Scleroderma-like disorders.

Abstract

Scleroderma-like disorders are widely disparate conditions mimicking either systemic sclerosis or cutaneous localized scleroderma, not infrequently displaying features of both. Some are exclusively sclerotic, some scleroatrophic with prevailing sclerosis or atrophies. The recognition of scleroderma-like disorders is of practical importance because by establishing the cause of the disease, it is possible to introduce an effective therapy, as in scleredema Buschke or scleredema diabeticorum, sclerodermiform porphyria, Borrelia burgdorferi-induced sclerodermiform acrodermatitis atrophicans, sclerodermiform phenylketonuria, drug-induced conditions, and so on. Scleroderma-like disorders strongly suggest that the pathogenesis of skin sclerosis and internal involvement may be divergent, and of various causes. Some of them, such as atrophoderma Pasini-Pierini or progressive facial hemiatrophy, frequently overlapping with scleroderma, make the differentiation very difficult, if at all possible, and the diagnosis is often arbitrary. Some, as sclerodermiform graft-versus-host reaction, point to the autoimmune origin of scleroderma. The amply-covered congenital sclerodermiform conditions present a large spectrum of still not widely known and extremely heterogeneous syndromes, associated with numerous anomalies and/or malignancies.