Mucocutaneous leishmaniasis masquerading as Wegener granulomatosis

J Clin Rheumatol. 2010 Apr;16(3):125-8. doi: 10.1097/RHU.0b013e3181d52c96.


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.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Amphotericin B / administration & dosage
  • Antiprotozoal Agents / therapeutic use*
  • Brazil
  • California
  • Diagnosis, Differential
  • Female
  • Granulomatosis with Polyangiitis / diagnosis*
  • Humans
  • Infusions, Intravenous
  • Leishmaniasis, Mucocutaneous / diagnosis*
  • Leishmaniasis, Mucocutaneous / drug therapy*
  • Meglumine / therapeutic use*
  • Meglumine Antimoniate
  • Organometallic Compounds / therapeutic use*
  • Referral and Consultation


  • Antiprotozoal Agents
  • Organometallic Compounds
  • Meglumine
  • Meglumine Antimoniate
  • Amphotericin B