Neşe GÖÇER GÜROK, Savaş ÖZTÜRK, Ahmet KILIÇARSLAN

Nadir Bir Olgu; Fokal Akral Hiperkeratoz

Fokal akral hiperkeratoz (FAH) nadir görülen bir genodermatozdur. FAH genellikle yaşamın ikinci veya üçüncü dekatlarında ortaya çıkan; avuç içi ve ayak tabanlarının anormal kalınlaşması ile karakterize, heterojen bir hastalık grubu olan palmoplantar keratodermanın bir türüdür (1). Otozomal dominant kalı- tılmakla birlikte sporadik de olabilir. Costa’nın Akrokeratoelastoidozisi’nin (AKE) nadir bir varyantı olarak da bilinir (2). Etiyolojisi tam olarak bilinmez ve etkin bir tedavi seçeneği yoktur. Burada oldukça nadir görülmesi ve AKE ile sık karıştırılması nedeniyle yeni tanı almış 43 yaşındaki FAH olgusu güncel literatür bilgileriyle sunulmuştur.

Anahtar Kelimeler:

Autosomal dominant inheritance, focal acral hyperkeratosis, palmoplantar keratoderma

A Rare Case; Focal Acral Hyperkeratosis

Focal acral hyperkeratosis (FAH) is a rare genodermatosis. FAH is usually observed in the second or third decades of life, and it is a type of palmoplantar keratoderma, a heterogeneous group of diseases characterized by abnormal incrassation of the palms and soles. Although it is an autosomal dominant inher- itance, it could also be sporadic. It is also known as a rare variant of Costa’s Acrokeratoelastoidosis (AKE). Its etiology is not known clearly and there is no effective treatment. In the present study, a 43-year-old recently diagnosed FAH case is presented due to its rarity and frequent confusion with AKE based on current literature.

Keywords:

Autosomal dominant inheritance, focal acral hyperkeratosis, palmoplantar keratoderma.,

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