Epidermolysis bullosa pruriginosa a clinico-pathological study in an index case, highlighting its affliction in 11 of 27 member of the family

Abstract

Epidermolysis bullosa pruriginosa, a rare variant of dominant inheritance pattern, characterized by itchy, papules and/or nodules coalescing to form plaque(s), studded by blisters, is describe in a young man an index case. In all 12 afflicted individuals of the 27 family members was interesting, the diagnosis of which was made by its morphology and inflammatory changes in the epidermis, comprising hyperkeratosis, moderate acanthosis, presence of inflammatory cells, and above all inflammatory cell containing sub-epidermal cleavage or cleft.

Keywords

• Epidermolysis bullosa
• Hallopeau-Siemens
• Dystrophic epidermolysis bullosa