+ What is the Most Likely Diagnosis
Mucocutaneous Leishmaniasis
Although you don’t have access to diagnostic tools these children most likely have leishmaniasis based on clinical exam findings and historical information. This patient has multiple clinical factors that make leishmaniasis the likely diagnosis. Multiple people in the home have similar lesions, the lesions are painless and in varying stages of ulceration, and the family lives in an area undergoing active urbanization. Due to the mutilating effects of prolonged infection with leishmaniasis this patient's presentation warrants treatment.
Leishmaniasis is caused by the Leishmania parasite which is transmitted through the bite of the female sandfly and can manifest as cutaneous, mucocutaneous, or visceral disease. There are more than 20 species of Leishmania parasite that result in 600,000 – 1,000,000 new cases annually. Leishmaniaisis is endemic in Guatemala and commonly manifests as cutaneous and mucocutaneous forms of the disease. The bite of the female sandfly is typically painless making it difficult to identify the onset of disease. Once a host is infected, the parasites invade macrophages and replicate intracellularly. The most common species in Central America are L. braziliensis, L. Mexicana, and L. guayanensis. L. braziliensis and L. guyanensis are responsibly for the majority of mucocutaneous disease. There can be multiple species of Leishmania endemic in a single area and some people who are infected may never exhibit symptoms. Visceral, also known as old-world, leishmaniasis is found primarily in East Africa, the Middle East, Brazil, and India.
Cutaneous disease presents as small painless papules that ulcerate over time. Cutaneous lesions can heal without treatment in 3-60 months but do cause significant scaring. You may have heard of “Chiclero’s ulcer” which is an ulcer on ears of gum tree harvesters caused by L. Mexicana. Mucocutanouse disease presents as multiple painless ulcers in various stages of healing, intermittent oozing which is concerning for bacterial superinfection, and multiple family members in same home with similar lesions. Mucocutaneous disease typically occurs on the face and can cause debilitating deformities of the nose. Mucocutaneous disease can appear years after initial cutaneous lesions have healed, highlighting why proper treatment of initial cutaneous infection is important. Traditionally, mucocutaneous disease is diagnosed with skin scraping and Giemsa stain. However, in areas with limited diagnostic availability empiric treatment of mucocutaneous disease based solely on clinical presentation is common.
There are a variety of treatment options that can be used to treat leishmaniasis. Pentavalent antimonial, which is IV sodium stibogluconate and IM meglumine antimoniate, are considered first line and have an efficacy of 91%. Both of these medications have multiple side effects, most notably pancreatitis, QTc prolongation, hepatitis, and bone marrow suppression. Amphotericin is another medication that is FDA approved for treatment of cutaneous disease. Treatment is typically dependent on parasite species and local availability of medications. Cutaneous lesions can be treated topically with local infiltration of lesions with pentavalent antimonials though providers must be careful to only inject dermis as these medications are quickly absorbed from subcutaneous tissue and can have systemic effects. Médecins Sans Frontières recommends waiting to initiate treatment unless lesions have been present for more than 6 months.
Although leishmaniasis is classically a disease of Latin America and the Middle East, with increasing migration to North America there have been reported cases of mucocutaneous disease along the southern US border, in Texas and Oklahoma. Lesions have also been reported in military personnel returning from tours in the Middle East.