Subject(s)
Hair Diseases/diagnosis , Hair Diseases/genetics , Hair/ultrastructure , Microscopy, Electron, Scanning , Child , Female , Humans , Rare DiseasesABSTRACT
Objetivo: Determinar los casos de liquen plano en edades pediátricas que acudieron al servicio de Dermatología del Hospital Universitario de Caracas entre 1989 y 2003. Métodos: Estudio analítico retrospectivo clínico de una muestra de pacientes pediátricos con diagnóstico de liquen plano a partir de los archivos del laboratorio de histopatología. Las variables analizadas fueron edad, sexo, diagnóstico clínico, distribución y morfología de las lesiones, y evolución. Resultados: Se encontró un total de 58 casos de liquen plano en niños entre 18 meses y 12 años. Hubo un predominio del sexo femenino (58%). La edad promedio de presentación fue de ocho años. La mayoría de los pacientes presentó la variante de liquen plano clásico. La localización más común fue en los miembros inferiores. Sólo en un caso se observó compromiso mucoso. Conclusiones: Las características halladas en los pacientes estudiados concuerdan en forma general con lo reportado en la literatura (AU)
Objective:To identify cases of lichen planus in pediatric patients who attended the service of Dermatology at Hospital Universitario de Caracas from 1989 to 2003. Methods: A retrospective analytic study of a sample of pediatric patients with a diagnosis of lichen planus from the archives of the histopathology.The variables studied were age, sex, clinical diagnosis, distribution and morphology of lesions, and evolution. Results:We found a total of 58 cases of lichen planus in children between 18 months and 12 years of age. There was a predominance of females (58%).The average age of presentation was eight years. Most of the patients had classical lichen planus.The most common location was on the lower limbs. Mucosal involvement was observed in only one case. Conclusions:The features found in the patients studied where consistent with those previously reported in the literature (AU)
Subject(s)
Humans , Male , Female , Infant , Child, Preschool , Child , Venezuela , Lichen Planus , Leg DermatosesABSTRACT
Bullous pemphigoid (BP) is a very rare acquired immunbullous disease in children and infants. We report the case of a 15-month-old boy referred to our service with multiple tense bullae located predominantly on the trunk, neck and proximal portion of the limbs. Palms, soles and oral mucosa were also affected. Histopathologic and immunopathologic features were characterized with bullous pemphigoid. The patient responded well to systemic steroids, with improvement of his condition. After a 10-year follow up the patient was no longer suffering from the disease.
Subject(s)
Pemphigoid, Bullous/pathology , Skin/pathology , Child , Child, Preschool , Fluorescent Antibody Technique, Direct , Follow-Up Studies , Humans , Immunoglobulin G/immunology , Infant , Male , Skin/immunology , Time FactorsABSTRACT
Two native Yanomami children from the Venezuelan Amazonia with erythroderma were hospitalized on our service. Clinical, histologic, and immunofluorescence studies diagnosed endemic pemphigus foliaceous. Human leukocyte antigen class II showed DRB1*04 subtype *0411, which has not been previously associated with this disease. However, it shares a common epitope with all the human leukocyte antigen DRB1 alleles that have been involved in this disease among Brazilian populations. Although this condition is endemic in Brazil, our patients are the first two reported in Venezuela.
Subject(s)
Pemphigus/diagnosis , Adolescent , Child , Chloroquine/therapeutic use , Diagnosis, Differential , Female , Humans , Male , Pemphigus/drug therapy , Prednisone/therapeutic use , VenezuelaABSTRACT
We report a 10-month-old girl who, at 4 months, developed a small, reddish, plaquelike lesion on her tongue. This lesion began to enlarge rapidly, resulting in difficulty in swallowing and breathing. On physical examination, there was a large, red, friable, hard in consistency, irregular tumor over the distal portion of her tongue. The histopathology and immunohistochemical findings were consistent with an embryonal rhabdomyosarcoma. The patient was assigned clinical stage I, group III, and began treatment according to the Intergroup Rhabdomyosarcoma Study guidelines, on triple-agent chemotherapy consisting of vincristine, actinomycin D, and cyclophosphamide. She only received four cycles because her parents then refused this treatment. After the tumor size was reduced by chemotherapy, a partial anterior glossectomy was performed. After 30 months of follow-up she has had no recurrences.
Subject(s)
Rhabdomyosarcoma, Embryonal/pathology , Tongue Neoplasms/pathology , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Combined Modality Therapy , Cyclophosphamide/administration & dosage , Dactinomycin/administration & dosage , Female , Humans , Infant , Photomicrography , Rhabdomyosarcoma, Embryonal/drug therapy , Rhabdomyosarcoma, Embryonal/surgery , Tongue Neoplasms/drug therapy , Tongue Neoplasms/surgery , Vincristine/administration & dosageABSTRACT
We describe an 11-year-old girl with a persistent pruritic papular eruption on the vulva. Clinically, the lesions consisted of whitish papules and erosions located on the inner aspect of the labia majora. There was no familial history of skin diseases. Histologically, a biopsy specimen showed difuse hyperkeratosis, parakeratosis, acantholysis throughout the thickness of the epidermis, and the presence of corps ronds. Those findings were consistent with a diagnosis of acantholytic dyskeratosis. At 3 years follow-up, only isolated hyperkeratotic, asymptomatic papules on the same location remained. The occurrence of this focal and sporadic, localized form of acantholytic dyskeratosis seems to be rare in the pediatric population, as we could find no other child with this entity reported in the literature.
Subject(s)
Acantholysis/diagnosis , Keratosis/diagnosis , Vulvar Diseases/diagnosis , Acantholysis/pathology , Biopsy , Child , Diagnosis, Differential , Female , Humans , Keratosis/pathology , Vulvar Diseases/pathologyABSTRACT
La poroqueratosis es una entidad clínica que se manifiesta por el desarrollo de lesiones queratósicas en la piel con un resultado histológico característico, la presencia en la epidermis de una columna de células paraqueratósicas denominada laminilla cornoide. Clínicamente se han descrito cinco formas clínicas de la enfermedad. El mecanismo de transmisión descrito en la mayoría de los casos es autosómico dominante, aunque estudios familiares sugieren que puede tratarse de un mismo trastorno genético con diferente expresividad clínica. En vista de lo poco frecuente de esta enfermedad se decide presentar el siguiente caso
Subject(s)
Humans , Female , Aged , Epidermis , Hyperkeratosis, Epidermolytic , Porokeratosis , VenezuelaABSTRACT
Con la finalidad de determinar la frecuencia y tiempo de aparición de lesiones mucocutáneas en los pacientes con pénfigo vulgar (PV), se realizó un estudio retrospectivo en el cual se revisaron las historias clínicas de 39 pacientes con diagnóstico clínico e histoinmunopatológico de PV efectuado en el Servicio de Dermatología del Hospital Universitario de Caracas. En los resultados se observó una mayor frecuencia de lesiones en la mucosa oral como sitio de inicio de la enfermedad. De éstos, más del 50 por ciento progresaron a piel en los primeros 6 meses, siendo este período de tiempo indicador pronóstico importante. Concluimos que la afectación de la mucosa oral es un hallazgo frecuente en el PV, debiéndose tener presente para su diagnóstico y tratamiento
