Positive Immuno-Reactions in a Case of Unilateral Scleroderma or Progressive Hemiatrophy

Summary

A case of unilateral scleroderma in a boy aged 14 years is reported. The disease began when the patient was five years old with slowly progressive dermal and subcutaneous changes. The face was not involved.

Microscopically, the connective tissue was found to be dense and the collagen fibers thick and hyalinized. Round cell infiltration was seen around the blood vessels and the skin glands. The bulk of the inflammatory cells were plasma cells and lymphocytes. The serologic abnormalities found were elevated gamma globulin and antistreptolysin titers, positive latex and Waaler-Rose tests, presence of cryoglobulins and antibodies against nucleoproteins and augmented gamma-M streak in immunoelectrophoresis. The patient has been receiving small doses of prednisone for nine years. The progress has been slow.