Post-kala-azar dermal leishmaniasis

ARTICLE

Post-kala-azar dermal leishmaniasis (PKDL) is a dermatosis seen in patients with a previous history of kala-azar or visceral leishmaniasis. The first person to describe such an entity was Brahmachari, who, at the Asiatic Society of Bengal in 1922, presented four patients with skin eruptions and plaques, 6 months to 2 years after treatment of kala-azar [1]. Leishman-Donovan bodies (LDB) were demonstrated by slit-skin smears and the term dermal leishmanoid was adopted. Shortly after, the term Brahmachari's dermal leishmaniasis was suggested to avoid confusion with other types of leishmaniasis. Later work was done on proving that organisms causing dermal leishmaniasis were the same as those causing kala-azar and when this was finally proven, the entity was renamed post-kala-azar dermal leishmaniasis. This condition is mainly confined to the Indian subcontinent although there have been reports of this disease in parts of Africa, China, and Iraq. We report here a case of PKDL in a 5-year-old Iranian boy. This is only the second report of this condition from Iran [2].

Case report

A 5-year-old Iranian boy presented with multiple, asymptomatic papules on his trunk and extremities of 6 weeks' duration. His past medical history was not significant except for one hospitalization for kala-azar 4 years earlier which had been completely cured. No similar lesions were reported among his immediate family members. General physical examination was normal. No lymphadenopathy or mucous membrane involvement was observed. Cutaneous examination revealed multiple skin ­ to coppery-red ­ colored papules over the patient's trunk, especially on the neck (Fig. 1), and around the axillae and groin. Scattered papules were observed on the extremities and a brownish, figurate discoloration was present on both his legs (Fig. 2). A skin biopsy from one of the papules was performed. Histopathological examination revealed a severe, lymphohistiocytic infiltration in the upper and lower dermis with numerous Leishman bodies (Figs. 3 and 4). A diagnosis of post-kala-azar dermal leishmaniasis (PKDL) was made and treatment was started with a pentavalent antimonial compound (Glucantime), 20 mg/kg as daily, intra-muscular injections for 20 days. During this time period, the patient showed a dramatic response and the papules almost completely disappeared. The brownish, figurate discoloration also faded to some extent, although further evaluation of the patient's response to treatment was not possible due to loss to follow-up.

Discussion

Recent data has shown that PKDL is seen after the visceral disease has healed either spontaneously or as a result of treatment, in approximately 5% of East African patients and 20% of Indian patients [3]. The onset of the disease differs with respect to the geographical area under study. In Africa, the condition usually begins during convalescence, but in India, the onset of disease is typically 1-2 years after recovery from kala-azar, although longer intervals have also been reported. The age of predilection is usually between 20 and 40 years of age with an equal sex distribution, although some believe that it occurs more frequently in men [4]. The number of cases reported beyond 40 years and in children under 10 years is very low. Three main types of lesions [5], of which one or two may predominate, can be seen in patients with PKDL and include:

(a) Erythema and induration with the initial site of involvement being the butterfly area of the face. Involvement of the trunk and extremities may also be observed. Lesions resembling this erythematous induration have been described by some as brownish discoloration [1, 6]. On rare occasions the erythema may resemble erythroderma.

(b) Pin-point, hypopigmented macules which gradually increase in size having an irregular margin. They tend to be symmetrical and may be either generalized or localized to the extremities and trunk. Hypopigmented macules may form large figurate areas by coalescence, leaving islands of normal skin in between.

(c) Papules and nodules arising in the affected area or on apparently normal skin. Usually the central area of the face, the forehead, and the chin are affected. The condition may also affect the whole of the face, the trunk, and the extremities, with special affinity for the genitalia. Large plaques may be seen but no ulceration occurs.

Apart from the three main types of presentation, unusual variants of PKDL have been reported and include the annular type, xanthomatous transformation of chronic nodules, hypertrophic forms, and the presence of lesions in unusual sites such as eyelids, palms, and perionychium [4]. Mucous membrane involvement as well as laryngeal, ocular, nasopharyngeal and esophageal involvement have also been reported [4].

Histopathlogical examination of the various lesions described in PKDL usually reveals a polymorphic infiltrate of macrophages, plasma cells and lymphocytes. In most cases, a variable number of Leishman bodies can be detected in dermal macrophages. Leishman bodies may also be demonstrated in slit-skin smears taken from typical PKDL lesions.

PKDL usually follows a chronic course. The cases occurring in East Africa are usually not treated and the condition subsides spontaneously. This form is believed to be due to the gradual development of specific cellular immunity clearing up the scattered parasites remaining in the skin. However, cases from the Indian subcontinent must be treated. It is presumed that this type represents failure of cellular immunity against a dermotropic variant of L. donovani. Intra-muscular injections of pentavalent antimonials for 4 to 5 months alone or in combination with ketoconazole and allopurinol have been recommended [5, 7]. In resistant cases, amphotericin B has been used as a suitable alternative [8]. A very important point to consider is that skin lesions are a potential source of infection to the community.

Our case, as well as the previous cases reported from Iran, have all been young children. The clinical picture of PKDL in the Iranian cases reported to date appears to be more similar to the cases reported from India. However, response to treatment seems much faster in the Iranian cases. More cases need to be reported from Iran in order to be able to present the various characteristics, which may be unique to PKDL in Iran.

REFERENCES

1. Brahmachari UN. A new form of cutaneous leishmaniasis, dermal leishmanoid. Ind Med Gaz 1922; 57: 125-7.

2. Kumar PV, Handjani F, Baghestani S. Post-kala-azar dermal leishmaniasis in Iran (abstract booklet). The Fourth Asian Dermatological Congress, Dubai, UAE, January 13-15, 1996.

3. Bryceson ADM, Hay RJ. Parasitic worms and protozoa. In: Champion RH, Burton JL, Ebling FJG, eds. Textbook of dermatology, 5th ed., Vol. 2. Oxford: Blackwell Scientific Publications, 1992: 1251-63.

4. Ramesh V, Mukherjee A. Post-kala-azar dermal leishmaniasis. Int J Dermatol 1995; 34: 85-91.

5. Anonymous. Control of the leishmaniases. Report of a WHO Expert Committee, Tech Rep Ser, Wld Htlh Org, Geneva, n° 793, 1990.

6. Dey NC. A case of post-kala-azar dermal leishmaniasis. Ind Med Gaz 1929; 64: 147-8.

7. Ramesh V, Misra RS, Saxena Y, Mukherjee A. Post-kala-azar dermal leishmaniasis. A clinical and therapeutic study. Int J Dermatol 1993; 32: 272-5.

8. Ramesh V. Treatment of post-kala-azar dermal leishmaniasis. Int J Dermatol 1994; 33: 153-6.