LAUSR

A 25yearold man was referred with fever, skin ulceration and recent intestinal perforation. The intermittent fever and oral ulcers (top left) had led to an initial diagnosis of Behçet's disease 2 years previously. Initially, he had been treated with corticosteroids and immunosuppressive agents, which gave only shortterm disease control. The ulcers relapsed in different places and quickly evolved into extensive mucocutaneous ulceration involving the trunk, limbs and external genitalia (top middle and top right). Endoscopy showed multiple deep ulcers ranging from 1 to 2.5 cm in diameter, extending from the pharynx to the rectum. Biopsy of the bowel lesions was inconclusive. Adalimumab led to some healing but 3 months later he experienced high fever and acute abdominal pain. Computed tomography showed perforation of the ileum requiring emergency surgery and creation of a stoma. Pathological consultation on the resected intestine in our hospital established the diagnosis of chronic active Epstein– Barr virus infection (CAEBV), systemic form, Tcell phenotype. His serum EBVDNA quantification was 3.5 × 104 copies/ml. Unfortunately, 2 weeks after the surgery the patient developed haemophagocytic lymphohistiocytosis (HLH) with a high fever, pancytopenia, decreased fibrinogen and elevated ferritin. Moreover, new ulcers developed. Biopsy showed dense CD3+ Tlymphocytic infiltration with monoclonal Tcell receptor rearrangement. Worse still, progressive ulceration and necrosis occurred around the stoma with extension into the abdominal musculature (bottom left and centre). Partial healing occurred with closure of the stoma and local measures (bottom right). Considering the systemic and aggressive nature of his disease, different chemotherapeutic regimens involving etoposide, dexamethasone, pegaspargase, gemcitabine and a PD1 antibody were tried. Nevertheless, the disease progressed after a short response, presenting as repeated HLH, spontaneous intestinal perforations and intractable intraabdominal infection. Mucocutaneous lesions are rare in CAEBV and in the early stages can be very difficult to distinguish from Behçet's disease and inflammatory bowel disease due to overlapping clinical and endoscopic findings. Management of this condition requires novel therapeutic modalities.