Post - Kala - Azar - dermal - leishmaniasis: An unusual presentation from Uttarachal (A non - endemic hilly region of India)

Introduction

PKDL develops in 5% of cases following kala-azar which is endemic in the North - eastern states of India like Bihar, West Bengal, Assam and plains of eastern Uttar Pradesh. It usually presents as hypopigmented macules, erythematous or juicy papules, nodules and plaques on face, limbs and trunk, usually appearing in that order. We report a patient from a non - endemic hilly region (Uttaranchal) presenting with a single verrucous plaque on chin.

Case Report

A 26-year-old, male, army personnel presented with a single large, erythematous firm plaque studded with juicy papules producing papillomatous appearance on chin for last 1 - year. The lesion started as a few coalescing papules which progressed to form a plaque. There was no history of preceding hypopigmented macules or erythematous plaques anywhere on the body. He gave history of suffering from kala-azar 4 years back which was treated with injections SAG IM OD for 20 days. He had been staying In the hilly regions since birth and had not visited or stayed in any region of the country endemic for KA/PKDL. He had been treated with courses of antibiotics and topical steroids without any benefit before presenting to us. There was no family history of similar illness in the family. His general physical and systemic examination were normal.There was no lymphadenopathy. Cutaneous examination revealed a single, erythematous, 12x 8 cm, verrucous plaque studded with 2-5 mm papules covering his entire chin and involving the lower lip [Figure - 1]. On close examination, three, 0.5 - 1.0 cm hypopigmented macules were noted on the eyelids and forehead. The patient was not aware of these lesions. There was no hypoaesthesia in these lesions. Peripheral nerves were not thickened. Rest of the cutaneous examination was within normal limits. Clinical differential diagnoses of granulomatous skin conditions such as leishmaniaisis, cutaneous tuberculosis, Hansen′s disease with reaction and sarcoidosis were considered. Results of routine hemogram, liver and kidney function tests, urinalysis, and chest x-ray were normal. Slit - skin smears for M. leprae were negative. Skin biopsy from the plaque showed atrophic epidermis with marked chronic granulomatous inflammatory infiltrate rich in plasma cells, lymphocytes, macrophages and epithelioid cells in the dermis. Giemsa stain revealed leishmania donovan bodies in the macrophages. The patient was treated with injection SAG 20 mg/kg daily intramuscular for initial 40 days followed by intravenosly for next 20 days with complete clearance of the lesions.

Discussion

Leishmaniases are a group of diseases caused by several species of the genus leishmania. Each species tends to occupy a particular zoo - geographic zone.[1] Currently, leishmaniasis is endemic in 88 countries. The overall prevalence is 13 million cases. A common estimate of the incidence per year is 1.8 million newly reported cases. The incidence as well as geographic distribution of leishmaniasis is increasing.[2]

In India, different clinical forms of lesihmaniasis such as cutaneous leishmaniasis (CL); kala - azar (KA) or visceral leishmaniasis (VL) and PKDL occur in the varied geographic regions of this vast land where the conditions suitable for the respective causative species of leishmaniasis and their vectors are found. CL occurs mostly in the north - western desert and semi -arid regions of Rajasthan.[3] Indigenous cases have been reported from the hilly regions of western ghats of Kerala,[4],[5] and Assam.[6] A case of disseminated cutaneous leishmaniasis was reported from Chandigarh.[7]

In India, KA has its home in the plains of the Ganges and Brahmaputra. It occurs endemically and epidemically in the eastern sector of the country in the states of Assam, West Bengal, Bihar eastern districts of UP, foothills of Sikkim and to a lesser extent in Tamil Nadu and Orissa.[8] Following massive insecticide spraying campaigns for malaria eradication between 1958 and 1964, KA and CL declined dramatically. However, the incidence started rising gradually in the late 1960s and early 1970s. Currently KA is endemic in 30 districts of Bihar and 9 districts of West Bengal. Another endemic focus has been the state of Tamil Nadu. A few sporadic cases of VL have been reported from Gujarat, Uttar Pradesh, Madhya Pradesh, Punjab, foothills of Himalayas and Jammu and Kashmir.[2]

KA is mostly confined to the plains; it does occur in altitudes above 2000 feet. Developmental projects such as forest clearing and cultivation projects, large water resources schemes and colonization and resettlement programmes are exposing more people to leishmaniasis by bringing human being into areas of high vector and resource concentration.[2]

PKDL was first described by Brahmachari in 1922.[9] It is a late complication of KA It occurs in 5% of cases of KA in India. The figure is high (20%) following African KA.[1] The skin lesions usually appear 1-2 years after all signs and symptoms of VL have disappeared. Occasionally lymphadenopathy and bone marrow involvement may be seen concurrently. Majority of the patients give history of suffering from or having treatment for KA in the past. A variety of skin lesions such as hypopigmented macules, erythema, butterfly rash, juicy nodule, and plaques are seen. Rare types such as verrucous, papillomatous, hypertrophic and xanthomatous lesions most commonly affecting the nose and chin have been reported. Hypopigmented macules 1-10mm, discrete and coalescing, involving bilaterally symmetrically are usually the first lesions to appear. They gradually become infiltrated and evolve into papules and nodules most commonly on the chin and nose. After months or years papules and nodules coalesce to form plaques. Mucosal involvement in the form of papules/nodules on the tongue, lips, buccal mucosa and glans penis can occur. Verrucous lesions are reported on the hands, feet, nail folds whereas papillmatous lesions affect the nose and chin.[10]

Cases of KA and PKDL do occur in the eastern parts of Uttar Pradesh adjoining the endemic state of Bihar. To the best of our knowledge after literature search, no case has been found to be reported so far from the hills of uttaranchal, a newly created hilly state in the Northern India. Our patient hails from Narendranagar town in the Garhwal region of the Himalayas situated at a height of 3440 feet above sea level.

De novo presentation of PKDL as a verrucous plaque is unusual. Hilly regions of Uttaranchal may be a focus of KA and PKDL. The present case report highlights the changing geographic distribution and spread of leismaniasis in India with implications for its controls as a public health problem.