Vesicular lesions in a patient with lipoid proteinosis: a probable acantholytic dermatosis.

Lipoid proteinosis is a rare genodermatosis with characteristic clinical and histologic findings later in life. We present a case of lipoid proteinosis in which the diagnosis was originally missed because the patient presented in childhood with vesicles and a hoarse voice and lacked any other manifestations. Microscopic examination of a vesicle revealed extensive non-dyskeratotic acantholysis. Vesiculo-bullous lesions have been described clinically in lipoid proteinosis, but we are unaware of any histopathologic description regarding bullae formation. Although only a single patient is herein described, it seems unlikely that this type of acantholysis is purely coincidental. It is proposed that lipoid proteinosis should be in the differential of acantholytic vesiculo-bullous lesions in a child.