Giant Chronic Cutaneous Leishmaniasis: Case Report of an Unusual.

Sir,

Leishmaniasis consists of a wide spectrum of clinical presentations caused by infection with intracellular flagellate protozoan parasites belonging to the genus Leishmania. Leishmaniasis is a vector-borne disease transmitted by bite of infected female sandfly. Atleast 20 species of Leishmania are known to cause human infection and produce distinct clinical forms. Based on the extent and severity of involvement, leishmaniasis is divided into four broad groups - cutaneous leishmaniasis (CL), diffuse cutaneous leishmaniasis (DCL), mucocutaneous leishmaniasis (MCL), and visceral leishmaniasis (VL).[1]

The most common form of leishmaniasis is CL. It is mainly seen in adults between 20 and 40 years of age on exposed body parts.[2] The diverse clinical spectrum of CL is dependent on numerous factors, such as the type and duration of clinical lesion, strain of organism, geographic location, parasitic load, disease reservoir, and host immune competence.[34]

A 62-year-old male patient presented with a single, large, erythematous, infiltrated plaque of size 26 cm × 16 cm covering mainly the left side of the trunk for more than 10 years. The lesion showed superficial erosions with crusting and purulent discharge. Outer border of the lesion was elevated and well-defined, with multiple inner elevated rings giving the lesion a gyrate appearance [Figure 1].

Figure 1

Clinical picture showing giant plaque with multiple raised rings giving gyrate appearance

The lesion started as a small pea-sized asymptomatic papule probably caused by an insect bite 10 years earlier. Initially, the lesion regressed, though incompletely, and then reappeared with a gradual increase in size. It reached its present size in approximately 4 years, and subsequently, showed very slow spread with no sign of regression. Repeated superficial erosions, crusting, and pus discharge were seen. Medical history included pulmonary tuberculosis 2 years previously for which 6 months of antituberculosis treatment (AKT category I) was taken.

Considering the clinical differential diagnosis of chronic pyoderma gangrenosum, cutaneous leishmaniasis, subcutaneous mycosis, and atypical mycobacterial infection, skin biopsies were taken. Patients’ routine investigations showed – Hb – 11g%; total WBC count – 12,000/cmm with 86% neutrophils; ESR – 30 in 1st hour; CRP – mildly elevated; renal and liver function –within reference range. Chest X-ray showed right upper lobe and left lower zone fibrotic Koch's. Ultrasonography of the abdomen was normal. Serum Rapid Plasma Reagin and serology for hepatitis B and C and human immunodeficiency virus were nonreactive.

Histopathology showed irregularly acanthotic epidermis and diffuse chronic inflammatory infiltrate in upper and mid dermis. Hematoxylin and eosin (H and E) section revealed numerous intracellular and extracellular organisms which were confirmed on Giemsa stain to be Leishman bodies [Figure 2a and b]. Periodic Acid Schiff (PAS) stain for fungi and AFB stains for lepra bacilli and tuberculous bacilli were negative. Clinical and histopathological findings led to the diagnosis of chronic cutaneous leishmaniasis. As it was not possible to avail polymerase chain reaction (PCR) and culture, species identification was not done.

Figure 2

(a) H and E sections showing large number of intra and extracellular LD bodies (×10). (b) Close view of section showing multiple intracellular LD bodies(×40)

Patient was given amphotericin B 50 mg daily for 21 days, followed by oral itraconazole 200 mg daily. Weekly liquid nitrogen cryotherapy by cryojet was also administered. As sodium stibogluconate or miltefosine could not be procured due to lack of availability; oral itraconazole and weekly cryotherapy were continued for 6 weeks. Lesion started showing signs of regression in the form of flattening of entire plaque as well as disappearance of rolled out edges and appearance of scar tissue in 6 weeks [Figure 3]. The patient was lost to follow-up after 6 weeks.

Figure 3

Posttreatment partial resolution of lesion (after 6 weeks)

Leishmaniasis is prevalent in approximately 88 countries in the world, with old world disease more prevalent than new world disease.[5] In India, most cases are reported form Rajasthan, Punjab, Himachal Pradesh, Delhi, and Uttar Pradesh.[6]

Incubation period for CL is 2–8 weeks. The disease begins as a small erythematous papule at the site of the bite of sandfly. It slowly increases in size upto 2 cm or more and becomes a nodule. Central crusting and shallow ulcers develop with raised border.[7] Lesion persists for few months to a year and gradually heals leaving a slightly depressed scar. L. tropica generally produces chronic lesions or treatment resistant lesions.[1]

Diagnosis of leishmaniasis can be done by tissue smear which may show organisms as amastigotes on Giemsa stain. Culture on Novy-McNeal-Nicole medium or PCR may help in species identification.[178] Biopsy from lesion shows diffuse dermal infiltrate composed of varying proportion of histiocytes, lymphocytes, plasma cells, and neutrophils. Amastigotes may be found within dermal macrophages on H and E stain which can be confirmed with Giemsa stain. Immunological diagnosis with enzyme-linked immunosorbent assay (ELISA), Leishman skin tests are not very useful or easily available.

Most CL lesions are self-healing. However, persistent lesions, mucocutaneous lesions and larger lesions require systemic therapy. Sodium stibogluconate, intramuscular, intravenous, or intralesional, is the first line therapy. Other effective agents include pentamidine isethionate injections, oral miltefosine, intravenous amphotericin B, oral azoles including ketoconazole, fluconazole, and itraconazole etc., Local paramomycin ointment, cryotherapy, and heat therapy also aid in resolution of lesion.[7]

Our case showed an unusual presentation of single chronic CL on the trunk reaching a huge size. In contrast to self-resolution seen in many cases of CL, our case did not show any sign of resolution over 10 years. Even the number of organisms seen in our patient after more than 10 years was phenomenal; as with time it is difficult to find organisms on smear or histopathology in most cases. Inability to avail species identification due to lack of facility is a limitation of our study. As CL can present in very unusual forms, any nonhealing chronic lesion even on unexposed body parts should be investigated for leishmaniasis, particularly in endemic areas.

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Conflicts of interest

There are no conflicts of interest.