Deep dermatofibrosarcoma protuberans in a ninety-year old-woman.

A 92-year-old woman was referred for the assesment of an asymptomatic subcutaneous tumor that developed after an accidental fall. The mass clinically and radiologically simulated a subcutaneous hematoma. Finally, the histological study was consistent with subcutaneous dermatofibrosarcoma protuberans.

Estudio epidemiológico sobre la demandahistopatológica en Dermatología, en unÁrea de Salud de la provincia de Zaragoza (1997-2001) / Epidemiologic study about the histopathologic requirements in Dermatology in a Sanitary Area of Zaragoza

En el presente estudio hemos recogido, de forma retrospectiva y descriptiva, los datos referentes a los diagnósticos histopatológicos de todos lospacientes que requirieron estudio histopatológico, bien por motivo diagnóstico (biopsia cutánea) o terapéutico (cirugía), en el Departamento de Dermatologíadel Hospital Clínico, durante el periodo comprendido entre el 1 de Enero de 1997 y el 31 de Diciembre de 2001 (periodo total de 5 años).Con ello, se obtiene y describe un perfil de aquellas patologías que más frecuentemente requieren biopsia cutánea, así como su distribución por gruposde edad y sexo. Durante el periodo estudiado, se han contabilizado un total de 11.647 registros histopatológicos con una media de 2.329 biopsiaspor año y de 6,4 biopsia por día. El 59,3% (N: 6.911) de los registros corresponde a pacientes del sexo femenino, mientras que el 40,7% (N: 4.736)corresponde a pacientes varones, con una proporción varón:mujer de 1:1,46.De forma global, el grupo de diagnóstico más frecuentemente biopsiado es el de Tumores Pigmentarios, el cual supone un 40,4% (N: 4.706) del totalde las biopsias. Los siguientes grupos en frecuencia son el de los Tumores Cutáneos Benignos (de origen no pigmentario), el cual reúne un 28% (N:3.256) de los registros, y el de los Carcinomas Cutáneos, con un 16% (N: 1.859) del total.Con mucho menor porcentaje, aparecen los grupos de Lesiones Premalignas (3,7%; N: 432), Patología no concluyente o ausencia de ésta (2,5%: N:291), y Lesiones de Origen Traumático (2,4%; N: 275). De un total de 250 diagnósticos histopatológicos registrados diferentes, los de mayor frecuenciason el de Nevus Intradérmico con un 24,1% (N: 2.812) y el Nevus Melanocítico Compuesto con un 12,1% (N: 1.406) (AU)

All the patients requiring histopathologic study in our Dermatology Department of HCU Lozano Blesa in Zaragoza, from January 1997 to december2001, were registered.So, we have obtained and described a feature about the more frequent histopathological diagnoses, and its distribution by sex and groups of age. Atotal among of 11647 patients were studied during this survey (2329 biopsies per year, and 6.4 per day); including 4736 male (40.7%) and 6911female (59.3%). The ratio male/female was 1:1.46.The five groups more frequently biopsed in a decreased order was: Pigmentary Tumours (40.1%, N: 4706), Benign Tumours (28%, N: 3256), CutaneousCarcinomas (16%, N: 1859), Cutaneous Premalign Lesions (3.7%, N: 432), and Dermatosis with Traumatic origen (2.4%, N: 275).The most frequent histopathologic diagnose was Intradermal Nevus (24.1%, N: 2812), and the second was Compound Melanocytic Nevus (12.1%,N:1406) (AU)

Histiocitoma fibroso maligno pleomórficocutáneo de rápido crecimiento / Cutaneous pleomorphic malignant fibrous histiocytoma with a rapidly growing

El histiocitoma fibroso maligno es un sarcoma de partes blandas, de presentación preferente entre la cuarta y quinta década de la vida, localizándoseprincipalmente en la zona proximal de extremidades inferiores. Histológicamente existen varios tipos según la morfología celular del tumor: la formapleomórfica, estoriforme (predominando los fibroblastos o los histiocitos como células dominantes), mixoide, de células gigantes e inflamatorio.La forma clínica más frecuente de presentación es como lesión tumoral profunda, localizada en el músculo esquelético, que puede protuir la piel que larecubre.Comentamos el caso de un varón de 81 años que consultó por una tumoración cutánea asintomática, de gran tamaño y rápido crecimiento (siete meses),localizado en pared torácica izquierda. Histológicamente el tumor mostraba un patrón típicamente estoriforme, formado por histiocitos y fibroblastos atípicos.Las células fueron positivas para vimentina, lisozima y CD68, hallazgos compatibles, con un histiocitoma fibroso maligno pleomórfico.Se revisan las características clínico patológicas y citogenéticas de este tumor, así como las distintas opciones terapéuticas (AU)

Malignant fibrous histiocytoma,(MFH) is the most common soft-part sarcoma arising between the fourth an fifth decades of life, that usually involvesthe proximal lower extremities. Its histology has shown a wide variety of cell morphology, including storiform-pleomophic (fibroblastic or histiocyticcells dominant), myxoid, giant cell and inflammatory types. The most usual presentation is a tumoral lesion located within skeletal muscle that mayspread to the skin as a painless nodule.We report a 82-years-old man who was seen with 7-month history of a rapidly growing and asynthomatic tumour of the left flank. Histologically thetumor showed a typical storiform pattern consisting of atypical histiocytic and fibroblastic cells. The cells were positive for vimentina, lisozima andCD68. These findings were compatible with a pleomorphic malignant fibrous histiocytoma.We review the clinical manifestations, histological findings and histogenesis of this tumour, and the different therapeutical options (AU)

Histiocitosis eruptiva generalizada en un varón de 53 años de edad / Generalized eruptive histiocytosis in a 53-years old male

La histiocitosis eruptiva generalizada es un cuadro clínico muy poco frecuente, incluída dentro de las histiocitosis de células distintas de las de Langerhans.Presentamos un caso recientemente visto en nuestro Servicio, planteando el diagnóstico diferencial con otras formas de histiocitosis, aunque losúltimos estudios sugieren que pueden tratarse de distintas entidades formando parte de un único espectro clínico (AU)

Generalized eruptive histiocytosis is a rare benign disorder, categorized into non-LCH histiocytosis. We present a case of this disease and provide thedifferential diagnosis with other forms of histiocytosis. Recent literature has suggested that generalized eruptive histiocytosis may be a part of a continuousspectrum of non-LCH histiocytic disorders (AU)

[Diagnostic agreement between primary care physicians and dermatologists in the health area of a referral hospital]. / Estudio de concordancia diagnóstica en Dermatología entre Atención Primaria y Especializada en el área de salud de un hospital de referencia.

INTRODUCTION: Skin diseases account for a high proportion of presenting complaints in primary health care. In Spain, the growing demand for consultations and the resulting longer waiting lists make it necessary to establish criteria for appropriate referrals to a specialist. This study aimed to investigate the characteristics of referrals from primary care centers to dermatology specialists as well as the correlation between the presenting complaint and the final dermatologic diagnosis. PATIENTS AND METHODS: We collected data from 3164 patients seen for the first time by dermatologists in our specialist service during 1998. Patients were stratified according to the referring primary health care center and the reason for referral. The agreement between the presenting complaint and the final dermatologic diagnosis was studied. For each dermatologic condition, the positive predictive value, diagnostic sensitivity, and k statistic were calculated. RESULTS: The overall diagnostic agreement was 65.52 %. Primary care physicians were found to over diagnose diseases caused by papillomavirus and the diagnostic sensitivity was very low for diseases such as basal cell carcinoma and seborrheic keratosis. CONCLUSIONS: It is necessary to insist on training primary care physicians, ensuring appropriate referral from primary health care clinics, and promoting an effective dialogue with the specialist.

Evolución cutánea atípica (tipo paquidérmico) de una mastocitosis / Atypical pachydermatous cutaneous course of mastocytosis

Presentamos el caso de un varón de 75 años que fue estudiado por primera vez en nuestro Servicio hace 32 años, por presentar unas lesiones papulosas eritemato-amarillentas generalizadas, junto con crisis de prurito, taquicardia y flushing. El diagnóstico propuesto a partir de este cuadro clínico y de la biopsia cutánea fue de urticaria pigmentosa. A lo largo de estos años el paciente ha sido revisado periódicamente, realizándose pruebas analíticas seriadas, biopsias cutáneas, estudios radiológicos y gammagráficos óseos y ultrasonografías hepáticas y esplénicas, sin hallazgos significativos. Los síntomas derivados de la liberación de mediadores han decrecido progresivamente. En una de sus últimas revisiones se le realizó un aspirado y biopsia de médula, apareciendo infiltrados multifocales de mastocitos típicos, CD117+, por lo que fue catalogado por el Servicio de Hematología en fase de mastocitosis sistémica indolente. Desde el punto de vista cutáneo queremos destacar los prominentes cambios que se han producido a lo largo de la evolución: la piel actualmente aparece engrosada, infiltrada, redundante y de color grisáceo, mostrando aspecto paquidérmico. Este es un tipo extremadamente raro de afectación cutánea en las mastocitosis, habiéndose descrito solamente un caso en la literatura

We describe the case of a 75-year-old man first seen in our department 32 years ago for generalized yellowish erythematous papular lesions along with an attack of pruritus, tachycardia, and flushing. A diagnosis of urticaria pigmentosa was proposed on the basis of these symptoms and the results of skin biopsy. Periodic follow-up in the intervening years included serial laboratory analyses, skin biopsy, radiological studies, bone scintigraphy, and ultrasound of the liver and spleen, with no remarkable findings. The symptoms caused by release of mediators decreased progressively. In one of the most recent visits, bone marrow aspirate and biopsy were performed, revealing multifocal infiltrates of typical CD117+ mast cells. Consequently, the hematology department diagnosed indolent systemic mastocytosis. A number of marked cutaneous changes were observed during the follow-up period: the skin currently appears thickened, indurated, redundant, and grayish, with a pachydermatous appearance. This represents an extremely rare form of cutaneous involvement in mastocytosis and only 1 case has been described in the literature

Diagnósticos dermatopatológicos en la población de 0 a 16 años (1997-2002) en un hospital español de tercer nivel / Dermatopathological diagnoses in population between 0-16 years (1997-2002) in a third level Spanish hospital

Presentamos una revisión de los diagnósticos histopatológicos obtenidos en nuestro Departamento de Dermatología del HCU “Lozano Blesa” deZaragoza, en la población entre 0 y 16 años, durante un periodo de 6 años (1997-2002).Los datos de cada paciente (nombre, edad, sexo y diagnóstico histopatológico) fueron registrados en una base de datos informatizada, y se estudiaronlos siguientes parámetros: distribución de los pacientes por edad y sexo, distribución de los diagnósticos histopatológicos según frecuencia y edad; yclasificación de las dermatosis en grupos específicos.De 1997 a 2002 se emitieron 14.042 diagnósticos histopatológicos, de los cuales 762 (5,4%) correspondieron a la población en estudio.Los grupos de patología más frecuentemente biopsiada de forma global fueron: tumores pigmentarios (70%), tumores cutáneos benignos (15,2%),lesiones traumáticas (4,2%), enfermedades de los anejos cutáneos (2,3%) y patología viral (1,8%).Estos diagnósticos histopatológicos se distribuyeron de forma variable en los diferentes grupos de edad, predominando en el primer año de vida lasenfermedades de los anejos cutáneos (65,2%); los tumores cutáneos benignos, de 1 a 6 años (24%); y, posteriormente, los tumores pigmentarios conunos porcentajes crecientes con la edad

Patients between 0 and 16 years requiring histopathologic study in our Deparment of Dermatology of the CUH Hospital Lozano Blesa in Zaragoza, fromjanuary 1997 to december 2002 were registered where 14,042 biopsies were performed. A total number of 762 pediatric patients were studied duringthis survey period (301 male and 461 female).The diagnoses five more frequent a decrease order included: pigmentary tumours (70%), benign tumours (15.2%), dermatosis with traumatic origin(4.2%), diseases of cutaneous annexes (2.4%) and viral infections (1.8%).These histopathologic diagnoses changed in the differents groups of age. In the first year of life, the annexes cutaneous disease was the most frequentgroups of disease (65.2%), cutaneous benign tumours, in children between 1 to 6 years (24%) and, pigmentary tumours in teenager

[Atypical pachydermatous cutaneous course of mastocytosis]. / Evolución cutánea atípica (tipo paquidérmico) de una mastocitosis.

We describe the case of a 75-year-old man first seen in our department 32 years ago for generalized yellowish erythematous papular lesions along with an attack of pruritus, tachycardia, and flushing. A diagnosis of urticaria pigmentosa was proposed on the basis of these symptoms and the results of skin biopsy. Periodic follow-up in the intervening years included serial laboratory analyses, skin biopsy, radiological studies, bone scintigraphy, and ultrasound of the liver and spleen, with no remarkable findings. The symptoms caused by release of mediators decreased progressively. In one of the most recent visits, bone marrow aspirate and biopsy were performed, revealing multifocal infiltrates of typical CD117+ mast cells. Consequently, the hematology department diagnosed indolent systemic mastocytosis. A number of marked cutaneous changes were observed during the follow-up period: the skin currently appears thickened, indurated, redundant, and grayish, with a pachydermatous appearance. This represents an extremely rare form of cutaneous involvement in mastocytosis and only 1 case has been described in the literature.

Foreign body post-varicella granulomas due to talc.

Foreign body granulomas in the skin have been described frequently and may have various causes. Diagnosis is relatively easy in most cases, as most are related to foreign material penetrating into the skin due to different kinds of traumas. We present the case of a boy with multiple facial granulomas, persisting more than 6 months and related to the use of topical anti-pruriginous talc powder applied to papuloexudative lesions caused by varicella. Data gathered in the anamnesis, examination with polarized light and images seen through the electron microscope enabled us to identify the causal agent talc as the inducer of the granulomatous lesions that the patient presented, discarding other types of foreign body granulomas of exogenous origin.

Recurrent lentigo maligna as amelanotic lentigo maligna melanoma.

Amelanotic lentigo maligna and lentigo maligna melanoma are extremely rare tumours. Even rarer is a recurrent amelanotic lentigo maligna or amelanotic lentigo maligna melanoma at the site of a previously removed pigmented lentigo maligna. We describe two cases of recurrent amelanotic lentigo maligna melanoma manifesting as erythematous plaques evolved from previously excised pigmented lentigo maligna.

Penfigo crónico familiar benigno (enfermedad de Hailey-Hailey). A propósito de un caso / Familial benign chronic pemphigus (Hailey-Hailey disease). Report of a case

La enfermedad de Hailey-Hailey (pénfigo crónico familiar benigno) es una dermatosis ampollosa, de evolución crónica, recidivante, autosómica dominante, de penetrancia y expresividad variables, localizada predominantemente en los pliegues. Presentamos el caso de una mujer de 58 años de edad que presentaba una erupción cutánea pruriginosa, eccematosa, localizada en las axilas e ingles, en forma de brotes recurrentes de 36 años de evolución. La biopsia cutánea reveló una intensa acantólisis que daba lugar a una ampolla intraepidérmica suprabasal. Se instauró tratamiento con antibióticos orales y corticoides y antibióticos tópicos, con desaparición de las lesiones pocos días después. Queremos destacar la buena respuesta de nuestra paciente al tratamiento instaurado. Hailey-Hailey disease (familial benign chronic) is an autosomal dominant hereditary bullous disorder characterized by recurrent lesions predominating in folds with variable penetrance and expressisivity (AU)

Penfigoide ampolloso producido por captopril / Bullous pemphigoid associated with captopril

Presentamos el caso de un varón de 82 años que, tras iniciar un tratamiento con captopril, desarrolló una erupción cutánea ampollosa cuyos estudio histopatológico e inmunofluorescencia fueron compatibles con penfigoide ampolloso. Tras suspender el tratamiento con captopril e instaurar un tratamiento con corticoides, la afección desapareció (AU)

Visceral leishmaniasis with cutaneous lesions in a patient infected with human immunodeficiency virus.

We report a case of visceral leishmaniasis (VL) with cutaneous lesions in a patient infected with human immunodeficiency virus (HIV). The cutaneous lesions consisted of erythematous papules on the legs. Biopsy of one lesion showed abundant Leishmania amastigotes within epithelial cells of an eccrine sweat gland in the dermis. Leishmania organisms were also found in a blood smear. Rapid and complete clearance of the cutaneous lesions was achieved after antimony therapy. Cutaneous lesions in VL are being reported increasingly frequently in patients with HIV infection and their significance remains in discussion.

[Dermatomycosis in plaques caused by Botryomyces caespitosus. A new causative agent]. / Dermatomicosis en placa por Botryomyces caespitosus. Nuevo agente causal.

The clinical and histological features of a dermomycosis caused by a new dematiaceum fungus are described. The mycological characteristics of this fungus individualize and differentiate it from other demiataceum hyphomycetes, proposing the name of Botryomyces caespitosus for it. The submicroscopic features are commented, as well.

[Infantile and progressive papular mucinosis]. / Mucinosis papulosa infantil y progresiva.

We comment the histologic and clinical features of a process studied in a 2-year old patient with neither clinical nor laboratory evidence of any other disease, being characterized by the existence of multiple papules, grouped but not confluent, asymptomatic, skin-coloured or slightly erythematous and located on the lumbar area. The outset of the lesions occurred at birth and their number has increased all through the first two years of file. The histologic image is consistent with mucin focus in the middle dermis, relatively well limited but without a perilesional capsule, associated to a fibroblast proliferation. The case is considered to be a reactive modification of fibroblasts with an alteration of their secretory capacity, which causes an increase of mucin with a decrease in the connective fibrillar components. The differences of this process with other primary or secondary cutaneous mucinosis especially with focal mucinosis are discussed. The non-specificity of the clinical features, makes it necessary to carry out the microscopic examination to get the correct diagnosis, being the surgical excision of the lesion the treatment of choice.