Case ReportPrimary Cutaneous T-cell Lymphoma: Two Rare Presentations
Introduction
Cutaneous T-Cell Lymphoma (CTCL) is a subset of non-Hodgkin's T-cell lymphomas which presents with malignant lymphocytic infiltration of the skin. The disease spectrum is characterized by clonal proliferation of helper T-cells of the CD4 phenotype in the skin. Mycosis Fungoides (MF), the commonest variant of CTCL, is characterized clinically by an indolent clinical course with subsequent evolution of patches, plaques andtumours and histologically by the infiltration of the epidermis by medium-sized to large atypical T-cells with cerebriform nuclei [1]. Although not very rare worldwide, it is commonest among Africans and relatively uncommon among Asians. Hypopigmented MF is a rare variant of patch stage MF in the dark skinned. Folliculotropic MF is an uncommon variant characterized by folliculotropic T-cell infiltrates with or without mucinous degeneration of the hair follicles.
Section snippets
Case 1
A 43 year old male presented with insidious, dark, raised, painless lesions over his face for four years. There was no history of fever, weight loss, epistaxis, red eyes, testicular pain, swelling of hands/feet, muscle weakness, photosensitivity, oral ulcers, joint pains or systemic symptoms.
General physical and systemic examination was normal. Dermatological examination revealed two discrete, well defined, dusky-erythematous, boggy, alopecic plaques studded with follicular papules over the
Discussion
Primary CTCLs have been classified by the European Organization for Research on the Treatment of Cancer (EORTC) and World Health Organisation (WHO) based on cell type (T cell, NK cell, B cell or precursor cell) and clinical outcome (indolent or aggressive) [3].
Folliculotropic MF has distinctive clinical and histologic features, is more refractory to standard treatment and has a worse prognosisthan classical MF. First described in 1957 as ‘Alopecia Mucinosa’, and later as ‘Follicular Mucinosis’,
Conflicts of Interest
None identified
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