Immunocompromised Infection with Behçet Disease

Immunocompromised Infection with Behà §et Disease Abstract Behà §et disease is a relapsing multiorgan inflammatory disorder characterised by mucocutaneous, opthalmic, neurological, vascular and gastrointestinal involvement. We report an intriging case with rare manifestations of myositis and myocarditis, and the first reported occurrence of an immunocompromised-associated infection (polymicrobial necrotising fasciitis) without immunosuppresive therapy use in this presumably hyperimmune disorder. Lessons from practice: 1. Behà §et Disease is a rare autoimmune condition with significant geographical variation in its distribution and is most prevalent in people of Turkish background. 2. Behà §et disease is a systemic disease with predominant symptoms of oral ulcerations, genital ulcerations and uveitis. Other systemic involvement include neurological, gastrointestination, rheumatic, dermatological manifestation. Less common but well described manifestation include muscle and cardiac involvement. 3. The unexplained manifestation of Type 1 necrotising fascitis in this patient suggests that immune dysregulation in Behà §et disease could contribute to primary immunosuppression. It is important to maintain high viligance for risk of infection in Behà §et disease. Clinical Record A 40-year-old woman presented to hospital for investigation of progressive malaise, anorexia and generalised proximal myalgia over 6 days with an elevated serum creatine kinase [CK] of 3550 U/L (reference range [RR] < 145). She is of Spanish and Turkish ethnic background. Her medical history included transfusion-related Hepatitis C Virus (HCV) infection, lower limb deep vein thrombosis with Factor V Leiden mutation and depression. She denied any infective symptoms, recent trauma or stenuous exercise, rash or athralgias. Physical examation was unremarkable except for proximal generalised myalgia without weakness. Laboratory investigations initially revealed a normal erythrocyte sedimentation rate and C-reactive protein (CRP). Over the next 72 hours, she developed an acute bilateral polyathropathy of the wrist and metacarpalphalangeal joints. Multiple cutaneous manifestations were observed including (1) a rapidly evolving erythema nodosum-like rash over the legs which later progressed into a purpuric non-blanching rash with fluctuant subcutaneous oedema, (2) multiple oral aptheous ulcers and (3) perioral acneiform papular nodules. Further laboratory studies showed mildy raised inflammatory markers and a normalising CK of 428 U/L. The results of investigations for differential diagnoses were unremarkable. (Table 1) Multiple skin biopsies showed a neutrophilic-dermatosis like reaction. On day 6, she developed a mildly tender natal cleft excoriation which progressively became pustular over 2 days with a dramatic increase in CRP >380mg/L and worsening renal function. She remained systemically well. A wound swab grew methicillin-sensitive staphylococcus aureus. Oral flucloxacillin and metronidazole were commenced. MRI showed enhanced T2 signals in the presacral fascia correlating to the sacral wound. A biopsy of the natal cleft wound showed similar findings to previous skin biopsies. Simultaneously, a pathergy-like reaction was noted with previous intravenous-cannula and biopsy sites becoming pustular. At this point, further clinical history revealed a 5 year history of recurrent oral and gential ulcerations up to 4 episodes a year. A clinical diagnosis of Behà §et disease was made. Subsequent HLA-B51 testing and ophthalmological examination were unremarkable. On day 10, she deteriorated acutely becoming hypotensive and hypoxic. Computer tomography (CT) pulmonary angiography revealed bilateral pneumonia. Serum white cell count and CK increased substantially from 11.0 x109/L to 37.2 x109/L (RR 4.0 – 11.0) and 233 U/L to 3715 U/L respectively in 4 days. She was intubated, commenced on broad-spectrum antibiotics with systemic glucocoticoid and inotropic support. A further CT of the pelvis revealed numerous sacral subcutaneous gas locules and fluid. Multiple debridement procedures achieved drainage of large amount of pus and excision of the necrotic tissue. Sacral necrotising myofasciits was confirmed on a frozen section specimen showing extensive necrosis within subcutis, fascia and skeletal muscle with numerous mixed bacteria. Post-operatively, a substantially elevated cardiac Troponin-I level of 51.33 ug/L (RR