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Spiny keratoderma is a rare condition that presents as many small, firm hyperkeratotic papules on the palms and soles. This condition can be familial, typically arising in adolescence or young adulthood. However, if the condition arises later in adulthood, it is more frequently associated with internal malignancy. Therefore, a thorough workup is required to identify a potential underlying cancer. It is rare for spiny keratoderma to spontaneously arise in individuals in adulthood and yet not be associated with systemic disease, but such is the scenario for our patient.
RESUMO
Papular epidermal nevus with "skyline" basal cell layer (PENS) is a very rare type of keratinocytic nevus and is associated with extracutaneous findings such as neurological symptoms in about 50% of the cases. Therefore, it is also referred to as PENS syndrome. Clinically visible hyperkeratotic papules and plaques already appear at birth or shortly thereafter, while neurological symptoms such as epilepsy and mental retardation manifest themselves during childhood. Genetics suggests gonadal mosaicism as a possible cause for the disease. Another hypothesis is that genetic mutation can occur in a mendelian trait or through a paradominant inheritance.
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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.
RESUMO
@#<p style="text-align: justify;">Arsenic is a known human carcinogen and skin manifestations are the earliest and most specific markers of chronic arsenic poisoning. A 43-year-old man from Luzon presented at the Section of Dermatology with a one-year history of hyperkeratotic papules and plaques on the palms and soles. Numerous round hypopigmented macules were scattered on the upper back. Initial 24-hour urine arsenic level was elevated at 288mcg/liter. The patient underwent successful chelation with N-acetylpenicillamine and the palmoplantar keratoses were treated with cryotherapy and topical 20% salicylic acid in white petrolatum. In cooperation with the Department of Health, a comprehensive health and environmental assessment was conducted in the affected communities. This case highlights the role of dermatologists in the diagnosis and management of this public health problem.</p>