Asian Journal of Research in Infectious Diseases

Background: Buruli ulcer, caused by Mycobacterium ulcerans, is the third most common mycobacterial infection in immunocompetent individuals and is endemic in West Africa. Lesions predominantly involve the limbs and are characteristically painless; head and neck involvement is rare and reported mainly in children. Paradoxical reactions, defined as clinical worsening of existing lesions or appearance of new lesions during or after antibiotic therapy, occur in a substantial minority of treated patients and are associated with high baseline bacterial load and large lesions. We report a fatal centrofacial Buruli ulcer in an adult in which a severe paradoxical reaction appears to have been precipitated by the simultaneous withdrawal of corticosteroid-containing chemotherapy and the initiation of specific antimycobacterial therapy.

Case Presentation: A 25-year-old immunocompetent Ivorian woman living in a swampy, Buruli-endemic area of Abidjan presented with a five-month history of a painful, febrile, rapidly extending ulceronecrotic lesion of the midface, corresponding to World Health Organization category III. Noma was diagnosed and treated with debridement and antibiotics without benefit. By week 7 the upper maxilla was almost entirely destroyed. Klebsiella rhinoscleromatis was isolated, but rhinoscleroma was excluded histologically. Because of fulminant progression and suspected extranodal NK/T-cell lymphoma, empirical chemotherapy with cyclophosphamide, vincristine and prednisone was started at week 12; the lesion stabilised for the first time since admission. At week 15, M. ulcerans DNA was detected by PCR targeting the IS2404 insertion sequence. Chemotherapy was discontinued after two cycles and rifampicin plus clarithromycin were started. Twenty-seven days after initiating antimycobacterial therapy, the lesion resumed rapid destructive extension, nutritional status deteriorated, and the patient died at week 24 from terminal cachexia.

Conclusions: Centrofacial Buruli ulcer may occur in adults and present atypically as a painful, febrile, necrotising lesion closely mimicking noma and other midfacial destructive lesions. A positive molecular result obtained from a chronically superinfected wound must be interpreted alongside clinical and histopathological coherence. In extensive lesions at critical anatomical sites, abrupt withdrawal of immunosuppression at the time antimycobacterial therapy is initiated may precipitate a fatal paradoxical reaction, and corticosteroid cover should be maintained or tapered rather than stopped abruptly.