Familial benign chronic pemphigus (Hailey-Hailey disease)

Abstract

SUMMARY: Familial benign chronic pemphigus (Hailey–Hailey disease) is a blistering dermatosis, which is inherited as an autosomal dominant trait and usually presents around the third and fourth decades. Painful erosions, vesico-pustules and scaly erythematous plaques appear at sites of friction such as the sides of the neck, the axillae, the groins and the perineum. A case of familial benign chronic pemphigus in a 54 year-old woman is presented. The disease started at the age of 35. The areas of predilection are the axillary and submammary folds and genital area. Erythematous, macerated plaques were found with multiple painful fissures, vesicles and crusts in its periphery. The diagnosis was established on the basis of physical examination and was confirmed by histological examination of a skin biopsy. A suprabasal cell separation (acantholysis) of the epidermis was found. A family history is present. An autosomal dominant mode of inheritance was confirmed by genealogical analysis. Thirteen members have been affected by the same disease. In the presented case topical and systemic antibiotics, antimycotics, corticosteroids and retinoids were applied with a transient result. The patient was treated with topical Pimecrolimus which showed a good result. Postlesional hyperpigmentations were found after this treatment.