title: Mucocutaneous leishmaniasis masquerading as Wegener granulomatosis.
abstract: A 43-year-old Brazilian female presented in 2001 with nasal stuffiness and sinusitis. A biopsy was consistent with limited Wegener's granulomatosis although antineutrophil cytoplasmic antibodies were negative. Her nasal inflammation progressed despite trials of prednisone, methotrexate, and azathioprine. A septal perforation developed and a repeat biopsy showed granulomatous inflammation. In 2006 the patient was referred to Division of Rheumatology, University of California, Los Angeles. The nose was grossly erythematous and a magnetic resonance imaging revealed nasal destruction and sinusitis. Palatine biopsies showed chronic inflammation. Cyclophosphamide at 150 mg/d resulted in markedly improved mucocutaneous lesions. The patient developed a leg and arm rash in 2007. A skin biopsy was positive for Leishmania braziliensis. The cyclophosphamide was discontinued and amphotericin B was initiated with transient benefit. Remission was achieved with pentavalent antimony. Despite multiple nasopharyngeal biopsies, for a 6-year span, mucocutaneous leishmaniasis masqueraded as Wegener's granulomatosis. Cyclophosphamide not only resulted in clinical improvement, due to reduced inflammatory response, but also allowed widespread cutaneous dissemination.
authors.affiliation: Division of Rheumatology, UCLA School of Medicine, Los Angeles, CA 90095, USA. ebrahn@mednet.ucla.edu
authors.forename: Ernest
authors.initials: E
authors.lastname: Brahn
authors.forename: David A
authors.initials: DA
authors.lastname: Pegues
authors.forename: Qingping
authors.initials: Q
authors.lastname: Yao
authors.forename: Noah
authors.initials: N
authors.lastname: Craft
mesh_terms: D000328:Adult; D000666:Amphotericin B / Q000008:administration & dosage; D000981:Antiprotozoal Agents / Q000627:therapeutic use*; D001938:Brazil; D002140:California; D003937:Diagnosis, Differential; D005260:Female; D014890:Granulomatosis with Polyangiitis / Q000175:diagnosis*; D006801:Humans; D007262:Infusions, Intravenous; D007897:Leishmaniasis, Mucocutaneous / Q000175:diagnosis* / Q000188:drug therapy*; D008536:Meglumine / Q000627:therapeutic use*; D000077485:Meglumine Antimoniate; D009942:Organometallic Compounds / Q000627:therapeutic use*; D012017:Referral and Consultation
chemical_list: D000981:Antiprotozoal Agents; D009942:Organometallic Compounds; D008536:Meglumine; D000077485:Meglumine Antimoniate; D000666:Amphotericin B