Cutaneous manifestations associated with gamma heavy chain disease

Gamma heavy chain disease (GHCD) is a disorder of the immune system in which abnormal proteins of a certain type (heavy chain) appear in the blood and urine. The case history of a 78-year-old man with GHCD who had skin changes as one of the symptoms is presented. The patient developed livedo reticularis (bluish patches) on the skin, followed by necrosis (tissue death) of the fingers and toes. The patient also had many nodules (lumps) under the skin. Results from skin biopsies showed that white blood cells were concentrated in the dermis (the layer of the skin below the epidermis). In the necrotic areas, the patient's blood vessels were inflamed. Based on other signs and symptoms, a diagnosis of GHCD was made. It now appears that skin lesions are the most frequent abnormality associated with GHCD besides blood cell abnormalities; they have been reported in 17 of the 90 cases in the medical literature. Skin nodules have been reported in a smaller number of GHCD patients. The patient in the report improved briefly after treatment with prednisone (an immunosuppressive drug), but became sicker and died approximately one year after diagnosis. (Consumer Summary produced by Reliance Medical Information, Inc.)