Mucocutaneous Leishmaniasis
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Direct answer
Nasal stuffiness that ends years later in septal perforation and the "tapir nose" of destroyed naso-oral mucosa is mucocutaneous leishmaniasis — espundia — classically caused by Leishmania braziliensis in the New World, arising long after an untreated cutaneous lesion and demanding systemic therapy. The Indian exam angle is different: post-kala-azar dermal leishmaniasis (PKDL), a purely cutaneous-mucosal sequel of treated visceral leishmaniasis caused by L. donovani, appearing months to years after apparent cure as hypopigmented macules, patches and nodules on the face and trunk in patients from endemic Bihar, Jharkhand, West Bengal and Uttar Pradesh. Diagnosis is clinical-epidemiological (parasites are scanty; rk39 is typically negative in PKDL), and treatment under the national programme is oral miltefosine for about twelve weeks, with liposomal amphotericin B regimens as alternatives; untreated PKDL patients serve as the infection reservoir that keeps transmission alive.
What you must remember
- Two diseases, one exam label: New World espundia (L. braziliensis complex — mucosal destruction years after cutaneous disease, "primordial lesion" history) versus Indian PKDL (L. donovani — polymorphic skin lesions after treated visceral leishmaniasis).
- PKDL timeline: develops in a minority of visceral leishmaniasis patients, typically 6 months to 5 years (occasionally concurrent or earlier) after treatment, more frequent with miltefosine- or antimonial-treated disease in the Indian subcontinent.
- PKDL morphology: three recognised forms — macular (hypopigmented, the commonest and subtlest), maculopapular, and nodular (erythematous nodules mimicking leprosy); face first, then trunk; sensation is preserved, which separates it from lepromatous leprosy at the bedside.
- Diagnostic logic: parasites are scarce — slit-skin smear or biopsy positivity is patchy, rk39 serology is usually negative because the visceral infection has been cured; diagnosis is clinical plus endemic-district exposure, with histology (amastigotes in a granulomatous dermis) supportive when positive.
- Espundia features: persistent nasal congestion, epistaxis, then ulceration perforating the septum, spreading to palate, pharynx and larynx; Leishmanin (Montenegro) skin test positive; biopsy and PCR of mucosal lesions; systemic treatment always.
- Treatment, Indian programme: oral miltefosine about 2.5 mg/kg/day (50 mg twice daily for adults over 25 kg) for 12 weeks is the standard PKDL course under national guidance, with evidence that some Indian cases need longer (16 weeks has been studied); liposomal amphotericin B courses are the alternative — pregnant women must not receive miltefosine (teratogenic).
- Why treat PKDL at all: patients are the residual human reservoir for Phlebotomus argentipes transmission — treatment is an elimination-programme tool, not cosmetics; India's programme targets fewer than one case per 10,000 population at block level.
- Differential shortlist: lepromatous leprosy (anaesthetic lesions, thickened nerves), pityriasis versicolor, secondary syphilis, cutaneous lymphoma — the sensation-preserving, non-scaling, endemic-exposure pattern points to PKDL.
From a macule in Muzaffarpur to the programme's logic
A 28-year-old agricultural worker from Bihar had visceral leishmaniasis treated with single-dose liposomal amphotericin two years ago; he now presents with six months of progressively numerous hypopigmented macules over the cheeks and forehead plus a few erythematous nodules on the chin. Sensation over the lesions is intact, no peripheral nerve thickening, no Leonard Muir reaction — the bedside exclusion of leprosy in one move. A slit-skin smear from a nodule shows scanty amastigotes (Leishman-Donovan bodies); rk39 is negative, which fits cured visceral disease. Under national kala-azar elimination guidance he receives oral miltefosine for twelve weeks with weekly tolerance checks (vomiting, diarrhoea, and the reversible transaminase rise), with contraception counselled for men and women alike given teratogenicity. He is reported and tracked precisely because his skin sustains the sandfly cycle every season the lesion stays untreated. Contrast a Brazilian patient with a healed facial scar from a year-old cutaneous ulcer who now has septal perforation: that is espundia, treated systemically with liposomal amphotericin B, miltefosine or pentavalent antimonials — never topically — because mucosal disease never responds to local care.
Where students slip
Equating rk39 negativity with absence of leishmaniasis is the classic: rk39 detects antibodies from active visceral infection and is typically negative in PKDL, whose diagnosis is clinical-epidemiological. The second error is choosing a topical or abbreviated therapy for mucosal disease — systemic treatment is mandatory in both espundia and PKDL. Third, the leprosy mimic: preserved sensation and absence of nerve thickening are the two bedside facts that flip the diagnosis, and vivas probe them.
Frequently asked questions
What causes mucocutaneous leishmaniasis in the New World?
Leishmania braziliensis complex, typically invading naso-oral mucosa years after an untreated cutaneous lesion (espundia), unlike Indian PKDL which follows treated visceral disease.
How does post-kala-azar dermal leishmaniasis present?
Hypopigmented macules, maculopapular lesions or erythematous nodules on face and trunk, with preserved sensation, appearing months to years after treated visceral leishmaniasis.
Why is rk39 usually negative in PKDL?
Because rk39 detects antibodies generated by active visceral infection; by PKDL stage the systemic infection has been treated and antibody titres have waned, so diagnosis rests on clinical and endemic-exposure grounds.
What is the first-line treatment of PKDL in India?
A twelve-week course of oral miltefosine under national programme guidance, with liposomal amphotericin B as an alternative; miltefosine is contraindicated in pregnancy.
How is PKDL differentiated from lepromatous leprosy at the bedside?
Preserved sensation over lesions and absence of peripheral nerve thickening in PKDL, supported by endemic-district exposure history and, when positive, scanty amastigotes on slit-skin smear.