Lymphomatoid granulomatosis with primary manifestation in the skeletal muscular system.

Lymphomatoid granulomatosis (LYG) has been recognized as a peculiar disease which primarily affects the lungs and occasionally the upper respiratory tract. Typical histopathologic lesions present features of a nonneoplastic lymphoproliferative disorder associated with necroses of the infiltrated tissues and with angiodestructive processes. The authors observed a peculiar case in which lesions typical for LYG first developed in the striated muscles. In this case multiple tumorous muscular infiltrates and affections of the pharynx, larynx, paranasal sinuses, and orbita preceded the infiltration of the lung, which was first recognized 6 months after the first muscular infiltrate and 2 months before death. Because primary muscular manifestation of LYG has never been reported, this case is remarkable both for its unusual manifestation and for its striking irresponseveness towards therapy. Clinical and experimental data suggest impairment of T-cell functions.