Case Report Mucormycosis of the Vulva Following Bone Marrow Transplantation

Case report Mucormycosis of the vulva following bone marrow transplantation

J Nomura1, J Ruskin1, F Sahebi2, N Kogut2 and PM Falk2

1Department of Internal Medicine and Pediatrics, Infectious Disease Division, and 2Division of Bone Marrow Transplantation, Kaiser Permanente Medical Center, Los Angeles, CA, USA

Summary: ing antimicrobial therapy to imipenem and continuing flu- conazole. Blood cultures were negative and the chest X- Mucormycosis is uncommon in bone marrow transplant ray was normal. recipients. Primary cutaneous mucor in any setting is Granulocytes were у0.5 × 109/l on day +20. Six days unusual. It may mimic necrotizing bacterial infection later the inflammation, which had spread to involve the but- particularly in patients with severe neutropenia. We tocks and labia, receded. Over the next week, the center report a case of vulvar mucormycosis in a transplant of the lesion became necrotic. On day +41, she underwent recipient. Diagnosis depends on histological identifi- extensive debridement of the perineum, vagina and rectum, cation of fungal hyphae invading tissue and blood a diverting colostomy was performed and a suprapubic vessels. catheter placed. Histologic examination of the debrided Keywords: mucormycosis; vulva; post-transplantation tissue demonstrated severe acute and chronic inflammation. Broad non-septate hyphae, branching at right angles, were noted in necrotic areas and within blood vessels. Bacterial cultures were negative but a fungal culture grew Rhizopus Mucormycosis usually refers to diseases caused by sapro- phytic fungi of the order Mucorales. It occurs predomi- sp, a member of the Mucorales order. nantly in immune compromised people including those with diabetes and persons with hematologic cancers. The fungus invades blood vessels resulting in extensive tissue necrosis. Typically, mucormycosis is rapidly progressive and is often fatal. Discussion Although bone marrow transplant recipients have severe immune suppression and are at high-risk for opportunistic fungal infections, mucormycosis is unusual.1 When it In a recent review, only 13 of 1500 transplant recipients 2 occurs, the disease usually involves the lungs, sinus and (0.9%) developed mucormycosis. Sites of involvement nasal regions, gastrointestinal tract, kidneys and brain. Skin included lung, brain, sinus and nasal regions and dissemi- involvement is rare and may be difficult to distinguish from nated disease. Skin or soft tissue infection was not reported. necrotizing bacterial infections. It may be difficult, however, to differentiate cutaneous mucormycosis from bacterial necrotizing fasciitis, especially in the perineum. Case report Cutaneous mucor is uncommon but the incidence may be increasing.3,4 It is described in surgical wounds covered A 20-year-old female with acute promyelocytic leukemia by non-sterile elasticized bandages5 and at sites of in second complete remission was admitted for an HLA- trauma.6,7 Although cutaneous mucormycosis is often rap- matched, unrelated donor marrow transplant. Six days post- idly progressive with extensive tissue necrosis, it may transplantation she developed a fever of 38.9°C. The WBC advance slowly over a 2- to 4-week period, as was the case was 0.1 × 109/l and she received vancomycin and ceftazi- with our patient.7 dime. Blood cultures grew Klebsiella oxytoca. The fever Cutaneous mucormycosis should be considered when resolved but she remained neutropenic. Thirteen days post- skin necrosis continues despite appropriate antibiotics. transplantation she developed painful swelling in the peri- Definitive diagnosis requires histological identification of neum; there was a tender, indurated lesion in the posterior the characteristic hyphae in tissue and blood vessels and/or vaginal vault extending to the perineum. The lesion fungal cultures. Surgical debridement is required for cure increased in size over the next several days despite switch- and adjunctive medical therapy with amphotericin B is gen- erally recommended.8 Although obtaining adequate tissue for diagnosis is difficult in transplant recipients with throm- Correspondence: Dr J Nomura, 1505 N Edgemont Street, Los Angeles, CA 90027, USA bocytopenia and neutropenia, it may be necessary in clini- Received 9 December 1996; accepted 18 December 1996 cally suspected cases. Vulvar mucormycosis after BMT J Nomura et al 860 References acquired mucormycosis (Rhizopus rhizopodiformis) of skin and subcutaneous tissue. New Engl J Med 1978; 299: 1115–1118. 1 Gaziev D, Barconciani D, Galimberti M et al. Mucormycosis 6 Vainrub B, Macareno A, Mandel S, Musher DM. Wound zygo- after bone marrow transplantation: report of four cases in thal- mycosis (mucormycosis) in otherwise healthy adults. Am J Med assemia and review of the literature. Bone Marrow Transplant 1988; 84: 546–548. 1996; 17: 409–414. 7 Wilson CB, Siber GR, O’Brien TF, Morgan AP. Phycomycotic 2 Morrison VA, McGlave PB. Mucormycosis in the BMT popu- gangrenous cellulitis. Arch Surg 1976; 111: 532–538. lation. Bone Marrow Transplant 1993; 11: 383–388. 8 Sugar AM. Agents of mucormycosis and related species. In: 3 Fisher J, Tuazon CU, Geelhoed GW. Mucormycosis in trans- Mandell GL, Bennett JE, Dolin R (eds). Principles and Practice plant patients. Am Surg 1980; 46: 315–322. of Infectious Diseases. Churchill Livingstone: New York, 1995, 4 Adam RD, Hunter G, DiTomasso J, Comerci G Jr. Mucormy- pp 2311–2321. cosis: emerging prominence of cutaneous infections. Clin Infect Dis 1994; 19: 67–76. 5 Gartenberg G, Bottone EJ, Keusch GT, Weitzman I. Hospital-