Bazex Syndrome

A 76-year-old woman presented to our hospital with a 3-month history of scaly plaques on the extremities. Prior to visiting our hospital, she visited a dermatology clinic and was diagnosed with psoriasis vulgaris. She was treated with topical corticosteroid and methotrexate, but these failed to relieve her symptoms, and she was referred to our hospital for a further evaluation and treatment. On presentation, she had violaceous psoriasiform dermatitis on the distal extremities, which was more prominent on the lower distal extremities than the trunk (Picture 1). A blood examination revealed elevated liver enzymes, biliary enzymes, pancreatic amylase and conjugated hyperbilirubinemia. Computed tomography (CT) of the abdomen revealed cancer of the pancreatic head with biliary obstruction and multiple liver metastases (Picture 2). Based upon the distribution of the skin lesions and the complication of the malignancy, the psoriasiform lesions were diagnosed as paraneoplastic acrokeratosis (Bazex syndrome) (1). Bazex syndrome is a rare cutaneous paraneoplastic disorder characterized by psoriasiform lesions on acral areas, such as the hands, feet, nose and ears. The peak incidence occurs in the 60s. There is a male predominance of about three-to-one over women. While the pathogenesis of Bazex syndrome is unknown, one hypothesis holds that growth factors, such as epidermal growth factor (EGF) and insulin-like growth factor (IGF), produced by tumor cells stimulate keratinocytes. Another hypothesis states that autoreactive T cells provoked by tumor cells play a pathogenic role in psoriasiform lesions. Skin lesions precede the diagnosis of malignacy in most cases (2). Thus, psoriasiform dermatitis can be an indicator of internal malignancy.

Picture 1.

Picture 2.

The authors state that they have no Conflict of Interest (COI).