F. Worthmann et al., MUSCULAR INVOLVEMENT IN BEHCETS-DISEASE - CASE-REPORT AND REVIEW OF THE LITERATURE, Neuromuscular disorders, 6(4), 1996, pp. 247-253
Acute necrotizing myositis is described in a 22-yr-old man with clinic
ally diagnosed Behcet's disease. Light microscopic examination reveale
d a prominently granulocytic-monocytic infiltration of the muscle with
severe necrosis. An infectious (bacterial, fungal or parasitic) etiol
ogy could be excluded by specific staining techniques and by immunohis
tochemistry. Vascular deposition of immune complexes was detected by d
irect immunofluorescence. Electron microscopy revealed severe structur
al damage and phagocytosis of muscle fibers. In the endomysium, leukoc
ytes and occasional erythrocytes were found. Virus-like particles were
not seen. The relevant literature on muscular involvement in Behcet's
disease is reviewed. It is suggested that two different stages of inf
lammation occur in Behcet's disease. In the acute stage it presents as
a granulocytic-monocytic necrotizing reaction developing from a neutr
ophil-mediated vasculitis. In the later phase lymphocytic infiltration
s predominate. Despite the rare involvement of muscles the diagnosis o
f Behcet's disease should be considered particularly in younger patien
ts presenting with muscular symptoms like pain and swelling pre-domina
ntly of the lower extremities.