Scleromyxedema: treatment of widespread cutaneous involvement by total skin electron-beam therapy

Case Report The patient, a 37-year-old woman, presented with generalized cutaneous erythema, papular eruption, and sclerodermoid induration of the dermis. The eruption was especially localized on the face, with distortion of the facial features and a “leonine” appearance. Nodular infiltration was particularly thick on the neck, chin, all around the ears, eyes, and lips with severe functional impairment. Initially, a dynamic arc electron therapy technique with a 4-MeV electron beam was used to treat the thicker face lesions: a total dose of 13.60 Gy was delivered in eight daily fractions. A lead eyeblock was used. From February 2003 to March 2003, TSEBT was performed using the Stanford Standing University technique. A total dose of 20.40 Gy was delivered in 20 fractions; fractions were given daily (5 days/week). Lead blocks were used for eye and nail protection. The patient underwentin vivodosimetry. The treatment was completed with a boost dose to the soles of the feet using a 6-MeV electron beam (10 Gy in five fractions). TSEBT produced immediate improvement with a mild acute toxicity including cutaneous erythema and epilation. At 6 months and 1 year after TSEBT, we noted a progressive decrease in size and number of the papulo-plaques on the face and a generalized decrease in skin thickness and edema with improvement of mobility. At 20 months, further improvement was observed, with almost total flattening of the infiltration on the face and disappearance of the “leonine” aspect.We noted the formation of a network of micropapules on the lower part of the face, as described in the literature. In addition,the folds on the neck were significantly improved by the treatment, but slight thickening persisted on the elbows and knees.