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Background:Adult T-cell leukemia/lymphoma (ATL) is caused by human T-cell lymphotropic virus type-1 (HTLV-1). India is considered as a nonendemic region for HTLV-1. Recent upsurge of cases have been noted in southern parts of India.Aims and objectives:The objective was to describe skin manifestations in various types of ATL.Materials and Methods:Clinical examination, blood investigations, skin biopsies, lymph node biopsies, and immunohistochemistry were performed in five patients. Flow cytometry was performed in two cases.Results:Serological testing was positive for HTLV-1 in all patients. All patients presented with skin lesions. Rare presentations of molluscum contagiosum like papules, purpuric macules and plaques, hypopigmented macules and verrucous papules were seen. Dermatophytic infections occurred in two patients. Mucosal lesion was seen in one patient. Histological features include dermal lymphoid infiltrate with or without epidermotropism. Presence of epidermotropism did not correlate with the severity of disease. All patients except one succumbed to illness within few months to 1 year period.Conclusions:ATL manifest in myriad presentations and skin lesions are often the earliest manifestation. Cutaneous manifestations of ATL vary from subtle hypopigmented macules to florid nodular lesions, and HTLV-1 screening need to be carried out in all doubtful cases.

Neurolymphomatosis (NL) refers to nerve infiltration by neurotropic neoplastic cells in the setting of a known or an unknown hematological malignancy. It typically presents as painful or painless peripheral mononeuropathy, mononeuritis multiplex, polyneuropathy, polyradiculopathy, or cranial neuropathy. A 32-year-old male presented with a hyperpigmented hypoesthetic plaque over the anterolateral aspect of the right leg with thickening of the right common peroneal nerve and foot drop clinically diagnosed as Hansen's disease. Biopsy taken from skin showed infiltrates of pleomorphic small and medium sized lymphocytes in the dermis and subcutis. On immunohistochemistry, the cells were positive for CD3, CD4 and negative for CD8, CD20, and CD30. Ultrasonography-guided fine-needle aspiration of the thickened nerve showed infiltrates of atypical lymphoid cells. Based on these findings, a diagnosis of NL in primary cutaneous CD4+ pleomorphic small/medium-sized T-cell lymphoma was made. The disease responded to systemic chemotherapy and localized radiotherapy with no evidence of relapse during 3 years follow-up. NL in primary cutaneous CD4+ pleomorphic small/medium-sized T-cell lymphoma presenting with manifestations redolent of Hansen's disease is not described in available literature. This case also demonstrates the utility of fine needle aspiration of nerve, a minimally invasive procedure in the diagnosis of NL.

Background:Little data are available concerning clinical and pathological patterns of cutaneous lymphomas in India.Aim:To analyze the clinical and histopathological characteristics of cutaneous lymphomas in Indian patientsMaterials and Methods:This is a single-center, prospective, observational study carried out from January 1, 2010, to December 31, 2015. The patients underwent clinical examination, human T-cell lymphotropic virus-1 (HTLV-1) screening, skin biopsy with hematoxylin and eosin and immunohistochemistry staining.Results:Among 35 cases, 33 (94.3%) were T-cell, and 2 (5.7%) were B-cell lymphomas. The mean age was 52.66, and the male to female ratio was 2.5:1. The most common types of T-cell lymphomas included mycosis fungoides (MF) (57.1%) followed by adult T-cell lymphoma/leukemia (ATL) (17.1%). Primary cutaneous peripheral T-cell lymphoma not otherwise specified was diagnosed in 17.1% and anaplastic large cell lymphoma in 2.9%. The morphological types of MF included polymorphic, poikilodermatous, folliculotropic, hypopigmented, hyperpigmented, mixed, and purpuric. Skin manifestations of ATL included ulcerated plaques and erythroderma. Epidermotropism was very marked in ATL (83.3%) than in MF (70%). Larger Pautrier's microabscess was noted in ATL compared to smaller ones in MF. Markedly dense, diffuse infiltrate of atypical cells was noted in ATL in contrast to mild to moderate nodular or perivascular infiltrate in MF. ATL had an extremely poor prognosis.Limitations:Identification of DNA integration of HTLV-1 by Southern blot could not be analyzed, and the number of cases studied is limited.Conclusions:The study showed unique patterns of subtypes of cutaneous lymphomas in our country. Variations in the clinical pattern and histopathological analysis will help to differentiate T-cell lymphoma types which have prognostic implications.

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