This 43-year-old man with alpha-1-antitrypsin deficiency developed a progressive infiltrative process involving the hands and feet that spread to the proximal extremities. Early lesions consisted of fixed skin colored and red papules some forming fixed urticarial annular plaques. The plaques and diffuse infiltration of the skin also involved his face but spared the trunk. A skin biopsy showed changes typical of scleromyxedema. Serum protein electophoresis was pending. Scleromyxedema is a variant of lichen myxedematosus characterized by generazied erythematous soft 2-3 mm papules and diffuse infiltration of the skin. Often there is accentuation of the skin folds. In most patients an IgG paraprotein has been identified, but IgM and IgA paraproteins have occasionally been reported.
fixed annular infiltrated red plaques, discrete follicular papules, and diffuse infiltration of the distal extremities