ABSTRACT
La acroqueratoelastoidosis de Costa es una genodermatosisde herencia autosónimica dominante con penetrancia incompleta. Es un trastorno de las fibras elásticas exclusivamente cutáneo y a nivel acral. Se caracteriza por la presencia de pápulas eritematosas, amarillentas o del color de la piel normal en la cara marginal de los dedos de las manos o de los pies, que se agrupan, forman placas con aspecto de empedrado y se extienden de forma simétrica al dorso, las palmas y las plantas. El hallazgo histológico patognomónico es la elastorrexis. Se presenta el caso de un niño de 7 años con una acroqueratoelastoidosis que comprometía las manos y los pies, asociada a prurito.
Acrokeratoelastoidosis of Costa is a genodermatosis of autosomal dominant inheritance with incomplete penetrance.It is a cutaneous disorder of the elastic fibers at the acral level.The clinical feature is the presence of erythematous, yellowish or normal skin-colored papules on the marginal aspect of the fingers and/or toes, which agminate to form cobblestone-like plaques and extend symmetrically to the dorsum and palms and soles. The pathognomonic histologic finding is elastorrhexis. We present a 7-year-old boy with acrokeratoelastoidosis involving hands and feet, associated with pruritus.
Subject(s)
Humans , Male , Child , Skin Diseases, Genetic/diagnosis , Skin Diseases, Genetic/pathology , Keratoderma, Palmoplantar , Elastic Tissue/abnormalitiesABSTRACT
Focal acral hyperkeratosis is a rare skin disorder, initially described by Dowd et al., which is sporadic or inherited in an autosomal dominant way. Clinically, the condition presents with small yellowish to white papules located on lateral aspects of the hands and feet. Histopathological alterations are limited to the epidermis and there are no changes in the elastic fibers of the dermis. In this case report, a 14-year-old girl had a two-year history of persistent, asymptomatic, multiple papules along the border of the hands and feet. A histologic section of papules from the left hand and foot showed marked hyperkeratosis and hypergranulosis of epidermis.