A case of facio-scapulo-humero-peroneal myopathy with inflammatory changes. Preliminary data on distribution of mononuclear cells in muscle tissue.

We report the case of a 49 year old woman with clinical, electrophysiological and histochemical signs of facio-scapulohumeroperoneal dystrophy characterized by highly inflammatory changes. Lymphocyte typing by immunofluorescence and immunoperoxidase techniques on a muscle biopsy fragment revealed a large number of T8 cells at endomysial sites. There was no evidence of direct invasion of sound fibers by lymphocytes, which are thus unlikely to have exerted a primary cytotoxic action on the muscle tissue. Another finding was an increased number of macrophages in both endomysial and perivascular regions. Cell-mediated immunity did not play a significant role in the pathogenesis of our case, unlike other reported cases of polymyositis and facioscapulohumeral dystrophy with inflammatory findings.