Painful ophthalmoplegia as ocular manifestation in cytophagic histiocytic panniculitis associated with Churg Strauss syndrome.

摘要

Churg Strauss syndrome (CSS) is a systemic vasculitic disorder characterized by granulomatous inflammation affecting small to medium-sized blood vessels. Although the cause of CSS remains unknown, tissue damage seems more likely to be mediated by eosinophilic tissue infiltrates. The American College of Rheumatology (ACR) criteria (Masi et al., 1990) for the classification of CSS defined the disease by 4 out of 6 manifestations (specificity of 99.7% and sensitivity of 85%): the presence of asthma, hypereosinophilia (>10%), mononeuropa-thy or polyneuropathy, non-fixed pulmonary infiltrates, paranasal sinus abnormalities, and extravascu-lar eosinophil infiltration in biopsy specimens. CSS can also affect the skin, heart, gastrointestinal tract, and eyes, even though it is rare.