Scleromyxedema. A variant of Lichen myxedematosus

Abstract

Two patients with typical skin lesions of scleromyxedema (lichen myxedematosus) are described. The disorder is characterized by proliferation of fibroblasts and excessive deposition of acid mucopolysaccharides in the dermis. Quantitative elevated levels of acid mucopolysaccharides were demonstrated in the serum and skin of 1 patient. Each patient presented a clinical course ending in fatality, consisting of coronary atherosclerosis and bizarre central nervous system symptoms. Acid mucopolysaccharides were not demonstrated by histochemical examination of other organs at autopsy. A review of other similar cases indicates that abnormalities of serum proteins, bone marrow, and other systemic symptoms may rarely accompany this condition. It is postulated that scleromyxedema may be a disorder of altered fibroblast-mucopolysaccharide balance and represent a special group under the lichen myxedematous conditions.