BEHCET’S Dz(SYNDROME)

BEHCET = A TURKISH DERMATOLOGIST (1937)

A CHRONIC IDIOPATHIC SYSTEMIC VASCULITIS (INFLAMMATORY DISORDER)

MALE > FEMALE

3RD DECADE

HIGH INCIDENCE IN TURKEY, JAPAN, EUROPE

MAJOR TRIAD: RECURRENT APTHOUS ULCERS (100%), GENITAL ULCERS(65%), OCULAR INFLAMATION (IRIDOCYCLITIS)—-CAN LEAD TO BLINDNESS IN ABOUT 3 YEARS!!!!—ALSO PROMINENT IS CUTANEOUS VASCULITIS(HYPERIRRITABLE SKIN)

APTHOUS ULCERS ARE CHARACTERISTICALLY “PUNCHED OUT” WITH SURROUNDING ERYTHEMA AND COVERED WITH A PALE PSEUDOMEMBRANE—-PAINFUL–OCCUR IN CLUSTERS

PROGRESSIVE SNHL

AN AVERAGE OF 4 YEARS FROM ONSET TO DIAGNOSIS

OFTEN + FHx

MINOR Sx: ARTHRALGIAS, MENINGOENCEPHALITIS(LATE=ASEPTIC MENINGITIS—-THE PRIMARY CAUSE OF DEATH), FORCED LAUGHTER, THROMBOPHLEBITIS(DVT), BULBAR PAULSEY, ESOPHAGITIS, COLITIS, C-V DISORDER, GLOMERULAR NEPHRITIS

LEUKOCYTOSIS

HIGH ESR AND CIRCULATING IMMUNE COMPLEXES, HIGH AB TO MUCOSAL CELLS

EOSINOPHILIA

Rx: COLCHICINE FOR ULCERS, STEROIDS, AZOTHIAPRINE, CYCLOSPORIN A

POSSIBLE SURGERY