[Late manifestation of a fatal Behçet's disease with cardiac involvement and lethal outcome].

HISTORY AND CLINICAL FINDINGS: One year before admission a 67-year-old man of German origin developed extensive ulcerations of the limbs, later also of the trunk and neck. In addition to recurrent oral aphthous ulcers it was associated with erythema nodosum and intermittent arthritis of the elbow knee and ankle joints. During the last month he also had dyspnoea. Echocardiography revealed a dilated cardiomyopathy (DCM). He was admitted because of the progressive skin disorder. Physical examination showed a disoriented man with dyspnoea, cyanosis of the lips and pretibial oedema. He had aphthous ulcers in the mouth and on the genitals. Adamant Behçet disease was suspected.

INVESTIGATIONS: Erythrocyte sedimentation rate was raised to 21/48 mm/h, white cell count to 15,800/microliter. Tests for HLA B5 and B27 were negative, skin biopsy revealed superficial necrotizing vasculitis of postcapillary venules.

TREATMENT AND COURSE: Initially high doses of prednisolone, 100 mg/d intravenously) and azathioprine (100 mg/d orally) were administered, and within a few days both the skin and cardiac changes had regressed. Prednisolone dosage was reduced and cyclosporin (350 mg/d) substituted for azathioprine. He was discharged in a markedly improved general condition and with only a few tibial ulcerations. 4 months later he had a severe recurrence with dramatic mucocutaneous involvement and rapidly deteriorating DCM, together with radiological signs of pneumonia. He died 4 months later.

INTERPRETATION: Although very rare, Behçet disease should even in the elderly patients be considered in the differential diagnosis: in the presence of appropriate symptoms cardiac involvement should be looked for.