Scleredema of Buschke Successfully Treated With Electron Beam Therapy

A 58-year-old white woman with an 11-year history of type 1 diabetes mellitus presented in April 1995 with a complaint of flushing of the neck and the upper part of her trunk. She had had no antecedent febrile illness. The flushing resolved within a few days, but approximately 1 month later, the patient developed thickening and induration of the skin of the posterior section of her neck (Figure 1). She described restriction of neck movement, tenderness of the involved area, and a rash on the back of her neck. The results of her physical examination showed that she had several violaceous, indurated plaques involving the posterior section of her neck and the upper part of her back. The diagnosis of scleredema was confirmed with a skin biopsy specimen of the upper part of her back that showed thick bundles of collagen in the middle and lower dermis that were separated by abundant mucin (Figure 2). The results of serum protein electrophoresis were normal on 2 separate occasions. The plaques were treated with intralesional and topical corticosteroid therapy for 2 months without improvement. Oral corticosteroids were initially avoided given the patient’s history of diabetes mellitus, but when the induration spread to the lateral and anterior part of her neck and shoulders, oral corticosteroid therapy was initiated. This approach produced no improvement, and the patient developed cushingoid features. Prednisone therapy was therefore tapered and discontinued. In November 1995, she began to complain of tightness of her chest and face, with difficulty opening her eyes. Pulmonary function tests performed in December 1995 showed the following results: forced vital capacity, 57% of predicted; forced expiratory volume in 1 second, 53% of predicted; vital capacity, 57% of predicted; and total lung capacity, 64% of predicted. The restrictive component of these test results was thought to be secondary to the presence of scleredema on the patient’s trunk.