Diffuse Cutaneous Leishmaniasis in an Immunocompromised Patient Resembling Histoid Hansen's Disease.

Cutaneous leishmaniasis is caused by protozoan parasites of the genus Leishmania. Atypical presentation and widespread progression of the lesions may be seen in patients with HIV disease and diffuse cutaneous leishmaniasis and HIV co-infection is emerging as a serious new threat. We report a case of diffuse cutaneous leishmaniasis in a HIV- infected patient resembling Histoid Hansen.

Introduction

Leishmaniasis is a vector-borne disease transmitted by sandflies, caused by protozoan of genus Leishmania. The disease can occur in following different forms—visceral leishmaniasis or kala azar, cutaneous leishmaniasis (CL), mucocutaneous leishmaniasis, and diffuse cutaneous leishmaniasis (DCL). CL can become disseminated (diffuse) in human immunodeficiency virus (HIV)-positive patients. It is clinically characterized by multiple (>200), atypical, widespread, infiltrated skin lesions full of parasites, which seldom ulcerate, respond poorly to standard antimonial treatment, and show frequent relapses.[12] Very few cases are reported in Indian[12345] and Western literature.[67] We therefore report this case from South Rajasthan.

Case Report

A 40-year-old male driver, diagnosed as HIV positive 2 years ago and presently on antiretroviral therapy (tenofovir, lamivudine, and efavirenz), sought consultation for 8 months history of generalized multiple, asymptomatic skin lesions. History revealed that the lesions first appeared on elbow followed by involvement of extremities, buttocks, ears, and face. There was no past history of prolonged fever, nasal stuffiness, and glove and stocking hypoesthesia. Cutaneous examination revealed multiple, discrete, nontender, 5mm to 2 cm sized, erythematous to skin colored, papulonodular lesions, symmetrically present on extremities, buttocks, and ear [Figure 1a–c]. The lesions on extremities were particularly prominent around the elbow and knee joints. Few lesions around the elbow had enlarged to form crusted plaques [Figure 1d]. The lesions on the face prominently involved both ears and eyebrows [Figure 1e]. However, the trunk was relatively spared. The palms and soles had similar lesions. A few lesions on palms showed umbilication and verrucosity [Figure 1f]. The general health was unaffected. There was no nerve thickening on peripheral nerve examination. Differential diagnoses of histoid Hansen's disease, histoplasmosis, post kala azar dermal leishmaniasis (PKDL), and DCL were made.

Figure 1

(a) Multiple, discrete, erythematous to skin colored, papulonodular lesions, symmetrically present on extremities. (b) Papulonodules on buttocks. (c) Multiple, nodular lesions over ear. (d) A few large, ulcerated, and crusted lesions around elbow. (e) Lesions on face prominently involved both ears and eyebrow. (f) Palmar lesions showing umbilication and verrucosity

Systemic examination was normal. Complete blood count, urinalysis, hepatic and renal function tests, chest X-ray, and ultrasonography of the abdomen were normal. His baseline CD4 count was 16 cells/mm3 and became 104 cells/mm3 after 16 months of initiation of antiretroviral therapy (ART), at the time when he started developing skin lesions. Giemsa-stained slit skin smear showed presence of abundant 2–3 μm Leishman Donovan bodies both extracellularly and intracellularly inside macrophages [Figure 2]. Skin biopsy revealed diffuse dermal infiltration of histiocytes filled with plenty of Leishman Donovan bodies [Figure 3]. Histoid Hansen disease and histoplasmosis were excluded by negative Ziehl–Neelsen and Giemsa stain, and negative PAS stain on histopathology. Patient was treated with ketoconazole 200mg twice daily for 2 months without any significant response. Later, he was started on itraconazole 200 mg twice daily dose. However, the patient was lost to follow-up.

Figure 2

Slit skin smear showing multiple Leishman Donovan bodies (red arrows) within and outside macrophages (Giemsa, ×100)

Figure 3

Diffuse dermal infiltration of histiocytes filled with plenty of Leishman Donovan bodies (red arrows) (H and E, ×40)

Discussion

Leishmaniasis is emerging as an important disease in HIV infected persons, particularly in subtropical and tropical regions. The HIV/acquired immunodeficiency syndrome pandemic is spreading at an alarming rate in Africa and the Indian subcontinent. The number of cases of Leishmaniasis/HIV coinfection is expected to rise owing to the overlapping geographical distribution of the two infections.[8]

Leishmania species can cause a wide spectrum of cutaneous lesions in HIV-positive patients, including localized cutaneous, mucosal, mucocutaneous, diffuse cutaneous, or post kala-azar leishmaniasis.[9] DCL is a rare anergic variant of cutaneous leishmaniasis in which, the lesions are disseminated, resembling lepromatous leprosy. The disease usually begins with an initial primary lesion and subsequently disseminates to involve other parts of the body due to an underlying deficiency in cellular immunity.[3]

Our case had an advanced HIV disease (CD4 count 104cells/mm3), which resulted in atypical and severe clinical presentation of CL in the form of DCL. Our patient was unusual on several accounts, namely occurrence in South Rajasthan, a nonendemic area for CL, sparing of trunk, involvement of palms and soles, and absence of mucosal or visceral affection. Mucosal involvement has been reported in some Indian studies.[14] Affection of palms and soles has not been reported in the past. Clinical features and treatment outcome of DCL coinfected with HIV as seen in previous studies is summarized in Table 1. A differential diagnosis of histoid Hansen's disease and histoplasmosis was initially entertained based on morphology of lesions, but ruled out on meticulous clinical examination, cytology, and biopsy. PKDL was ruled out because there was no history of kala azar in the past, and there were no hypopigmented lesions anywhere.

Table 1

Clinical features and treatment outcomes of DCL coinfected with HIV in different case reports

Systemic pentavalent antimonials (sodium stibogluconate and meglumine antimoniate), liposomal amphotericin B, miltefosine, and ketoconazole can be used in the treatment of CL. Diffuse cutaneous forms have a chronic relentless course and are usually refractory to treatment.[10] Due to unavailability of the antimonials, we treated the patient with azole antifungals, without any apparent clinical benefit.

To our knowledge, DCL has not been described previously from South Rajasthan and this prompted us to report this case. HIV and Leishmania both modulate host immunity in ways that may lead to difficulty in making clinical diagnosis. Clinicians should be aware of the atypical presentation of the disease in an era of HIV pandemic. There is a high risk of making an incorrect diagnosis of lepromatous leprosy instead of DCL, as the former is endemic in India.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.