Leishmaniasis mucocutaneous B55.2

Chronic, therapy-resistant leishmaniasis of the skin and adjacent mucous membranes due to lympho- or hematogenic spread of the pathogens. It is possible that mucosal involvement is triggered or promoted by immunosuppression. The mucosal lesions occur during the cutaneous symptomatology or after a clinical interval of several years.

Mucocutaneous leishmaniasis occurs in 1-3% of infected persons suffering from cutaneous leishmaniasis. In Bolivia, this form is found in up to 20% of infected patients.

Often epistaxis (due to the early involvement of the nasal septum) is the first clinical sign of mucocutaneous progression of the disease. First skin lesions are found between the upper lip and the nasal septum. Initially recurrent, later persistent swelling of the lips and nose occurs, which becomes fibrous over the course of months. Noticeable are bulging, hardened upper lips (facies leishmaniotica); redness and bulging swellings of the tip of the nose (tapir nose).

In the primary ulcerative form of mucocutaneous leishmaniasis (also known as espundia) there is rapid tissue destruction with rapid destruction of the nasal septum and deformation of the nose; destruction of the upper lip possible.

The destruction can spread to the nasopharynx with destruction of the palate and tongue. Involvement of the larynx, pharynx and trachea lead to complicating secondary processes such as hoarseness, difficulty swallowing, obstruction of the upper airways. Cause of death is often an aspiration pneumonia.

• Antimony preparations (Antimony = Stibium = Sb): Basically the remedy of choice for infections with Leishmania brasiliensis! Sodium Stibogluconate (e.g. Pentostam) contains 10% Stibium (100 mg/ml); Megluminantimonate (e.g. Glucantime) contains 8.5% Stibium (85 mg/ml). Dosage: 20 mg/kg bw/day i.m. (painful) or slowly (10-20 min.) via small caliber needle i.v. Cave! Venous thrombosis! When administered i.v. the drug should be dissolved in 50 ml 5% glucose. Therapy duration: 20 days for cutaneous forms and 28-30 days for mucocutaneous forms. If toxic side effects occur, reduce the dose by 2 mg/kg bw Sb. Clinical healing often occurs 4-6 weeks after the end of treatment. Individual uncomplicated lesions of leishmaniasis can also be treated intralesionally (apply 1-3 ml of undiluted solution from the edge of the lesion! Perform the procedure 1-2 times/week. Duration of therapy: 3-6 weeks, depending on the acuteity of the lesion. Caution! Painfulness, toxicity to heart and liver!
• Pentamidine diisethionate (e.g. pentacarinate): therapy of choice for diffuse cutaneous leishmaniasis and for the failure of the mucocutaneous forms. Dosage: 4 mg/kg bw/day once a week for at least 4 months. Recent studies also show efficacy in cutaneous leishmaniasis at a low dosage of 2 mg/kg bw i.m. every 2nd day up to a total number of 7 injections.
• Liposomal amphotericin B (e.g. Ambisome): Drug of choice if pentavalent antimony is contraindicated or ineffective. ED: 0.5-1.0 mg/kg bw, every 2nd day, 20 doses in total.
• Ketoconazole (e.g. Nizoral): drug of choice for infections by Leishmania mexicana, Leishmania panamensis and Leishmania major. Not or insufficiently effective for Leishmania brasiliensis, Leishmania tropica and Leishmania aethiopica. Dosage: 600 mg/day (evening) for 4 weeks.
• Fluconazole (e.g. Diflucan): In studies very effective against infection with L. major. Dosage: 1 time a day 200 mg p.o. for 6 weeks
• Miltefosine (Impavido): Adults and children from 3 LJ: 1,5-2,5 mg/kg bw/day for 28 days. Max. TD: 150 mg. In immunocompromised patients (HIV-infected) a longer treatment may be necessary. Promising are also the therapeutic successes with cutaneous leishmaniasis in the New World. Approval for this indication is pending in Pakistan and Colombia. Recent reports indicate that miltefosine can also be successfully used in cutaneous L. of the Old World.

1. Boggild AK et al (2019) Cutaneous and Mucocutaneous Leishmaniasis in Travelers and Migrants: A 20-year GeoSentinel Surveillance Network Analysis. J Travel Med pii: taz055.
2. Handler MZ et al (2015) Cutaneous and mucocutaneous leishmaniasis: Clinical perspectives. J Am Acad Dermatol 73:897-908; quiz 909-10.
3. Handler MZ et al (2015) Cutaneous and mucocutaneous leishmaniasis: Differential diagnosis, diagnosis, histopathology, and management.J Am Acad Dermatol 73:911-926; 927-928.
4. Tejura N et al (2019) Case Report: Mucocutaneous Leishmaniasis Masquerading as Idiopathic Midline Granulomatous Disease. Am J Trop Med Hyg 101:1107-1110.