Acquired perforating dermatosis: clinicopathological study of 31 cases, emphasizing pathogenesis and treatment.

BACKGROUND: Acquired perforating dermatosis (APD) is a rare group of skin disorders of unknown aetiology and pathogenesis and is associated with several systemic diseases. OBJECTIVE: We review the clinicopathological features, associated systemic diseases and treatment response in a series of APD patients. METHODS: We conducted a retrospective observational study of all patients histologically diagnosed with APD in Hospital San Jorge (Huesca, Spain) between 2002 and 2014. Demographic and clinical features were collected from medical records. Statistical analyses were carried out using SPSS software (version 20.0; IBM Corp, Armonk, NY, USA). RESULTS: The study population consisted of 31 patients (19 women and 12 men), with a mean age of 54 years. Reactive perforating collagenosis (n = 15, 45%) was the most common histopathologic type. The most frequently affected area was the lower limbs (66.6%, n = 22), and pruritus was present in 19 patients (61.3%, P = 0.005). Arterial hypertension (30.3%) was the most common associated condition. Five patients were receiving treatment with infliximab when diagnosed with APD. Most patients responded to topical steroids and oral antihistamines (n = 15). LIMITATIONS: This study is retrospective, and the sample size is limited. CONCLUSION: Acquired perforating dermatosis is an underdiagnosed dermatosis frequently associated with systemic disorders. Its pathogenesis may involve vascular damage, not only in patients with diabetes, but also in those with arterial hypertension and chronic venous insufficiency. Control of pruritus and underlying extracutaneous disorders, as well as discontinuation of the treatment with biologics, are important aspects of the management of this dermatosis.

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)

El melanoma subungueal: un retodiagnóstico / Subungueal melanoma: a challenge for the diagnosis

El melanoma subungueal sufre con frecuencia un importante retraso en el diagnóstico.Presentamos el caso de una paciente con cambios ungueales distróficos que fueron diagnosticados en nuestro Servicio como onicomicosis, debido a laclínica y al cultivo de hongos positivo, pero que, al realizar una biopsia, dada la mala respuesta al tratamiento antifúngico, mostró una histología demelanoma lentiginoso acral con avanzada invasión local.En el siguiente artículo se revisan las principales características clínicas que pueden orientarnos hacia el diagnóstico precoz del melanoma subungueal,así como la utilidad de la dermatoscopia en el diagnóstico de esta neoplasia; siempre teniendo en cuenta que el diagnóstico definitivo se establece pormedio de la biopsia (AU)

Diagnosis of subungual melanoma is usually difficult and often is a delayed diagnosis. A patient with nail dystrophy that was diagnosed as a onychomicosisby means of mycological culture is reported. The patient don´t responded to the treatment during three weeks, so, we made a biopsy that showedthe diagnosis of acral lentiginous melanoma.In this article, we review the world literature on subungual melanoma and arranged the available information in a system for the clinical detection ofsubungual melanoma. We believe that dermoscopic examination of the nail plate in cases of melanonychia provides useful information that couldhelp to decide if a nail apparatus biopsy should be performed; however, the absolute diagnosis of subungual melanoma is made by means of a biopsy (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)

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.

[Recurrent ingestion of foreign bodies. Unusual presentation of Munchausen by Proxy syndrome]. / Ingestión reiterada de cuerpos extraños. Forma inusual de presentación del síndrome de Munchausen por poderes.

A case with unusual presentation of Munchausen syndrome by proxy is reported. It was shown through reiterated ingestion of foreign bodies (two earrings, probably a button but not confirmed, a 5 cm. screw and two sewing needles) perpetrated by a 22-year-old mother with a psychiatric record of behavior disorders and family dysfunctions on a 10-month-old infant with a history of prematurity, repeated visits to emergency rooms, and nonjustified multiple hospital admissions. The difficulties in the diagnosis and the need for an early detection are brought up, making considerations in regard to indicators, diagnostic criteria, and management procedure.

[Children at risk. Our experience]. / Niños de riesgo. Nuestra experiencia.

Seventy children in a situation of risk, evaluated and treated in the Social Work Unit of the Children's Hospital "Virgen del Rocío" over a period of 22 months, were analyzed. Of these children, 89% corresponded to low-middle, low or very low social classes. The most common problems included those of the family (77.1%) with history of maltreatment and unwanted pregnancies being the most prevalent problems. In second place was the personal pathology of the parents (74.2%), with mental disorders and alcoholism being the main causes, followed by drug addiction and delinquency. Social factors (57.1%), which included predominantly unemployment and illiteracy, were the next most common finding. The final factor was the personal pathology of the child (10%). The consequences of the risk situation are described, emphasizing the psychic and treatment carried out. Final comments are made underlining the importance of awareness and professional training, multiprofessional teams, means of detecting and intervention into the problems, manpower and material as well as how to carry out studies in this area.