DERMATOMYOSITIS AND MALIGNANCY: A REVIEW OF THE LITERATURE

Abstract

The first report of dermato-myositis associated with malignancy in 1916 by Stertz has been followed by increasing attention in the literature. Of 590 cases reported in the literature, 92 have had tumor accompanying dermatomyositis. In these 92 case reports, sex distribution was equal and age range 40-70. The most common sites of associated malignancy were: stomach (16 cases), breast (15 cases), lung (14 cases), ovary (10 cases), lymphoma (6 cases), leukemia (9 cases), gall bladder,(4 cases), colon and rectum (4 cases), kidney (3 cases), uterus (3 cases), and larynx (3 cases). Less frequent tumor origins included: multiple myeloma, retroperitoneal sarcoma, parotid carcinoma, endothelial sarcoma, carcinoma of cervix, epithelioma of vagina, carcinoma of esophagus, plasmacytoma, chromophobe adenoma, and substernal sarcoma. Striking by their absence were carcinoma of the prostate and carcinoma of the pancreas. The clinical picture of patients with dermatomyositis and malignancy was characterized by acute onset of symptoms, often with skin manifestations preceding the muscular symptoms. In 58 full reports, 47 patients had dermatomyositis preceding recognition of associated tumor, whereas 11 developed dermatomyositis after tumor was discovered. Amelioration of dermatomyositis was most often noted after treatment for carcinoma of ovary or breast. The incidence of tumor accompanying dermatomyositis is estimated at 15.3% from available case reports. Similar associations with malignant disease are noted in migratory thrombophlebitis, pulmonary osteoarthropathy, acanthosis nigricans, and recurrent herpes zoster. Spinocerebellar degeneration, peripheral neuropathy, polycy-themia, and amyloidosis may also be companions of malignancy. Speculation as to cause and effect is advanced. Skin and muscle changes of dermatomyositis may be a reaction to malignant cells or possibly reflections of endocrine imbalance. The symptom-complex of acute onset of dermatomyositis in a patient of middle age should incite a careful search for occult tumor.