Leishmaniasis of the lip diagnosed by lymph node aspiration and treated with a combination of oral ketaconazole and intralesional sodium stibogluconate.

A 26-year-old male who presented to the dermatology OPD with complaints of a swelling on his lip of 6 months' duration was on examination found to have a solitary ulcerated nodule over the lip and an enlarged submental lymph node. Skin smear and biopsy from the lesion did not yield the diagnosis. Needle aspiration from the draining lymph node revealed the diagnostic Leishman-Donovan bodies. The patient responded to treatment with a combination of oral ketoconazole and intralesional sodium stibogluconate. We report this case because of both the unusual location of the lesion and the unusual method of diagnosis and treatment.

Introduction

Leishmaniasis is a protozoal disease transmitted by the bite of the sand fly infected with leishmania parasites. Economic development, deforestation, migration from rural to urban areas, and the development of new settlements are responsible for the global spread of the sand fly vector as well the expansion of the reservoir of leishmaniasis. Infection may be restricted to the skin in cutaneous leishmaniasis, to the mucous membranes in mucosal leishmaniasis, or may affect internal organs in visceral leishmaniasis. We present a case of cutaneous leishmaniasis, which is interesting because of the rare location of the lesion, the fact that it could be diagnosed only with FNAC of the lymph node, and because it was treated successfully with a combination of drugs that has not been described in literature earlier.

Case Report

A 26-year-old male, a native of Rajasthan, presented to the dermatology OPD with complaints of swelling over his upper lip of 6 month's duration. The patient had initially noticed a small red raised lesion on the middle part of the upper lip which was not painful, not itchy, and not fluid-filled. The swelling progressively increased in size over a few weeks and then spontaneously ruptured in the center, forming an ulcer. There was crusting and bleeding from the ulcer. There was no history of fever, loss of weight, cough, or other respiratory complaints. There was no history of travel to any country endemic for leishmaniasis. Treatment with multiple drugs, including antibiotics, by various medical practitioners had not caused regression of the lesion.

On examination, the vital parameters were normal. There was no hepatosplenomegaly. All other systems were also normal. Local examination of the face revealed a 4 × 3 cm nodular, indurated, swelling with well-defined edges over the upper lip and extending into the labial mucosa [Figure 1]. The center of the nodule was ulcerated and tender, with overlying crusting and pus discharge. There was a solitary 1 × 1 cm enlarged, nontender, submental lymph node on the left side. A clinical diagnosis of cutaneous leishmaniasis was made, with insect bite reaction and cutaneous tuberculosis kept in mind as remote possibilities.

Figure 1

Ulcerated nodule on the upper lip

Investigations revealed a total leukocyte count of 7800/mm3and absolute eosinophilic count of 1050/mm.[1] All other hematological, biochemical, and radiological investigations were normal. VDRL and ELISA for HIV were negative. Slit-skin smear from the lesion showed lymphocytes with plasma cells, but no diagnostic clue was obtained. Skin biopsy from the edge of the lesion showed well-defined lymphocyte granulomas, with few plasma cells [Figure 2]; however no definite diagnosis could be made. As we were not able to confirm the diagnosis with these usual tests, we carried out fine needle aspiration cytology (FNAC) from the draining lymph node. Slides from the aspirate showed macrophages filled with Leishman-Donovan (LD) bodies [Figure 3]. A few LD bodies were seen outside the cells also. The diagnosis of cutaneous leishmaniasis was thus confirmed.

Figure 2

Photomicrograph of histopathology of lip lesion

Figure 3

FNAC from submental lymph node demonstrating classical LD bodies

We treated the patient with intralesional sodium stibogluconate once a week for 4 weeks and tablet ketoconazole 400 mg once daily for the same duration. Two weeks after the start of treatment, the lesion had dried up and had started regressing in size [Figure 4]. The lesion completely regressed, without any residual scarring or pigmentation, over 4 weeks. The patient has now been followed up for 1 year and shows no signs of relapse.

Figure 4

Post treatment photographs

Discussion

Leishmaniasis is a protozoal disease that occurs widely throughout the world, with a global yearly incidence of 2 million new cases.[2] The geographic distribution of cutaneous leishmaniasis is mainly determined by the sand fly vectors Phlebotomus and Lutzomyia. In India, simple cutaneous leishmaniasis is usually caused by Leishmania tropica, and man is the most common reservoir. Leishmaniasis may have varying presentations: from the simple cutaneous forms to fatal systemic disease. Factors affecting the disease spectrum include species and number of parasites, site of bite, last non-blood meal of the sandfly vector, as also the immune status and nutritional status of the host. Cutaneous leishmaniasis may occur anywhere on the body but the most likely sites are the exposed parts. Most patients have one or two lesions, but the lesions may be multiple and there may be sporotrichoid nodular lymphangitis. Very few cases of isolated lip leishmaniasis have been reported from India earlier.[3] Our patient had a solitary ulcerated nodule with an enlarged draining lymph node.

The diagnosis of cutaneous leishmaniasis is mainly clinical. The parasite may not be demonstrated by the most efficient methods in tissue and by culture. Impression smears and culture in Novy-Nicolle-MacNeal (NNN) media are the gold standards, though polymerase chain reaction is being increasingly used these days. In a study from Columbia, the diagnostic yield of cutaneous leishmaniasis from different methods for all patients was as follows: histopathology 14%, impression smear 19%, dermal scraping 22%, aspirate-culture 58%, and biopsy-culture 50%.[1] In a study of 475 cases due to L major in Saudi Arabia, the parasite could not be demonstrated by smear, skin biopsy, or culture in 10%–20% of the patients, thus giving further evidence that a proportion of cases may remain undiagnosed by all methods.[4] In a study of 100 cases, FNAC from the lesion was shown to have remarkably high sensitivity (89%) and specificity (100%) for diagnosing cutaneous leishmaniasis.[5] Only one case has been reported in literature so far of diagnosis of cutaneous leishmaniasis by FNAC of a submandibular lymph node.[6]

Because of the diverse and varied presentation of cutaneous leishmaniasis, there is no single optimal treatment for all forms. Local injection of sodium stibogluconate, cryotherapy, local excision, and electrodesiccation are advocated for early noninflamed lesions.[7] The pentavalent antimony derivatives sodium stibogluconate and meglumine antimoniate that were developed in the 1940s still remain the mainstay of systemic treatment, though toxicity is very common. Rifampicin, aminosidine, pentamidine and ketoconazole have also been found to be effective.[8] The most promising oral drug today is miltefosine. The individual efficacy of intralesional sodium stibogluconate and Ketoconazole have been proved in several studies.[910] However, a combination of the two drugs has not been tried earlier. Our patient responded to treatment with intralesional sodium stibogluconate and oral ketoconazole, and we were thus able to minimize the duration of treatment and the side effects of both medications. This case is unique because of the rare location of the lesion, the diagnosis of the condition from lymph node aspiration alone, and the unique combination of drugs used for treatment.