[Bullous paraneoplastic acrokeratosis]

Bullous lesions of acral distribution are an uncommon finding in Bazex's syndrome (acrokeratosis paraneoplastica). We report here one of these rare cases.

A 65 year-old-man, an alcoholic and a smoker, presented with characteristic lesions of Bazex's acrokeratosis paraneoplastica associated with a right cervical lymph node mass. An epidermoid carcinoma of sinus piriformis was then discovered. The acrokeratosis lesions worsened and spread to the knees, elbows, trunk and genitalia, with an erosive aspect, pain in the extremities, and tender, hemorrhagic bullous lesions on the toes and sides of the feet. Histological examination of a biopsy sample showed a subepidermal blister with numerous eosinophilic leucocytes. Direct immuno-fluorescence showed C3 deposits on dermal capillaries and IgA and IgM on colloid bodies in the papillary dermis. Blood eosinophilia and high levels of IgE were noted. Indirect immunofluorescence was negative for anti-epidermal and anti-basement membrane antibodies. Radiation and chemotherapy for the neoplasia resulted in healing of the bullous lesions and almost complete disappearance of keratotic squamous lesions with residual hyperpigmentation.

Although bullous lesions are rare in Bazex's syndrome (acrokeratosis paraneoplastica), they were noted as early as 1968 by Degos et al. These acral bullae mimic autoimmune bullous disease. An immunological reaction is thought to occur with antigens of the dermal-epidermal junction and eosinophils appear to be involved.