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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.)

Autoantibodies, or antibodies against the body's own tissues, are found in a variety of different disorders, but are especially common among the rheumatic diseases, including rheumatoid arthritis (RA), scleroderma, and lupus erythematosus. The specificity of these autoantibodies varies from patient to patient, however. Antinuclear antibodies (ANA), or antibodies to one or more constituents of the cell nucleus, are common among patients with rheumatoid arthritis. These antibodies are likely to be directed against double-stranded DNA, single-stranded DNA, or histone proteins found in the nucleus. However, two RA patients have been observed who have antibodies to lamin, which is a constituent of the nuclear envelope. Anti-lamin antibodies are observed by their ring-like pattern of staining under the microscope. After this characteristic ring is observed, the Western blot, in which individual bands of separated proteins are stained, may be used to confirm that the antibodies are indeed recognizing lamin. Anti-lamin antibodies have been seen in other disorders, including chronic active hepatitis, autoimmune cytopenia (decreased numbers of blood cells), and inflammation of the blood vessels. These two rheumatoid arthritis patients have no indications of these other disorders, and therefore RA may be added to the list of disorders in which anti-lamin antibodies may occur. However, the authors caution that any patient who has anti-lamin antibodies should be frequently evaluated for signs of developing any of these other conditions. (Consumer Summary produced by Reliance Medical Information, Inc.)