Peña J, Navarrete FG, Márquez PCE

Language: Spanish
References: 22
Page: 163-169
PDF size: 223.90 Kb.

ABSTRACT

Hailey - Hailey disease (Familial Benign Chronic Pemphigus) is an autosomal dominant genodermatosis, first described by brothers Hailey in 1939, most commonly presents in the second to fourth decade of life with skin fragility and uncomfortable lesions in the flexures or at the sites of trauma. These lesions in the flexures or at the sites of trauma. These lesions begin as clear vesicle, bullaes or crust that may be misdiagnoses as eczema or infections with bacteria or fungi. Histologically there is characteristic lesional suprabasal acantholysis, and ultrastructurally there is a breakdown of desmosome – keratin filament complexes in established inherited skin disease is unclear, however some authors investigate that there is a gen that codifies for a Calcium –Dependent Pump and its explains part of the disease. Many treatments have been suggested in the literature but none is specific or consistently effective.

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