.
65.) Annular erythema of infancy.
66.) [Erythema annulare centrifugum revealing linear IgA dermatitis of childhood]
67.) Erythema annulare centrifugum: an unusual case due to hydroxychloroquine sulfate.
68.) 'Persistent' annular erythema of infancy.
69.) Erythema annulare centrifugum associated with ascariasis.
70.) Annular erythema of infancy.
71.) Annular erythema as an unusual manifestation of chronic disseminated lupus erythematosus.
72.) Multiple annular erythema [letter]
73.) Reactive erythemas: erythema annulare centrifugum and erythema gyratum repens.
74.) [Squamous erythema annulare centrifugum]
75.) Annular erythema associated with Sjogren's syndrome [letter; comment]
76.) An intensive care unit nurse with a recurring annular lesion. Erythema annulare centrifugum (EAC).
77.) Erythema annular centrifugum in Q fever.
78.) Erythema annulare centrifugum caused by hydrochlorothiazide-induced interstitial nephritis.
79.) [Erythema annulare centrifugum disclosing hyperthyroidism]
80.) Eruption resembling erythema annulare centrifugum due to thiacetazone [letter]
81.) [Erythema annulare centrifugum caused by aldactone]
82.) [On the physiopathology of erythema annulare centrifugum]
83.) Erythema annulare centrifugum associated with liver disease.
84.) [Erythema annulare centrifugum and Hashimoto's thyroiditis]
85.) Erythema annulare centrifugum associated with piroxicam [letter]
86.) Annular erythema with histologic features of leukocytoclastic vasculitis in ulcerative colitis.
87.) Erythema annulare centrifugum and malignant histiocytosis--report of a case.
88.) Pityrosporum infection in an infant with lesions resembling erythema annulare centrifugum.
89.) Erythema annulare centrifugum: a review of 24 cases with special reference to its association with underlying disease.
90.) [Pemphigoid with recurrence of the centrifugal-development bullous annular erythema type]
91.) [Bullous pemphigoid with an erythema annulare centrifugum appearance and a bullous border]
92.) [Erythema annulare centrifugum and antinuclear antibodies]
93.) Erythema annulare centrifugum. A case due to tuberculosis.
94.) Erythema annulare centrifugum and Graves' disease [letter]
95.) Cimetidine-induced erythema annulare centrifugum: no cross-sensitivity with ranitidine.
96.) [Nosologic problems posed by a case of desquamative erythema annulare centrifugum]].
97.) [Erythema annulare centrifugum Darier as a reaction form in diseases of the reticulo-histiocyte system].
98.) [Erythema annulare centrifugum of Darier, squamous variety].
99.) [Erythema annulare centrifugum with loco-regional recurrences during the course of tuberculous coxitis. Focal reaction to tuberculin].
100.) The Gyrate Erythemas
101.) Erythema annulare centrifugum: case due to hypersensivite to blue cheese penicillium.

102.) Erythema annulare centrifugum in a patient with crohn disease.
103.) Erythema annulare centrifugum associated with herpes zoster.
104.) Paraneoplastic erythema annulare centrifugum eruption: PEACE.
105.) The expanding spectrum of cutaneous borreliosis.
106.) Linear IgA dermatosis presenting with erythema annulare centrifugum lesions: report of three cases in adults.
107.) A new type of annular erythema with perieccrine inflammation: erythema papulatum centrifugum.
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ERYTHEMA GYRATUM REPENS AND MALIGNANCY / ERITEMA GYRATUM REPENS Y MALIGNIDAD.

 ERYTHEMA GYRATUM REPENS, ANOTHER CUTANEOUS MARKER OF MALIGNANCY. ??

 
 ERITEMA GYRATUM REPENS, OTRO MARCADOR CUTÁNEO DE MALIGNIDAD.??

 

EDITORIAL ENGLISH 
=================== 
Hello friends of the network, DERMAGIC again with ANOTHER CUTANEOUS SIGN of MALIGNANCY. THE ERYTHEMA GYRATUM REPENS (EGR).

HISTORY AND CHRONOLOGY:

in most cases. THE ERYTHEMA GYRATUM REPENS or The GAMMEL syndrome. For the first time described in the year 1.952-1953 by JOHN A. GAMMEL in a female patient of 55 years with BREAST CANCER (ADENOCARCINOMA), with axillary invasion, the cutaneous sign appeared 9 months before the malignancy and disappeared 10 days after the surgery. 

- In 1.975 SKOLNICK and MAIMAN reviewed the literature and found 31 cases of ERYTHEMA GYRATUM REPENS, all of them ASSOCIATED WITH MALIGNANCY, by that date only 3 cases NOT ASSOCIATED WITH MALIGNANCY had been reported. 

- By the year 1.985, 28 cases of ERYTHEMA GYRATUM REPENS had been reported, of which 24 (85%) were associated with malignancy. In 15 cases (68%) the CUTANEOUS SIGN was present before the malignancy, in 6 cases (27%) it appeared after the malignancy was detected, and in 1 case (4%),  it appeared at the same time as the malignancy. 

- By the year 1.995, 60 cases of ERYTHEMA GYRATUM REPENS had been described, of which 46 (77%) were associated with malignancy. And 14 cases (23%) not associated with malignancy. 

- In recent years, rashes have been described "LIKE" to ERYTHEMA GYRATUM REPENS in different pathologies, type EGR-like eruption. 

- By the year 2.012 One hundred twelve (112) original cases of EGR were selected from the literature for detailed review. Among these, 58 cases (70%) were associated with an underlying neoplasm, 25 non-paraneoplastic cases, and 29 cases were considered as different dermatoses simulating EGR, in its clinical presentation (EGR-like eruption). 

CLASSIFICATION:

Based on these findings, we can classify the ERYTHEMA GYRATUM REPENS in three variants: A.) ASSOCIATED WITH MALIGNITY (70-84%), in most cases, and the minority is B.) NOT ASSOCIATED with MALIGNANCY (16-30%). And another VARIANT: C.) CUTANEOUS ERUPTIONS MIMICKING (LIKE) ERITEMA GYRATUM REPENS. 

A.) MALIGNANCY ASSOCIATED WITH ERYTHEMA GYRATUM REPENS:
 
1.) LUNG.

2.) ESOPHAGUS. 

3.) BREAST.

4.) UTERUS.
 

5.) PHARYNX. 

6.) STOMACH. 

7.) ANUS. 

8.) BLADDER. 

9.) INTESTINE. 

10.) HODGKINS DISEASE 

11.) TONGUE. 

12.) PROSTATE. 

13.) PANCREAS. 

14.) MYELOMA. 

15.) METASTASIS 

B.) ERYTHEMA GYRATUM REPENS NOT ASSOCIATED WITH MALIGNANCY: 

1.) ICHTHYOSIS.

2.) PALMAR AND PLANTAR HYPERKERATOSIS.

3. PITYRIASIS RUBRA PILARIS.

4.) PSORIASIFORM LESIONS.

5.) BULLOUS PEMPHIGOID.

6.) PEMPHIGUS VULGARIS.

7.) BULLAE DISEASES.

8.) DISCOID LUPUS ERYTHEMATOSUS.

9.) PNEUMONIA..

10.) RHEUMATOID ARTHRITIS.

11.) HEALTHY PEOPLE.

12.) BREAST HYPERTROPHY.

13.) TUBERCULOSIS.

14.) HYPEREOSINOPHILIC SYNDROME. 

C.) ERYTHEMA GYRATUM REPENS-LIKE ERUPTIONS: 

1.) LEPROSY.

2.) LEUKOCYTOCLASTIC VASCULITIS.
 

3.) URTICARIAL VASCULITIS.
 

4.) PSORIASIS.
 

5.) EPIDERMOLYSIS BULLOSA ACQUISITA.
 

6.) MYCOSIS FUNGOIDES.
 

7.) ERYTHROKERATODERMIA VARIABILIS.
 

8.) SJOGREN SYNDROME.
 

9.) SYSTEMIC LUPUS ERYTHEMATOSUS.
 

10.) DRUGS (PENICILLIN). 

" ...The pattern of EGR has been described as "wood-grained,serpiginous, zebralike", cypress rings gyrate, whorled,and  swirls of rope. The expanding borders are usually macular but may occasionally be palpable Scale is usually present. 

The eruption of ERYTHEMA GYRATUM REPENS (EGR), moves rapidly across the surface of the skin, usually about 1 cm per day, That is why at the beginning in its first description it was called ERYTHEMA GYRATUM MIGRATORIUM, later it was changed to the definitive name used today, REPENS.

CONCLUSION:

So friends and colleagues dermatologists, if you find this CUTANEOUS SIGN in any of your patients, check yourself with this BIBLIOGRAPHIC REVIEW and perform the appropriate tests, mainly rule out MALIGNANCY, but remember that a Lower percentage is not associated with it.

In the  references the facts, in the attached, classic ERYTHEMA GYRATUM REPENS 

Greetings to all. 

Dr José Lapenta.

Dr José M. Lapenta.

EDITORIAL ESPAÑOL
=================== 
Hola amigos de la red DERMAGIC de nuevo con ustedes OTRO SIGNO CUTÁNEO marcador de MALIGNIDAD, EL ERITEMA GYRATUM REPENS (EGR).

HISTORIA Y CRONOLOGÍA: 

En la mayoría de los casos. EL ERITEMA GYRATUM REPENS O síndrome de GAMMEL. Por primera vez descrito en el año de 1.952-1953 por JOHN A. GAMMEL en una paciente femenina de 55 años con cáncer de mama (ADENOCARCINOMA) con invasión axilar, el signo cutáneo apareció 9 meses antes de la malignidad, y desapareció 10 días después de la cirugía. 

-  En el año de 1.975 SKOLNICK y MAIMAN revisaron la literatura y encontraron 31 casos de ERITEMA GYRATUM REPENS, todos ASOCIADOS A MALIGNIDAD, para esa fecha solo se habían reportado 3 casos NO ASOCIADOS A MALIGNIDAD. 

- Para el año de 1.985 se habían reportado 28 casos de ERITEMA GYRATUM REPENS, de los cuales 24 (85%) estaban asociados a malignidad. En 15 de los casos (68%) el SIGNO CUTÁNEO se presentó antes que la malignidad, en 6 casos (27%) apareció luego de detectada la malignidad, y en 1 caso (4 %) apareció al mismo tiempo que la malignidad. 

- Para el año de 1.995 se habían descrito 60 casos de ERITEMA GYRATUM REPENS, de los cuales 46 (77%) estuvo asociado a malignidad. y 14 casos(23%) no asociado a malignidad. 

- En los últimos años se han descrito erupciones cutáneas "similares al ERITEMA GYRATUM REPENS en diferentes patologías, tipo LIKE-EGR.

Para el año 2.012 Ciento doce casos (112) originales de EGR fueron seleccionados de la literatura para su revisión detallada. Entre estos, 58 casos (70%) se asociaron con una neoplasia subyacente, 25 casos (30%) no para-neoplásicos, y 29 casos fueron considerados como diferentes dermatosis simulando EGR en su presentación clínica (erupción tipo EGR). 

CLASIFICACIÓN:

En base a estos hallazgos podemos clasificar al ERITEMA GYRATUM REPENS en TRES variantes: A.) ASOCIADO A MALIGNIDAD, en la mayoría de los casos (70-84%), B.) NO ASOCIADO A MALIGNIDAD (16-30%), una minoría. y otra VARIANTE: C.) ERUPCIONES CUTÁNEAS QUE SIMULAN al ERITEMA GYRATUM REPENS. 

A.) MALIGNIDADES ASOCIADAS AL ERITEMA GYRATUM REPENS: 

1.) PULMÓN.

2.) ESÓFAGO.

3.) SENOS.

4.) ÚTERO.

5.) FARINGE.

6.) ESTÓMAGO.

7.) ANO.

8.) VEJIGA.

9.) INTESTINO.

10.) ENFERMEDAD DE HODGKIN

11.) LENGUA.

12.) PRÓSTATA.

13.) PANCREAS.

14.) MIELOMA.

15.) METASTASIS  

B.) ERYTHEMA GYRATUM REPENS NO ASOCIADO A MALIGNIDAD: 

1.) ICTIOSIS.

2.) HIPERQUERATOSIS PALMO PLANTAR.

3.) PITIRIASIS RUBRA PILAR.

4.) LESIONES PSORIASIFORMES.

5.) PENFIGOIDE BULOSO.

6.) PÉNFIGO VULGAR.

7.) ENFERMEDADES AMPOLLARES.

8.) LUPUS ERITEMATOSO DISCOIDEO CRÓNICO.

9.) NEUMONÍA.

10.) ARTRITIS REUMATOIDE.

11.) PERSONAS SANAS.

12.) HIPERTROFIA MAMARIA.

13.) TUBERCULOSIS.

14.) SÍNDROME HIPER-EOSINOFILICO.  

C.) ERYTHEMA GYRATUM REPENS-LIKE ERUPCIONES: 

1.) LEPRA.
 

2.) VASCULITIS LEUCOCITOCLASTICA.
 

3.) VASCULITIS URTICARIANA.
 

4.) PSORIASIS.
 

5.) EPIDERMOLISIS BULOSA ADQUIRIDA.
 

6.) MICOSIS FUNGOIDES.
 

7.) ERITROQUERATODERMIA VARIABILIS.
 

8.) SÍNDROME DE SJOGREN.
 

9.) LUPUS ERITEMATOSO SISTÉMICO.
 

10.) DROGAS (PENICILINA). 

CARACTERÍSTICAS CLÍNICAS:

"...El patrón del EGR se ha descrito como disposición de anillos longitudinales tipo "madera-veteada", serpiginoso, cebra-like, anillos de ciprés, y remolinos de cuerda. Los bordes de expansión suelen ser maculares pero ocasionalmente puede ser palpable, la descamación está generalmente presente (ver fotos).

La erupción del ERITEMA GYRATUM REPENS (EGR), Se mueve rápidamente a través de la superficie de la piel, usualmente alrededor de 1 cm por día, por ello al comienzo en su primera descripción se le denomino ERITEMA GYRATUM MIGRATORIO, luego se le cambio a la definitiva que se usa hoy dia, REPENS.

CONCLUSIÓN:

De modo amigos y colegas dermatólogos, si se te presenta este SIGNO CUTÁNEO en alguno de sus pacientes, orientate con esta REVISIÓN BIBLIOGRÁFICA y realiza los exámenes adecuados, principalmente descartar MALIGNIDAD, pero recordemos que un porcentaje menor no está asociado a ella.

En las referencias, los hechos, en las fotos: ERITEMA GYRATUM REPENS CLÁSICO. 

Saludos a todos.
 

Dr José Lapenta.

Dr. Jose M. Lapenta. 

=============================================================          
REFERENCIAS BIBLIOGRÁFICAS / BIBLIOGRAPHICAL REFERENCES 
=============================================================

1.) Erythema gyratum repens: A paraneoplastic eruption; Clinical review 

2.) Cutaneous manifestations of cancer. 

3.) Erythema gyratum repens in association with renal cell carcinoma. 

4.) Erythema gyratum repens: another case of a rare disorder but no new insight into pathogenesis. 

5.) Cutaneous paraneoplastic syndromes in solid tumors. 

6.) Erythema gyratum repens unassociated with underlying malignancy. 

7.) Erythema gyratum repens-like eruption in a patient with Sjogren syndrome. 

8.) Paraneoplastic bullous pemphigoid resembling erythema gyratum repens. 

9.) Eruption resembling erythema gyratum repens in linear IgA dermatosis. 

10.) Erythema gyratum repens associated with hypereosinophilic syndrome. 

11.) Erythema gyratum repens. A case studied with immunofluorescence,  immunoelectron microscopy and immunohistochemistry. 

12.) Erythema gyratum repens: direct immunofluorescence microscopic findings. 

13.) Erythema gyratum repens without underlying disease. 

14.)Reactive erythemas: erythema annulare centrifugum and erythema gyratum  repens. 

15.) Subcorneal accumulation of Langerhans cells in erythema gyratum repens. 

16.) Erythema gyratum repens in a healthy woman. 

17.)[Gammel's non-paraneoplastic erythema gyratum repens]. 

18.) [Erythema gyratum repens type eruption]. 

19.) A mechanism of peripheral spread or localization of inflammatory  reactions--role of the localized ground substance adaptive phenomenon. 

20.) Episodic erythema gyratum repens with ichthyosis and palmoplantar  hyperkeratosis without signs of internal malignancy. 

21.) Erythema gyratum repens. A cutaneous marker of malignancy. 

22.) Bullous pemphigoid with figurate erythema associated with carcinoma of the bronchus. 

23.) Erythema figuratum versus erythema gyratum repens. 

24.) Erythema gyratum repens, a stage in the resolution of pityriasis rubra  pilaris? 

25.)[Erythema gyratum repens--a paraneoplastic dermatosis]. 

26.)Erythema gyratum repens unassociated with internal malignancy. 

27.) Erythema gyratum repens. 

28.) Gyrate erythema. 

29.) Infantile epidermodysplastic erythema gyratum responsive to imidazoles. A new entity? 

30.) Erythema gyratum repens with associated squamous cell carcinoma of the lung. 

31.) [Cutaneous paraneoplastic syndromes]. 

32.) [Erythema gyratum repens and primary bronchial cancer. Disappearance of the dermatosis under general corticoid therapy]. 

33.) [Erythema gyratum repens of Gammel and Hodgkin's disease]. 

34.) Erythema gyratum repens-like figurate eruption in bullous pemphigoid. 

35.) [Erythema gyratum repens]. 

36.) [Erythema gyratum repens]. 

37.) Erythema gyratum repens: spontaneous resolution in a healthy man. 

38.) Erythema gyratum repens with pulmonary tuberculosis. 

39.) [Gammel's erythema gyratum repens and acquired ichthyosis associated with esophageal carcinoma]. 

40.) [Erythema gyratum repens or Gammel paraneoplastic syndrome. A case with 

epidermoid carcinoma developed on a megaesophagus]. 

41.) Erythema gyratum repens--an immunologically mediated dermatosis? 

42.) Erythema gyratum repens with metastatic adenocarcinoma. 

43.) [Erythema gyratum repens (Gammel's syndrome)] 

44.) Figurate and bullous eruption in association with breast carcinoma. 

45.) [Erythema gyratum repens associated with bronchial carcinoma] 

46.) Erythema gyratum repens. Reports of two further cases associated with  carcinoma. 

47.) Carcinoma of the breast, pemphigus vulgaris and gyrate erythema. 

48.) [Premycotic erythema simulating erythema gyratum repens]. 

49.) [An unusual paraneoplastic syndrome: erythema "gyratum repens" or Gammel's syndrome]. 

50.) [An unusual paraneoplastic syndrome: erythema gyratum repens. Its relation with bronchial cancer]. 

51.) Cutaneous manifestations of lung cancer.
52.) Cutaneous manifestations of breast cancer.
53.) Erythema gyratum repens.
54.) Erythema gyratum repens unassociated with underlying malignancy.
55.) Erythema gyratum repens unassociated with internal malignancy.
56.) Erythema gyratum repens associated with cryptogenic organizing pneumonia.
57.) Erythema gyratum repens preceding the onset of rheumatoid arthritis.
58.) Erythema gyratum repens associated with hypereosinophilic syndrome.
59.) [Erythema gyratum repens of Gammel and Hodgkin's disease].
60.) Erythema gyratum repens is not an obligate paraneoplastic disease: a systematic review of the literature and personal experience.
61.) Novel presentation of lepromatous leprosy in an erythema gyratum repens-like pattern.
62.) Leucocytoclastic vasculitis presenting as an erythema gyratum repens-like eruption.
63.) Urticarial vasculitis presenting as erythema gyratum repens-like eruption.
64.) Erythema gyratum repens-like eruption occuring in resolving psoriasis during methotrexate therapy.
65.) Erythema gyratum repens-like eruption in a patient with epidermolysis bullosa acquisita
associated with ulcerative colitis.
66.) Erythema gyratum repens-like eruption in mycosis fungoides: is dermatophyte superinfection
underdiagnosed in cutaneous T-cell lymphomas?
67.) Erythrokeratodermia variabilis with erythema gyratum repens-like lesions.
68.) Erythema gyratum repens-like eruption in a patient with Sjögren syndrome.
69.) Neutrophilic dermatosis with an erythema gyratum repens-like pattern in systemic lupus erythematosus.
70.) Penicillin-induced anti-p200 pemphigoid: an unusual morphology.
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