Résumé
La morfea profunda solitaria es una forma infrecuente de morfea localizada en la infancia. Se caracteriza clínicamente por la presencia de un área indurada, mal delimitada y asintomática, de localización paraespinal, que no tiende a la progresión ni se asocia con manifestaciones sistémicas. Histológicamente, muestra un engrasamiento e hialinización de las fibras de colágeno dérmico, junto con infiltrados de predominio linfohistiocitario en la dermis reticular y la hipodermis. Se presenta a una paciente de 7 años con una lesión solitaria en la región dorsal media izquierda, cuyos hallazgos clínicos, ecográficos e histológicos fueron compatibles con el diagnóstico de una morfea profunda solitaria.
Solitary morphea profunda is an unusual form of localized scleroderma in childhood. It is characterized by a single, poorly defined and indurate plaque often located on the upper trunk near the spine. Solitary morphea profunda is frequently asymptomatic and shows no associated systemic involvement. Histological examination reveals dense sclerosis of collagen and a marked lymphocytic infiltrate in the reticular dermis and subcutis. We report a 7-year-old girl with a solitary asymptomatic and sclerotic plaque on the back. Clinical, ultrasonographic and histological features were consistent with solitary morphea profunda.
Sujets)
Humains , Femelle , Enfant , Pédiatrie , Sclérodermie localisée , Échographie , CollagèneRésumé
A 4-year-old boy has had a solitary sclerotic depressed plaque on the right anterior chest since birth. The histopathologic findings are consistent with morphea profunda: thickening, hyalinization, and homogenization of collagen bundles in the dermis and subcutaneous tissues, admixture with a prominent lymphocytic and plasma cell infiltrate, and sweat glands en-trapped between the thickened collagen bundles. We report a case of congenital solitary morphea profunda.
Sujets)
Enfant d'âge préscolaire , Humains , Mâle , Collagène , Derme , Substance hyaline , Parturition , Plasmocytes , Sclérodermie localisée , Tissu sous-cutané , Glandes sudoripares , ThoraxRésumé
Solitary morphea profunda is a rare form of scleroderma, characterized clinically by a solitary sclerotic plaque, and histologically by marked dermal and subcutaneous fibrosis with an inflammatory infiltrate. We describe another case of this entity presented with an ulcerative, indurated plaque on the left iliac crest, which histologically revealed a focal incidental acantholysis in the overlying epidermis and a marked eosinophilic infiltration through the dermis to the sub-cutaneous tissue.