Atypical collagen disease. Polymyositis, prominent hematoxylin body formation and some manifestations of progressive systemic sclerosis.

An autopsy case of polymyositis with prominent hematoxylin body formation in various organs and tissues, florid focal and segmental necrotizing glomerulonephritis, and some manifestations of progressive systemic sclerosis was presented. A 34-year-old woman had erythematous edema on the face, joint pain, muscular weakness, cutaneous sclerosis and Raynaud's phenomenon for about 5 years. She had also a long history of pulmonary tuberculosis. Autopsy revealed polymyositis involving skeletal, smooth and heart muscles. This was widespread consisting of focal and extensive primary degeneration of muscle fibers and interstitial inflammatory cell infiltrates near and surrounding small veins and venules accompanied by the derivation of hematoxylin bodies from inflammatory cell nuclei, and in the posterior pharyngeal muscles severely affected, muscle substance was literally erased, leaving interstitial fibrous tissues and blood vessels. This focally restricted, severe necrosis of the skeletal muscle gives a special feature to the polymyositis in the present case. The diagnosis of polymyositis with prominent hematoxylin body formation and some manifestations of progressive systemic sclerosis was applied as a rather descriptive designation, but yet it does not absolutely exclude the possibility of a variant of SLE in an additional effort to search for an underlying disorder.