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OBJECTIVE: Patients with nonmelanoma skin cancer (NMSC)-ie, basal cell carcinoma (BCC) or squamous cell carcinoma (SCC)-have an increased risk of developing a second skin cancer. The aim of this study was to describe the frequency, incidence per 1000 person-years, and predictors of a second skin cancer in a cohort of patients with NMSC treated with Mohs micrographic surgery (MMS). MATERIAL AND METHODS: Prospective study of a national cohort of patients with NMSC who underwent MMS at 22 Spanish hospitals between July 2013 and February 2020; case data were recorded in the REGESMOHS registry. The study variables included demographic characteristics, frequency and incidence per 1000 person-years of second skin cancers diagnosed during the study period, and risk factors identified using mixed-effects logistic regression. RESULTS: We analyzed data for 4768 patients who underwent MMS; 4397 (92%) had BCC and 371 (8%) had SCC. Mean follow-up was 2.4 years. Overall, 1201 patients (25%) developed a second skin cancer during follow-up; 1013 of the tumors were BCCs (21%), 154 were SCCs (3%), and 20 were melanomas (0.4%). The incidence was 107 per 1000 person-years (95% CI, 101-113) for any cancer, 90 per 1000 person-years (95% CI, 85-96) for BCC, 14 (95% CI, 12-16) per 1000 person-years for SCC, and 2 (95% CI, 1-3) per 1000 person-years for melanoma. More men than women developed a subsequent skin cancer (738 [61%] vs 463 [39%]). The main risk factors were a history of multiple tumors before diagnosis (relative risk [RR], 4.6; 95% CI, 2.9-7.1), immunosuppression (RR, 2.1; 95% CI, 1.4-3.1), and male sex (RR, 1.6; 95% CI, 1.4-1.9). CONCLUSION: Patients have an increased risk of developing a second tumor after MMS treatment of NMSC. Risk factors are a history of multiple tumors at diagnosis, immunosuppression, and male sex.

Resumen Introduccion Los tumores neuroepiteliales disembrioplasicos (DNET) son un tipo de neoplasia glioneuronal benigna de localizacion tipicamente temporal que producen crisis epilepticas resistentes al tratamiento farmacologico en ninos y adultos jovenes. Objetivo Se muestran 4 casos valorando la utilidad de la resonancia magnetica funcional en el estudio prequirurgico de pacientes con tumores neuroepiteliales disembrionarios. Para la obtencion de imagenes se utilizo un equipo de resonancia magnetica Philipps Intera de 3.0 Tesla y la tecnica Blood Oxygenation Level-Dependent, permitiendo localizar las areas elocuentes de lenguaje y motora mediante la aplicacion de paradigmas especificos. Resultados En un caso el tumor se encontraba adyacente al area de Broca, en 2 casos coincidia con Wernicke, en un paciente estaba menos de 1 cm del area motora de la mano y en otro proximo a la memoria. Solo 2 de los pacientes fueron operados, no produciendose deficit funcional postoperatorio. Se observo activacion hemisferica contralateral al tumor sugestivo de neuroplasticidad en uno de los pacientes. Conclusiones La resonancia magnetica funcional supone un metodo no invasivo que permite evaluar la proximidad de las lesiones a las areas elocuentes, clave en la evaluacion del riesgo quirurgico. Ademas, ha permitido detectar probable neuroplasticidad en un caso, la cual ha garantizado el exito de la cirugia.

Resumen Introduccion La aplicacion WhatsApp es la herramienta preferida de los pacientes para realizar teleconsultas. Sin embargo, la falta de un claro marco de legalidad o un mal uso puede tener repercusiones emocionales negativas en el dermatologo. Objetivos Determinar las caracteristicas basicas de la consulta realizada por WhatsApp, cuantificar objetivamente la repercusion en tiempo consumido y evaluar las consecuencias emocionales en el dermatologo. Material y metodo Es un estudio descriptivo, observacional, transversal y retrospectivo elaborado a partir de la informacion recogida en una encuesta de opinion online, compuesta por 43 preguntas, que fue remitida a los 275 dermatologos de la Seccion Valenciana de la Academia Espanola de Dermatologia y Venereologia. Resultados La encuesta fue contestada por 128 dermatologos, el 46,6% de los encuestados. Todos los participantes manifestaron utilizar WhatsApp o Telegram. El 93% recibia consultas por esta via, y de estos, un 88,3% admitio responder a este tipo de mensajes. El 74,1% de las consultas fueron sobre enfermedad inflamatoria aguda en las que se suele aconsejar un tratamiento farmacologico. Casi un tercio de las mismas necesito de una visita presencial. La confianza con el dermatologo, la comodidad y la rapidez de respuesta fueron las motivaciones para este tipo de consulta. El 31% de los dermatologos participantes reconocio que este tipo de consultas repercute negativamente en su estado de animo y el 82,3% preferiria dejar de recibirlas. Conclusiones Es necesario regular este tipo de consulta. Hay muy pocos estudios sobre este ambito y quedan abiertas muchas cuestiones todavia por esclarecer.

Resumen Antecedentes El registro Regesmohs es un registro de ambito nacional, de pacientes evaluados y sometidos a una cirugia de Mohs, en 17 centros espanoles, desde julio de 2013. Como la cirugia de Mohs es el tratamiento que mejores resultados da para el manejo del carcinoma de celulas basales (CCB) de alto riesgo y otros tumores de la piel, queriamos describir los motivos por los que algunos pacientes fueron considerados no aptos para ser sometidos a este tratamiento y que tratamientos alternativos recibieron. Estos datos pueden ser utiles para evitar excluir a pacientes aptos para ser sometidos a una cirugia de Mohs, para calcular la demanda que estos pacientes generan a nivel sanitario, asi como la demanda que hay de tratamientos de inhibidores de la via de Hedgehog en dicho grupo de pacientes. Objetivo Describir a aquellos pacientes que fueron considerados no aptos para ser sometidos a una cirugia de Mohs tras valoracion prequirurgica y los tratamientos que recibieron. Metodos Regesmohs incluye a todos los pacientes consecutivos para ser sometidos a una cirugia de Mohs en los centros participantes, recogiendo datos sobre las caracteristicas de los pacientes, las intervenciones y los resultados a corto y largo plazo. Se hizo una descripcion de los pacientes considerados no aptos para ser sometidos a una cirugia de Mohs tras valoracion prequirurgica. Resultados Tres mil once pacientes fueron incluidos en el registro Regesmohs entre julio de 2013 y octubre de 2016. En 85 pacientes no se realizo cirugia de Mohs porque se consideraron candidatos inadecuados. Sesenta y siete pacientes presentaban CCB. Las razones para ser considerado paciente no apto fueron: contraindicaciones medicas (27,1%, n = 23), tumores de bajo riesgo (18,8%, n = 16) y tumores gigantes e invasion osea (15,3%, n = 13). Solo un paciente (1,2%) revelo compromiso de ganglios linfaticos y ningun paciente metastasis visceral. De los 85 pacientes considerados no aptos 29 (34,1%) fueron sometidos a cirugia convencional, 24 (28,3%) a radioterapia, 4 (4,7%) a inhibidores de la via de Hedgehog (solo indicado para el CCB) y 2 (2,4%) a tratamiento paliativo. No hubo datos de seguimiento de 14 pacientes (16,5%). Conclusion Las comorbilidades medicas fueron la razon mas habitual para retener la cirugia de Mohs. Retener un tratamiento en funcion de una propagacion a lugares distantes no es algo habitual. La mayoria de los pacientes considerados no aptos recibieron tratamientos mas sencillos: cirugia convencional o radioterapia, siendo los inhibidores de la via de Hedgehog una opcion novedosa.

Introduction The Spanish Mohs Surgery Registry is used to collect data on the use and outcomes of Mohs micrographic surgery (MMS) in Spain. The aim of this study was to describe perioperative and intraoperative data recorded for MMS procedures performed between July 2013 (when the registry started) and January 2016. Material and methods Prospective cohort study of data from 18 hospitals. The data collected included type of anesthesia, surgical technique, hospital admission, number of Mohs stages, management of preoperative risk factors, additional treatments, previous treatments, type of tumor, operating time, and complications. Results Data were available for 1796 operations. The most common tumor treated by MMS was basal cell carcinoma (85.96%), followed by squamous cell carcinoma (6.18%), lentigo maligna (2.81%), and dermatofibrosarcoma protuberans (1.97%). Primary tumors accounted for 66.9% of all tumors operated on; 19.2% of tumors were recurrent and 13.9% were persistent. The most common previous treatment was surgical. MMS was mostly performed under local anesthesia (86.7% of cases) and as an outpatient procedure (71.8%). The frozen section technique was used in 89.5% of cases. One stage was needed to achieve tumor-free margins in 56.45% of patients; 2 stages were required in 32.1% of patients, 3 in 7.1%%, 4 in 2.7%, and 5 or more in 1.8%. The defect was reconstructed by the dermatologist in 98% of patients and the most common technique was flap closure (47.2%). Intraoperative complications were recorded for just 1.62% of patients and the median (interquartile range) duration of surgery was 75 (60-100) minutes. Conclusion The characteristics of the patients and tumors treated by MMS are similar to those reported for similar studies in other geographic areas. Lentigo maligna and dermatofibrosarcoma protuberans accounted for a higher proportion of cases in our series, and repair of the surgical defect by a dermatologist was also more common. Operating times in MMS are not much longer than those reported for other procedures and the rate of intraoperative complications is very low.

Resumen Introduccion En julio de 2013 se inicio la recogida de datos del registro espanol de cirugia micrografica de Mohs, que describe la aplicacion y los resultados de esta tecnica en Espana. En este articulo se describen las caracteristicas del paciente y de los tumores tratados. Material y metodos Se trata de un estudio de cohortes prospectivo en el que participan centros en los que se practica al menos una intervencion semanal de cirugia micrografica de Mohs. En cada centro se incluyen todos los pacientes que son valorados para realizar cirugia de Mohs, excepto los declarados judicialmente incapaces. En este articulo describimos las caracteristicas de los pacientes y los tumores incluidos en la cohorte. Resultados El numero de pacientes incluidos desde julio de 2013 hasta octubre de 2014 es de 655. La mayoria de los tumores cutaneos intervenidos correspondieron a carcinoma basocelular, siendo el infiltrante el subtipo histologico mas frecuente. La mayoria de las cirugias se practicaron en tumores localizados en la cara y el cuero cabelludo, siendo la localizacion mas frecuente la nariz. Casi el 40% de los tumores operados son recurrentes o persistentes, y el tamano tumoral prequirurgico es similar en nuestro medio al descrito en otros estudios australianos o europeos. Hasta el 45,5% de los pacientes habia recibido algun tratamiento quirurgico previo. Conclusion Los datos observados son similares a los de otras series publicadas, y son relevantes para poder valorar la aplicabilidad en nuestro contexto de estudios realizados en otros medios.

Background and objective Chondrodermatitis nodularis helicis (CNH) is a painful idiopathic degenerative condition involving the skin and cartilage of the helix or antihelix of the ear. Topical nitroglycerin 2% is a relatively recent treatment option for CNH that has produced good results, although with adverse effects (17% of cases). The use of a lower concentration would probably achieve similar results with fewer adverse effects. The aim of this study was to evaluate the effectiveness and safety of topical nitroglycerin 0.2% in the treatment of CNH. Material and methods We performed a retrospective observational study of patients treated in 2 Spanish hospitals between 2012 and 2014. The effectiveness of treatment was determined by clinical photography and assessment of symptoms using a verbal numerical rating scale. Results Of the 29 patients treated, 93% showed clinical improvement. In the group of responders, mean treatment duration was 1.8 months and mean follow-up was 5.9 months. Overall tolerance was good in all cases. Conclusion Topical nitroglycerin 0.2% is an effective and well-tolerated conservative treatment option that improves the appearance of lesions and provides symptomatic relief in the majority of patients with CNH.

Resumen Introduccion La condrodermatitis nodular del helix (CNH) es un proceso idiopatico, degenerativo y doloroso que afecta a la piel y al cartilago del helix o del antehelix. Recientemente se ha descrito la utilidad de la nitroglicerina (NTG) topica a 2% en el tratamiento de la CNH con buenos resultados, aunque con una tasa de efectos secundarios en el 17% de los casos. Es probable que a una concentracion menor se pueda mantener el mismo efecto mejorando la tolerancia. Nuestra finalidad fue evaluar la efectividad y seguridad de la NTG topica al 0,2% para el tratamiento de la CNH. Material y metodos Se llevo a cabo un estudio observacional retrospectivo entre los anos 2012 y 2014 en 2 centros hospitalarios espanoles. La efectividad se determino a traves de la evaluacion clinica, realizada mediante seguimiento fotografico, y de los sintomas de la lesion, medido mediante una escala numerica verbal. Resultados Veintinueve pacientes recibieron el tratamiento, de los cuales el 93% manifestaron una mejoria clinica con una duracion media del tratamiento de 1,8 meses y un tiempo de seguimiento medio en los pacientes respondedores de 5,9 meses. La tolerancia fue buena en general en todos los casos. Conclusion La NTG topica al 0,2% se plantea como una opcion conservadora, efectiva y bien tolerada para el tratamiento de la condrodermatitis nodular del helix que mejora tanto la apariencia clinica como la sintomatologia en la mayoria de los pacientes.

Introduction The Spanish registry of Mohs micrographic surgery started collecting data in July 2013. The aim of the registry is to report on the use of this technique in Spain and the outcomes achieved. In the present article, we describe the characteristics of patients and the tumors treated. Material and methods This is a prospective cohort study of patients treated with Mohs micrographic surgery. The participating centers are hospitals where at least one intervention of this type is performed each week. All patients considered for Mohs micrographic surgery in participating centers are included in the registry except those who have been declared legally incompetent. Results Between July 2013 and October 2014, data from 655 patients were included in the registry. The most common tumor involved was basal cell carcinoma, and the most common histological subtype was infiltrative basal cell carcinoma. Most of the tumors treated were located on the face or scalp, and the most common site was the nose. Almost 40% of the tumors treated were recurrent or persistent, and preoperative tumor size was similar to that reported in other European studies and in Australia. In total, 45.5% of patients had received previous surgical treatment. Conclusion The findings are similar to those reported in other studies, and the data collected are useful for assessing whether the results of studies carried out elsewhere are applicable in Spain.

Resumen Introduccion El eritema acral (EA) es una reaccion cutanea relativamente frecuente producida por diferentes agentes quimioterapicos. Otros terminos con los que se le conoce son eritrodisestesia palmoplantar o sindrome pie-mano. Se presenta como un eritema doloroso en palmas y plantas asociado a parestesias en el contexto de un tratamiento oncologico. El EA parece ser dosis-dependiente, y tanto el pico plasmatico como la dosis acumulada del quimioterapico determinan su aparicion. La clinica y los hallazgos histopatologicos sugieren una citotoxicidad directa de la epidermis acral por las altas concentraciones de los quimioterapicos. Los agentes mas frecuentemente implicados son doxorrubicina, 5-fluorouracilo y derivados, citarabina y docetaxel. Material y metodos Se presentan las caracteristicas clinicas e histologicas de una serie de pacientes diagnosticados de eritema acral por quimioterapicos. Se incluyeron en el trabajo todos los pacientes sometidos a quimioterapia que desarrollaron lesiones de eritema acral durante un periodo de tiempo comprendido entre enero de 2000 y diciembre de 2003. Resultados y conclusion Se encontraron 44 casos entre los 2.186 pacientes sometidos a quimioterapia, lo que supuso una incidencia del 2,01 % durante el periodo de estudio, y el 16,75 % de todas las lesiones cutaneas atribuidas a la quimioterapia. El farmaco mas frecuentemente relacionado fue el 5-fluorouracilo en infusion continua, y la mayor incidencia se dio en pacientes tratados con doxorrubicina liposomial. El EA fue toxicidad limitante de dosis en el 29,5% de los casos. Los hallazgos histologicos variaron segun la intensidad clinica de las lesiones, encontrando una dermatitis de interfase con necrosis de queratinocitos variable, dilatacion del plexo vascular superficial y un escaso infiltrado inflamatorio. El tratamiento mas utilizado fue la piridoxina y las medidas locales como fomentos frios, emolientes y corticoids topicos.

Introduction. Acral erythema, also known as palmoplantar erythrodysesthesia or hand-foot syndrome, is a relatively common cutaneous reaction caused by a variety of chemotherapeutic agents. It presents during cancer treatment as painful erythema and paresthesia affecting the palms and soles. It seems to be dose dependent and its appearance is determined by both the peak plasma concentration and the cumulative dose of the chemotherapeutic agent. The symptoms and histopathology findings are suggestive of direct cytotoxicity affecting the epidermis of the extremities caused by high concentrations of chemotherapeutic agents. The most commonly implicated agents are doxorubicin, 5-fluoracil and its derivatives, cytarabine, and docetaxel. Material and methods. We present the clinical and histologic characteristics of a series of patients diagnosed with chemotherapy-induced acral erythema. The study included all patients who developed acral erythema lesions following chemotherapy between January 2000 and December 2003. Results and conclusions. Out of 2186 patients who underwent chemotherapy, 44 cases of acral erythema were identified, representing an incidence of 2.01% during the study period and 16.75% of all cutaneous lesions attributed to chemotherapy. The most commonly implicated drug was 5-fluoracil administered by continuous infusion and the highest incidence was observed in patients treated with liposomal doxorubicin. Acral erythema was a dose-limiting toxic effect in 29.5% of cases. The histologic findings varied according to the clinical severity of the lesions and included interface dermatitis with variable keratinocyte necrosis, dilation of the superficial vascular plexus, and limited inflammatory infiltrate. The most commonly used treatment was pyridoxine, along with topical treatments such as cold compresses, emollients, and topical corticosteroids.

Stewart-Bluefarb syndrome is a rare condition involving skin lesions that share clinical features with Kaposi sarcoma and that are secondary to an underlying arteriovenous fistula. We report the case of a 24-year-old man with progressive growth of skin lesions on the lower third of his left leg. Diagnosis of Stewart-Bluefarb syndrome was confirmed histologically and with detection of an arteriovenous fistula.

Dermatofibroma is a very frequent lesion that usually appears as a slowly growing nodule in the dermis, and preferentially involves the lower extremities of women. Several clinical variants have been described. Giant dermatofibroma has been defined as a rare variant of dermatofibroma measuring more than 5 cm that presents typical histological features and a benign biological behavior. We report the case of a 52-year-old man that presented a giant dermatofibroma with a diameter of 6 cm in the right shoulder and we review the few cases of this variant that have been described in the literature.

Dermatofibroma is a very frequent lesion that usually appears as a slowly growing nodule in the dermis, and preferentially involves the lower extremities of women. Several clinical variants have been described. Giant dermatofibroma has been defined as a rare variant of dermatofibroma measuring more than 5 cm that presents typical histological features and a benign biological behavior. We report the case of a 52-year-old man that presented a giant dermatofibroma with a diameter of 6 cm in the right shoulder and we review the few cases of this variant that have been described in the literature.

The extravasation of cytostatic agents is a known, serious situation that can easily occur and cause chronic, irreversible damage. The incidence of extravasation ranges from 0.1 %-6.5 %, according to different studies. Many cases of extravasation can be prevented by systematizing the administration techniques for cytostatic agents. We present the clinical and histological characteristics of a series of patients with extravasation lesions. Included in the study were all patients treated with chemotherapy who developed localized lesions in the area of the cytostatic injection after extravasation was detected during administration. The patients were studied and followed up for a three-year period, from January 2000 to December 2003, inclusive. We found nine cases of extravasation among the 2,186 patients who were treated with chemotherapy, which represents an incidence of 0.41 %, and 3.4 % of all chemotherapy-induced skin lesions. The cytostatic agent most often involved was vinorelbine, and the most frequent location was the antecubital fossa. The intensity of the lesions made it necessary to delay the next cycle of treatment in 55 % of the cases. The histological findings varied depending on when the biopsy was done, showing panniculitis with low cellularity together with epidermal lesions attributable to direct cytotoxicity. The best treatment for extravasation is prevention, but when it has already occurred, measures vary depending on the cytostatic drug extravasated and the intensity of the lesions. Conservative measures are advisable before surgery.

Adenoid cystic carcinoma is an uncommon tumor of the head and neck. Although it is mainly located in the salivary gland, a skin location has also been described. Metastases are rare, but 50 % of the cases relapse. A 65-year-old male patient had a lesion in the upper lip. After resection, the histological diagnosis was adenoid cystic carcinoma. Treatment was completed with radiotherapy. Ten years later, a nodule was detected in the neck. Its histological diagnosis was lymphatic metastasis due to adenoid cystic carcinoma. Primary cutaneous adenoid cystic carcinoma is a very uncommon tumor in which treatment consists in extensive local excision with free margins. Radiotherapy is not curative and should be reserved for palliative treatments. Multicenter, prospective studies are necessary to determine the best treatment and especially the adjuvant treatment for adenoid cystic carcinoma.

Bowen's disease is a special form of squamous cell carcinoma in situ that usually develops in photoexposed areas of skin. The treatment of choice is surgery. Less frequently, it may appear in other locations such as the nails, glans penis, intertriginous areas and the perianal region, where conventional treatment may be complicated. We contribute a new case of perianal Bowen's disease, which responded to treatment with imiquimod with no evidence of clinical recurrence after 3 years of follow up. Imiquimod appears to be a therapeutic alternative for perianal Bowen's disease, which seems to have a particular tendency to recur in this location where surgery may also be complicated.

Servicio de Dermatologia. Instituto Valenciano de Oncologia. Valencia. Espana. tablecimos el diagnostico de aplasia cutis congenita (ACC) membranosa del cuero cabelludo con signo del collar de pelo, asociada a polidactilia y sindactilia, lo cual podria corresponderse con el grupo 1 de ACC del cuero cabelludo sin anomalias asociadas o asociada a anomalias aisladas3. En relacion con este caso queremos destacar dos aspectos. En primer lugar, que ante cualquier lesion congenita de la linea media debemos realizar una evaluacion exhaustiva antes de la escision quirurgica o biopsia. La tomografia computarizada es el metodo mas exacto para determinar defectos craneales. Si se detecta un defecto oseo con este metodo, esta indicado un examen con resonancia magnetica para determinar si existe extension transcraneal de tejido blando. Si no hay defecto craneal y la lesion es compatible con una aplasia cutis membranosa no es necesario realizar una biopsia. En segundo lugar, que la asociacion de varios marcadores cutaneos en el cuero cabelludo, como por ejemplo el signo del collar de pelo y malformaciones vasculares, aumenta la posibilidad de un disrafismo cervical asociado4,5. Bibliografia 1. Commens C, Rogers M, Kan A. Heterotropic brain tissue presenting as bald cysts with a collar of hypertrophic hair. Arch Dermatol. 1989;125:1253-6. 2. Drolet B, Prendiville J, Golden J, Enjolras O, Esterly NB. Membranous aplasia cutis with hair collars. Congenital absence of skin or neuroectodermal defect? Arch Dermatol. 1995;131:1427-31. 3. Frieden IJ. Aplasia cutis congenita: a clinical review and proposal for classification. J Am Acad Dermatol. 1986;14: 646-60. 4. Herron MD, Coffin CM, Vanderhooft SL. Vascular stains and hair collar sign associated with congenital anomalies of the scalp. Pediatr Dermatol. 2005;22: 200-5. 5. Fujita Y, Yokota K, Akiyama M, Machino S, Inokuma D, Arita K, et al. Two cases of atypical membranous aplasia cutis with hair collar sign: one with dermal melanocytosis, and the other with naevus flammeus. Clin Exp Dermatol. 2005;30:497-9.

La enfermedad de Behcet se caracteriza por la triada de aftas orales y genitales, lesiones cutaneas y afectacion ocular. Tambien es posible observar afectacion articular, neurologica, vascular, gastrointestinal o pulmonar1-3. Una mujer de 38 anos consulto por unas lesiones localizadas en las piernas aparecidas hacia 10 dias. Se trataba de varios nodulos eritematosos, mal delimitados, calientes al tacto y dolorosos a la palpacion (fig. 1). La paciente habia desarrollado fiebre de hasta 38,5 °C desde 5 dias antes de la consulta. Se practico una biopsia donde se pudo observar, bajo una epidermis y una dermis respetada, un vaso de mediano calibre trombosado. La pared de dicho vaso estaba necrosada y presentaba un intenso infiltrado inflamatorio compuesto predominantemente por neutrofilos, pero tambien por linfocitos y eosinofilos. El infiltrado inflamatorio se extendia hasta los lobulillos del tejido celular subcutaneo, en forma de paniculitis de predominio lobulillar (fig. 2). La tincion con orceina puso de manifiesto que se trataba de una arteriola. Estos hallazgos histologicos eran compatibles con una panarteritis nodosa. La paciente volvio a consulta antes de lo previsto, porque pocos dias antes habia desarrollado lesiones orales y genitales. Se trataba de varias lesiones en el paladar duro, en forma de ulceras dolorosas con membrana amarillenta en superficie sobre base eritematosa (fig. 3). En la vulva presentaba una lesion de 1 cm de las mismas caracteristicas. La paciente refirio que habia presentado brotes recurrentes de este tipo de lesiones dolorosas en mucosa oral y genital desde hacia tres anos. Siguiendo los criterios diagnosticos establecidos se diagnostico a la paciente de enfermedad de Behcet con lesiones cutaneas tipo panarteritis nodosa. Durante un ano la paciente ha tenido brotes repetidos de lesiones cutaneas tipo eritema nodoso y papulopustulas y aftas orales y genitales, por lo que ha necesitado tratamiento oral con prednisona durante largos periodos de tiempo para controlar las lesiones y colchicina como tratamiento de mantenimiento. Las lesiones tipo eritema nodoso son una manifestacion cutanea frecuente en la enfermedad de Behcet. Se caracterizan clinicamente por nodulos eritematosos, calientes y dolorosos, localizados principalmente en la cara anterior de las piernas. Desde el punto de vista histopatologico estas lesiones no estan del todo caracterizadas, pero existen distintos trabajos que ponen de manifiesto que este tipo de lesiones, que clinicamente son indistinguibles de un eritema nodoso tipico, presentan una histopatologia distinta a este tipo de paniculitis. Se suele encontrar un infiltrado inflamatorio en la dermis, paniculitis de predominio lobulillar, aunque tambien mixta, y vasculitis, tanto leucocitoclastica como linfocitaria4,5. Cartas al director

oral and genital lesions a few days earlier. There were various painful ulcers on the hard palate, with a yellowish membrane on the surface and an erythematous base (Figure 3). The vulva presented a 1-cm lesion of the same characteristics. The patient reported that she had a 3-year history of recurrent outbreaks of these types of painful lesions in the oral and genital mucosa.

Servicio de Dermatologia, Instituto Valenciano de Oncologia, Valencia, Spain the diagnosis of membranous aplasia cutis congenita (ACC) of the scalp with hair collar sign, associated with polydactyly and syndactyly. This diagnosis could correspond to ACC group 1 of the scalp with no associated abnormalities or associated with isolated anomalies.3 Two aspects should be mentioned in relation to this case. First of all, a comprehensive assessment should be performed on any congenital lesion of the midline before surgical excision or biopsy. Computed tomography is the most accurate method for assessing cranial defects. Any bone defect observed with this method should be examined by magnetic resonance imaging to determine if there is transcranial extension of soft tissue. If there is no cranial defect and the lesion is consistent with membranous aplasia cutis, then biopsy is not necessary. Secondly, the association of various cutaneous markers on the scalp, such as the hair collar sign and vascular malformations, increases the possibility of associated cervical dysraphism.4,5 References 1. Commens C, Rogers M, Kan A. Heterotropic brain tissue presenting as bald cysts with a collar of hypertrophic hair. Arch Dermatol. 1989;125:1253-6. 2. Drolet B, Prendiville J, Golden J, Enjolras O, Esterly NB. Membranous aplasia cutis with hair collars. Congenital absence of skin or neuroectodermal defect? Arch Dermatol. 1995;131:1427-31. 3. Frieden IJ. Aplasia cutis congenita: a clinical review and proposal for classification. J Am Acad Dermatol. 1986;14: 646-60. 4. Herron MD, Coffin CM, Vanderhooft SL. Vascular stains and hair collar sign associated with congenital anomalies of the scalp. Pediatr Dermatol. 2005;22: 200-5. 5. Fujita Y, Yokota K, Akiyama M, Machino S, Inokuma D, Arita K, et al. Two cases of atypical membranous aplasia cutis with hair collar sign: one with dermal melanocytosis, and the other with naevus flammeus. Clin Exp Dermatol. 2005;30:497-9.