Clinical Challenge: Scarred Hypertrophic Plaque on Leg - MPR

Clinical Challenge: Scarred Hypertrophic Plaque on Leg

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A 53-year-old man presents to the clinic with ulcerated, scarred, and hypertrophic plaques on both lower extremities. He is a refugee from Sudan who arrived in the United States a month earlier.  He reports that the lesions started as red bumps that slowly expanded and developed a raised border. Several of his family members in Sudan have had similar lesions in the past, and he is concerned that he may have developed the same condition.

Leishmaniasis is a disease caused by intracellular protozoan parasites predominantly found in tropical areas of the world. Leishmania-like species have been discovered in amber dating back more than 100 million years.1 Archeologists discovered evidence of leishmaniasis in samples from Egyptian mummies as...

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Leishmaniasis is a disease caused by intracellular protozoan parasites predominantly found in tropical areas of the world. Leishmania-like species have been discovered in amber dating back more than 100 million years.1 Archeologists discovered evidence of leishmaniasis in samples from Egyptian mummies as well as tablets from ancient Arabic societies.1

William Boog Leishman, a Scottish pathologist, discovered ovoid bodies in smears taken from the spleen of a soldier who died from emaciation and splenomegaly while in Calcutta, India, in 1900.1 Around the same time, Charles Donovan, a physician and professor of physiology, discovered similar ovoid bodies in splenic smears from Indian patients who had fever and splenomegaly.1 These 2 physicians are credited with the discovery of the parasite that causes visceral leishmaniasis.1

Leishmaniasis is categorized into 3 types: cutaneous, mucocutaneous, and visceral. Cutaneous leishmaniasis can be found in many places around the world, specifically in Latin America, Africa, Asia, and southern Europe. An estimated 350 million people are at risk for the disease; the worldwide prevalence is 12 million, with an annual incidence of cutaneous leishmaniasis of 1.5 million cases.2 The condition is particularly prevalent in the Middle East and North African countries, such as Afghanistan, Iran, Iraq, Morocco, Pakistan, Saudi Arabia, Sudan, Syria, and Yemen.3

Transmitted via an insect bite of infected female phlebotomine sand flies, leishmaniasis is caused by intracellular protozoan parasites of the genus Leishmania.4 Specifically, when a sand fly bites a host, it transmits the parasite in the promastigote form through its proboscis. These promastigotes are then phagocytosed by the host macrophages, transformed into amastigotes and multiply, and then spread to different tissues of the body. Sandflies can then have a blood meal on an infected host and become infected with amastigotes, which migrate to the gut of the sand fly to multiply. These amastigotes develop into promastigotes and then migrate to the sand fly’s proboscis to repeat the process when having a blood meal on the next host.4

The amastigotes within the macrophages are termed Leishman-Donovan bodies and can be visualized histologically.2 Initially, a wide variety of inflammatory cells are present at the site of infection. Histologic appearance of chronic lesions is characterized based on the strength of the immune response of the host. If the immune response is sufficient, then epithelioid granulomata with few parasites are seen. If the immune response is poor, then a diffuse macrophage infiltrate with many parasites is seen. The immune response is T-cell-mediated, but the outcome differs in Th1 lymphocyte vs Th2 lymphocyte responses. Generally, the Th1 lymphocyte response leads to better outcomes.2,5

The cutaneous form of leishmaniasis is caused by approximately 14 different species of protozoa within the genus Leishmania, including L mexicana, L brasiliensis, L donovani, among others.2 Knowing the species of Leishmania that infected a patient can be useful because certain species are associated with prolonged, more severe courses of disease.

Risk factors for cutaneous leishmaniasis include living in or visiting endemic regions or urban areas, warmer climate, environmental factors such as proximity of housing to dams and cowsheds, cracks in the walls of houses that encourage sand fly aggregation, and a suppressed immune system such as in individuals with HIV.6 Additionally, travelers and military personnel are at risk.7

Clinically, leishmaniasis appears initially as a small papule. If the lesion enlarges and develops an area of central necrosis, resembling the appearance of a volcano, then the lesion is termed a “wet” lesion. 2,7 However, if the initial small papule either remains smooth or becomes hyperkeratotic, then the lesion is called a “dry” lesion.2,7 The outcome associated with a wet or a dry lesion can vary with different species of Leishmania. The edge of the lesion is thickened, and ulceration rarely extends into the subcutaneous tissue. Lesions develop on exposed areas of the skin where the sand fly can get access for a blood meal; however, satellite lesions may also develop due to the local spread of parasites. The size of the lesion is typically <5 cm in diameter but can reach up to 10 cm.2

The differential diagnosis of leishmaniasis can be broad, primarily due to considerable variation in presentation of the lesions. Several conditions that may mimic leishmaniasis include infected insect bites, ulceroglandular tularemia, cutaneous anthrax, cutaneous tuberculosis, and leprosy.2,7 It is also important to include noninfectious conditions such as basal cell carcinoma, squamous cell carcinoma, cutaneous lymphoma, and pyoderma gangrenosum within the differential.2,7

A diagnosis is made by performing a 4-mm punch biopsy at the edge of the lesion and histologic examination. Classically, the presence of amastigotes within macrophages is specific for a diagnosis of leishmaniasis.7 The amastigotes may also be visualized in skin scrapings or smears. Polymerase chain reaction is being used increasingly as a method to detect Leishmania DNA and make the diagnosis, as it is the most sensitive technique.7

Prevention is essential for controlling leishmaniasis. This can be accomplished by the use of nets and insecticide, especially while sleeping.7 Once an individual has developed cutaneous leishmaniasis, treatment methods include pentavalent antimonials (meglumine antimoniate and sodium stibogluconate), paromomycin, miltefosine, and pentamidine.2,7 The size of the lesion should decrease after several weeks of treatment. If left untreated, it may take several months to a year for the ulcers to self-heal. Disfiguring scars can often appear. Therefore, the goals of treatment are to prevent local dissemination of parasites, decrease the size of the scar, and accelerate the healing process.7,8

The patient in this case underwent a punch biopsy of his lesion, and histologic examination revealed Leishman-Donovan bodies. The Centers for Disease Control and Prevention was notified, and the patient was referred to an infectious disease specialist for treatment.

Yelena Dokic, BSA, and Eleanor Johnson, BS, are medical students at Baylor College of Medicine, and Christopher Rizk, MD, is a dermatologist affiliated with Elite Dermatology in Houston, Texas.

References

  1. Steverding D. The history of leishmaniasisParasit Vectors. 2017;10(1):82.
  2. Bailey MS, Lockwood DN. Cutaneous leishmaniasis Clin Dermatol. 2007;25(2):203-211.
  3. Postigo JAR. Leishmaniasis in the World Health Organization Eastern Mediterranean region Int J Antimicrob Agents. 2010;36(Suppl 1):S62-S65.
  4. Gossage SM, Rogers ME, Bates PA. Two separate growth phases during the development of Leishmania in sand flies: implications for understanding the life cycle Int J Parasitol. 2003;33(10):1027-1034.
  5. Ghersetich I, Menchini G, Teofoli P, Lotti T. Immune response to Leishmania infection in human skinClin Dermatol. 1999;17(3):333-338.
  6. Desjeux P. The increase in risk factors for leishmaniasis worldwideTrans R Soc Trop Med Hyg. 2001;95(3):239-243.
  7. Murray HW, Berman JD, Davies CR, Saravia NG. Advances in leishmaniasis Lancet. 2005;366(9496):1561-1577.
  8. Shin JY, Lee YB, Cho BK, Park HJ. New world cutaneous leishmaniasis treated with intralesional injection of pentavalent antimony.  Ann Dermatol. 2013;25(1):80-83.