Mucocutaneous Leishmaniasis

Mucocutaneous leishmaniasis (ML, espundia) is a destructive mucosal complication of New World cutaneous leishmaniasis, caused mainly by Leishmania (Viannia) braziliensis, also L. panamensis and L. guyanensis.

• Occurs in 1 to 10% of patients with L. braziliensis CL, months to years after the primary lesion (sometimes decades later), via lymphatic and haematogenous spread.
• Endemic in Bolivia, Brazil, Peru, and Colombia.
• Risk factors: inadequate or no treatment of CL, large or multiple lesions, lesions on the face and upper body, malnutrition, and immunosuppression.

• Nasal congestion, epistaxis, crusting, and discharge, progressing to septal perforation, nasal collapse ("tapir nose"), and destruction of the palate, lips, pharynx, and larynx.
• Hoarseness, dysphagia, and airway obstruction with aspiration pneumonia in advanced disease.
• Scars of previous CL are often present.
• Death results from respiratory failure and secondary infection.

• Biopsy of mucosa (few parasites), PCR (most sensitive), culture, and histopathology showing granulomas with plasma cells.
• Positive leishmanin skin test and serology (high titres) support the diagnosis.
• ENT endoscopy and CT of the sinuses and airway.
• Exclude granulomatosis with polyangiitis (GPA), paracoccidioidomycosis, histoplasmosis, rhinoscleroma, TB, syphilis, leprosy, NK/T lymphoma, and cocaine induced destruction.

• Liposomal amphotericin B 3 mg/kg/day for 7 days (total 20 to 60 mg/kg; 40 mg/kg is a common regimen).
• Pentavalent antimonials 20 mg/kg/day IM or IV for 30 days.
• Miltefosine 2.5 mg/kg/day for 28 days (lower efficacy in some regions).
• Amphotericin B deoxycholate 0.7 to 1 mg/kg/day for 15 to 20 doses as an alternative.
• Add pentoxifylline 400 mg three times daily for 30 days to antimonials to improve response in refractory cases.
• Corticosteroids (prednisolone 1 mg/kg) for laryngeal oedema and airway compromise; tracheostomy for obstruction.
• Reconstructive surgery after cure; follow up for 12 months, with relapse in up to 10 to 20%.
• Early treatment of CL caused by L. braziliensis prevents mucosal disease.