Abstract
A 42-year-old man with Behçet's disease was admitted because of visual loss in his right eye. He had been suffering from recurrent genital ulcer, uveitis, folliculitis and oral ulcer. On admission, studies using a monoclonal antibody as peripheral blood T lymphocyte surface marker, revealed OKT 4 antigen deficiency. His Leu 3 a and PHA, ConA response was normal. Examination of the family tree indicated that the hereditary type of OKT 4 epitope deficiency was imcomplete dominant.
The association of Behçet's disease and OKT 4 epitope deficiency has not been reported.
Oral administration of cyclosporin A (CYA) 400mg per day for severe uveitis was initiated. Some days later, his uveitis condition improved, but the patient noticed muscle weakness and muscle pain in proximal limbs. After the laboratory examinations, the serum levels of CPK, aldolase and transaminase were markedly elevated. The electromyograms revealed typical myogenic pattern. These findings gradually improved after administration of prednisolone. Only one case of CYA induced myopathy has been reported.
It is not clear whether the myopathy in this case was a common side effect of CYA, or if it occurred on the basis of OKT 4 epitope deficiency.
