Post-kala-azar dermal leishmaniasis: a manual for case management and control

Overview

Post-kala-azar dermal leishmaniasis (PKDL) is a sequela of visceral leishmaniasis that appears after patients have apparently been cured of visceral leishmaniasis. PKDL has also been reported in patients without a history of visceral leishmaniasis. PKDL appears as a macular, papular or nodular rash, or a combination of these, typically on the face, but it may subsequently affect all parts of the body. PKDL may also affect the conjunctival, nasal, oral and genital mucosa. There is increasing evidence that pathogenesis is largely immunologically mediated.

PKDL is prevalent in all areas where Leishmania donovani is endemic (that is, in East Africa especially in Sudan, and on the Indian subcontinent, especially in Bangladesh (Figure 1). It does not occur as a sequela of visceral leishmaniasis caused by infection with L. infantum, but it has been observed sporadically in patients coinfected with HIV and L. infantum. There are differences between the two continents: in Sudan, for example, up to 50% of patients develop the condition after visceral leishmaniasis has apparently been cured, and the severe forms occur mostly in children. In East Africa, PKDL is mostly self-healing. In Sudan, PKDL may occur soon after treatment for visceral leishmaniasis or even concurrently with the disease. On the Indian subcontinent, PKDL occurs after visceral leishmaniasis in about 5–15% of cases, often after a 2–3 year interval; it affects children and adults equally.

The main known risk factor associated with the development of PKDL is previous treatment for visceral leishmaniasis with antimonials; however, PKDL also occurs after treatment with other medicines. The rate of PKDL occurring after patients have been treated with new therapies for visceral leishmaniasis, such as paromomycin or miltefosine, is unknown; patients will need to be followed closely to evaluate the effects of these treatments on the development of PKDL.

Noting this fact, the leishmaniasis control programme at WHO’s headquarters collaborated with the vector-borne disease-control programme of the Regional Office for South-East Asia to organize a consultative meeting in July 2012 in Kolkata, India. At the meeting, experts reviewed the epidemiology of PKDL, as well as case-management, prevention and control strategies. They also reviewed recommendations made by the PKDL consortium in June 2012.