Epidermolysis bullosa pruriginosa affecting 3 successive generations

A 24-year-old man presented with multiple mildly itchy flesh-colored papules and plaques on both legs for the past decade. The lesions were preceded by transient vesicles that contained clear fluid. The papules and plaques used to develop on sites where vesicles had healed. Many family members in three generations had similar lesions (Figure 1). On examination, multiple discrete flesh-colored papules and plaques were found on both lower extremities, extending from the feet up to the knees (Figures 2 and 3). A few of the plaques were excoriated. No vesicles or bullae were noted, and the skin in between the lesions appeared normal. The nail of left great toe was discolored and dystrophic. The rest of the mucocutaneous examination was unremarkable. Bullous lichen planus, Neckam's disease, lichenoid amyloidosis, and epidermolysis bullosa pruriginosa (EBP) were considered as differential diagnoses. Histopathology from the plaque showed a subepidermal cleft with no inflammatory cells. The epidermis was acanthotic at places, and the dermis appeared normal (Figure 4a and 4b). Based on clinical presentation and histopathology, a diagnosis of EBP was made.