This 67-year-old woman developed the classic cutaneous findings of dermatomyositis without clinical or laboratory evidence of muscle disease one year ago. Serological analysis revealed a positive antinuclear antibody, negative anti-Jo-1 antibody, and normal muscle enzymes. A complete medical examination, MRI and CT examination of the chest, pelvis, and abdomen, and mammography revealed no evidence of internal malignancy. Cutaneous examination revealed diffuse erythema in a shawl-like distribution over the chest and shoulders, a V-shaped distribution over the anterior neck and chest as well as patches over the forehead, malar region and chin, and lower extremities. She also demonstrated Gottron's sign (a symmetric, scaly, violaceous erythematous eruption over the extensor surfaces of the metacarpophalangeal and interphalangeal joints of the fingers) and Gottron’s papules (symmetric,erythematous to purplish,scaly, flat papules on the extensor surfaces of the metacarpophalangeal and interphalangeal joints) and the heliotrope rash (a reddish-violaceous eruption on the upper eyelids, often accompanied by swelling of the eyelid). In some areas on the arms and legs she had necrotic papules and crusts
Dermatomyositis sine myositis or amyopathic dermatomyositis has been used to describe patients with the typical rash and histologic changes of dermatomyositis without evidence of myopathy. Many of these patients eventually develop clinical and/or histologic evidence of myositis. In some cases, however, muscle involvement has not occurred for as long as six years. Middle age patients with dermatomyositis and probably amyotrophic dermatomyositis should be carefully evaluated for occult malignancy which has been reported in up to 50 percent of cases.
Comments:Although the patient was treated with high doses of prednisone,IV IgG ,AZA & MTX the rash perstisted.
Description
symmetric violaceous patches with edema, some scale, and some necrotic crusts
