[Brauer-Buschke-Fischer keratoderma palmo-plantare punctata. Brauer's keratoderma dissipatum hereditarium...

Keratosis punctata palmaris et plantaris (KPPP) of Buschke-Fischer-Brauer is an autosomal dominant genodermatosis characterized by disseminated cup-shaped horny papules with central depression containing keratotic plugs. The disease is circumscribed on the palms and soles; associated abnormalities are unusual. The microscopic findings show normal structure of the epidermis with massive orthokeratotic hyperkeratosis, hypergranulosis and acanthosis; dermis is slightly compressed below the epidermis level by the keratotic plugs and free of inflammatory infiltrates. We describe a female of 37 years old (proband) affected by KPPP and her familiar pedigree showing that dermatosis is dominantly inherited. The clinical features of KPPP are like the first reports of Brauer (keratoderma dissipatum hereditarium palmoplantare). The criteria used to diagnose and classify the hereditary palmoplantar keratoderma are also discussed.