Acroqueratoelastoidosis de Costa / Acrokeratoelastoidosis of Costa

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.

Acroqueratoelastoidosis: reporte de un caso clínico / Acrokeratoelastoidosis: reportof a clinical case

Resumen La acroqueratoelastoidosis es una rara genodermatosis de herencia autosómica dominante, familiar o esporádica, siendo esta última la forma más frecuentemente reportada en la literatura. Fue descripta por el dermatólogo de origen brasileño Oswaldo Costa en el año 1953. Así las acroqueratodermias marginales son un subgrupo de queratodermiaspalmoplantares caracterizadas por la presencia de pápulas y placas queratósicas, con una disposición lineal, que asientan sobre el margen de transición entre la piel dorsal y palmar o plantar.Suelen iniciarse en la infancia, en la adolescencia o en la vida adulta temprana y tienen un curso crónico. Su diagnóstico diferencial con el resto de las acroqueratodermias es un gran desafío, siendo el hallazgo histológico de elastorrexis lo primordial para su correcto diagnóstico. Presentamos el caso de una mujer de 22 años con un cuadro compatible clínica e histopatológicamente con Acroqueratoelastoidosis.

Abstract Acrokeratolastoidosis is a rare genodermatosis of dominant autosomal, familial or sporadic inheritance, the latter being the most frequently reported form in the literature. It was described by the Brazilian dermatologist Oswaldo Costa in 1953. It is characterized by the presence of multiple hyperkeratotic papules, usually asymptomatic, located in the marginal area of the hands, feet or both. It usually begins in childhood, adolescence or early adult life and has a chronic course. Its differential diagnosis with the rest of the acrokeratosis is a great challenge, being the histological finding of elastorrexis the primary for its correct diagnosis. We present the case of a 22-year-old woman with a clinical and histopathology compatible with Acroqueratoelastoidosis.

Acroqueratoelastoidosis de costa: presentación de un caso con lesiones en pabellón auricular / Coastal acrokeratoelastoidosis: presentation of a case with lesions in both auricular pavilions

La acroqueratoelastoidosis de Costa es una genodermatosis rara, caracterizada por la presencia de múltiples pápulas queratósicas, pequeñas y firmes, en los márgenes laterales de palmas y plantas. Es-tas lesiones estacionarias y asintomáticas aparecen generalmente en la pubertad. Se desconoce su prevalencia. Es de herencia autosómica dominante con expresividad variable. La histología combina hiperqueratosis y acantosis, sin embargo, el hallazgo más importante es la elastorrexis. No se aconseja tratamiento en la mayoría de los pacienes. Se reporta el caso de una paciente de 61 años, sin antecedentes familiares de esta condición, que consulta por aparición en la adolescencia de lesiones hiperqueratósicas asintomáticas en márgenes laterales de ambas manos y lesiones similares en ambos pabellones auriculares.

Acrokeratoelastoidosis is a rare genodermatosis, characterized by the presence of multiple kerato-tic papules, small and firm, on the lateral margins of palms and soles. These stationary and asymptomatic lesions usually appear at puberty. Its pre-valence is unknown. It is of autosomal dominant inheritance with variable expressivity. Histology combines hyperkeratosis and acanthosis, howe-ver, the most important finding is elastorhexis. Treatment is not advised in most patients. We report the case of a 61-year-old female patient, with no family history of this condition, who consulted for appearance in adolescence of as-ymptomatic hyperkeratotic lesions in the lateral margins of both hands and similar lesions in both auricular pavilions.

Acroqueratoelastoidosis: Presentación de un caso caracterizado por ausencia de fibras elásticas / Acrokeratoelastoidosis: presentation of a case characterized by absence of elastic fibers

En el año 1953, Costa describe un cuadro clínico caracterizado por la aparición de pápulas hiperqueratóticas en márgenes laterales de manos y pies, que se asociaba en el examen histológico a cambios epidérmicos (hiperortoqueratosis y acantosis) y particularmente a la reducción y/o fragmentación de fibras elásticas, motivo por el cual esta entidad, considerada actualmente una genodermatosis, recibió el nombre de acroqueratoelastoidosis (AQE). Presentamos un caso en el que destaca la ausencia de fibras elásticas (AU)

In 1953, Costa described a clinical entity characterized by the appearance of hyperkeratotic papules on the lateral margins of the hands and feet, which was associated with epidermal changes (hyperortokeratosis and acanthosis) and to the reduction and /or fragmentation of elastic fibers at histological examination. This entity was named acrokeratoelastoidosis and is currently considered to be a genodermatosis. A case characterized by the absence of elastic fibers is reported.

A Case of Keratoelastoidosis Marginalis / 대한피부과학회지

Keratoelastoidosis marginalis is a rare skin disorder. Clinically it consists of small, firm, linear hyperkeratotic papules, characteristically along the margin of the hands. Histopathologically it shows hyperkeratosis, acanthosis, solar elastosis, and degeneration of collagen and elastic fibers. Long-term ultraviolet radiation exposure and chronic trauma are considered to be precipitating factors. Herein, we report a case of keratoelastoidosis marginalis and a review of the related literature.

A Case of Acrokeratoelastoidosis / 대한피부과학회지

Acrokeratoelastoidosis (AKE) is a rare skin disorder initially described by Costa, which is inherited by autosomal dominant, but also may be sporadic. Clinically, it consists of small, firm papules with occasional keratosis or umbilication, characteristically along the margins of hands and feet. Histopathologically, it shows hyperkeratosis, acanthosis in the epidermis and fragmentation and rarefaction of elastic fibers-elastorrhexis-in the dermis. A 32-year-old man presented with multiple papules along the border of the hands and feet, and from histopathology, AKE was diagnosed. Herein we report a case of acrokeratoelastoidosis and review the clinical and histopathologic features, etiology, differential diagnosis and treatment.

A Case of Acrokeratoelastoidosis

A 28-year-old woman had a 13-month history of skin-colored, well-circumscribed, hyperkeratotic plaques on both heels. Histological examination showed hyperkeratosis and acanthosis in the epidermis. Special stain for elastic tissue revealed marked fragmentation, diminution and thickening of elastic fibers in the dermis. We report a rare case with the lesions of acrokeratoelastoidosis on both heels.

Four Cases of Acrokeratoelastoidosis / 대한피부과학회지

Four cases of acrokeratoelastoidosis are reported. All four patients had symmetrically situated asymptomatic keratotic plaques along the margin of first interdigital space of the hand, The patients were all elderly korean farmers. Elastic stain showed aggregated thickened black elastotic materials occupying mainly the upper dermis. Epidermis showed marked hyperkeratosis and acanthosis. Although this cutaneous condition is not well known to physicians in the country, we think it may be not an uncommon condition in the rural part of Korea.