Review Article iMedPub Journals Journal of Neoplasm 2018 www.imedpub.com ISSN 2576-3903 Vol.3 No.2:33 DOI: 10.21767/2576-3903.100033 Patch, Plaque, Tumour - Mycosis Fungoides Anubha Bajaj* Department of Histopathology, A.B. Diagnostics, India Abstract Corresponding author: Anubha Bajaj Mycosis fungoides is a frequent cutaneous lymphoma contributing to an estimated half (50%) of the emerging dermal lymphomas. As an epidermotropic primary [email protected] cutaneous T lymphoma (CTCL), it may comprise of miniature or medium sized lymphocytes containing cerebriform nuclei and a T helper cell immune phenotype, Consultant, Department of Histopathology, although variants of a cytotoxic T lymphocyte (CTL) component may commonly A.B. Diagnostics, India. arise. “Mycosis Fungoides “as a terminology is restricted to classical manifestation of a disorder characterized by the appearance of patches, plaques and tumours or Tel: +919811693956 the diverse cutaneous disorders with an identical clinical evolution. The prevalence of mycosis fungoides is at an estimated 60% of cutaneous tumours, though the incidence may be augmented in the Africans. The lesions may depict a prominent Citation: Bajaj A (2018) Patch, Plaque, regional variability. Elderly and adult individuals may be frequently incriminated Tumour - Mycosis Fungoides. J Neoplasm. with a male predominance (M:F: 2:1), although children and adolescents may Vol.3 No.2:33 similarly manifest the cutaneous lymphoma Keywords: Mycosis fungoides; Lymphoma; Chronic dermatosis; Cutaneous Received: October 10, 2018; Accepted: November 02, 2018; Published: November 05, 2018 Introduction lesions predominantly arising in the sun protected zones, especially buttocks and the female breasts. A variable scaling Mycosis fungoides is a condition of obscure aetiology. Chronic may ensue; contingent to the preceding therapeutic remedies dermatosis may accompany the disorder. An extensive exposure undertaken [1]. ii) The plaques of mycosis fungoides may signify to diverse allergens or a genetic predilection may be elucidated an indiscriminately infiltrative, variously scaled, erythematous in specific instances. Viral infections have not been implicated or reddish-brown lesion. The classical patches may appear to in the genesis of mycosis fungoides. The condition may appear be contiguous with the plaques or may arise at adjunctive body subsequent to a solid organ transplant, thereby implying an sites [6]. The plaques of mycosis fungoides may necessitate a immune suppressive aetiology in disease evolution [1]. demarcation from the flattened tumefaction. Dusky or dark- Disease characteristics skinned individuals may generate greyish or silver hued patches and plaques with a diminished erythema [1] (iii) Tumefaction Mycosis fungoides as a disorder may be categorized into three contingent to mycosis fungoides may be solitary and localized or distinct stages: I) patch stage, ii) plaque stage and iii) tumour disseminated and concomitant to the classic patches or plaques stage. The prolonged clinical course may be undulating and or appear as singular tumour in the absence of adjunctive extends over a period of months or decades. The majority (>90%) lesions. Tumour ulceration may be frequent. Tumour progression of the initial lesions of mycosis fungoides may not depict an extra with mycosis fungoides may be divaricating, the tumours may cutaneous manifestation of the disease [1-3]. Mycosis fungoides quickly evolve within a few weeks or may be comparatively may exhibit plaques, patches or tumours appearing singularly indolent. Partially retrogressed tumours may be frequent [1]. or as commingled lesions [1,2]. Although patches classically The mucosa may be infrequently implicated in patients with delineate the preliminary form of mycosis fungoides, they may preliminary mycosis fungoides, though commonly involved be frequently admixed with adjunctive plaques and tumefaction. with the progressive phase. Enhancing erythroderma may be The progressive stage or the remitted, extensive lesions may delineated in mycosis fungoides, which mandates a distinction reoccur with subsequent reappearance of patches, plaques or from the classic Sezary syndrome [7]. The two conditions may be tumours. i) The typical patches of mycosis fungoides may be generically associated in cutaneous T cell lymphomas (CTCL) [1] generalized or localized, variegated, enlarged erythematous (Table 1). © Under License of Creative Commons Attribution 3.0 License | This article is available in: http://neoplasm.imedpub.com/archive.php 1 ARCHIVOSJournal of DE Neoplasm MEDICINA 2018 ISSNISSN 2254-6758 1698-9465 Vol.3 No.2:33 Histological elucidation intra-epidermal aggregates (Pautrier’s micro-abscesses). Micro abscesses may be the designated morphological indication Classically, mycosis fungoides may be categorized into of disease, though may be absent within preliminary lesions, three distinct stages: premycotic, mycotic and tumorous. tumefaction stage along with diagnostic biopsies acquired in The premycotic stage may delineate the scaling skin to be the therapeutic phase [2]. The assiduous categorization of the erythematous and pruritic. Thematic diversity in the clinical histological criterion of preliminary mycosis fungoides may be formulation of the disorder may be characterized by solitary, achieved with an appropriate clinical and pathologic concordance follicular granulomatous, pustular, bulbous, hyperkeratotic, [1] (Figures 1-6). Numerous histological configurations of verrucous and hypopigmented variants [2]. Lesions such as large inflammatory dermatosis may simulate the preliminary lesions, plaque parapsoriasis may transform into mycosis fungoides in a thus an accurate categorization on histopathology may be significant number of instances. The non-confirmatory microscopic challenging. The early lesions of mycosis fungoides may depict morphology of the premycotic stage may depict a picture akin a focal, lichenoid or a band like inflammatory infiltrate arising to chronic nonspecific dermatitis accompanied by psoriasiform in the fibrotic papillary dermis with a prominent component alteration of the epidermis. The infiltrative plaques may arise of mature, miniature lymphocytes [7,8]. The lymphocyte within the mycotic stage with the histological delineation of a aggregates may singularly articulate the phenomenon of dermal polymorphous inflammatory infiltrate which may exhibit “epidermotropism”. However, ‘Pautrier’ micro-abscesses a miniature quantity of definitive, a typical lymphoid cell. The may be infrequent. Constituent ‘halo lymphocytes’ (cells with cells may enunciate a dermal invasion (Partier’s micro-abscesses) enlarged nuclei circumscribed by a minimal halo) may be aligned or may congregate within the epidermal basal layer (with the along the epidermal basal layer (basilar epidermotropism), absence of spongiosis, the feature may suggest the emergence of mycosis fungoides). The lymphoid infiltrate may surround the hair follicles with a concomitant follicular mucinosis [2]. The tumorous stage may be defined by intense infiltrates of aberrant lymphoid cells which may distend the dermis. The categorical, definitive cell of mycosis fungoides may be a miniature or a medium sized lymphocyte with a cerebroid nucleus. The nuclear character may be typified by a markedly irregular nuclear outline with a thick nuclear membrane, a feature elucidated with thin paraffin embedded sections, the cerebroid cells may demonstrate a phenotype of T helper lymphocyte CD4+ though occasional CD8+ cytotoxic/suppressor or anomalous phenotypes of T lymphocytes may be exhibited [2]. Malignant lymphocytes within the advanced tumour stage may manifest the appearance of CD15. The characteristic histopathology enunciates an epidermotropic proliferation of variable, pleomorphic T Figure 1 T lymphocytic infiltrate in the upper dermis. lymphocytes (cerebriform cells) which may configure typical Table 1 Classification of cutaneous T cell lymphomas [5]. Cutaneous T cell and NK cell lymphomas Mycosis Fungoides (MF) MF variants and subtypes Folliculotropic MF Pagetoid reticulosis Granulomatous slack skin Sezary Syndrome Adult T cell Leukaemia Lymphoma (ATLL) Primary cutaneous CD30+ lympho-proliferative disorder Primary cutaneous anaplastic large cell lymphoma Lymphomatoid Papulosis Subcutaneous panniculitis like T cell lymphoma Extra-nodal natural killer/T cell lymphoma nasal type Hydroa vacciniforme like lympho-proliferative lesion Primary cutaneous CD8+ aggressive epidermotropic cytotoxic T cell lymphoma (provisional) Primary cutaneous ṿ/ᵟ T cell lymphoma Primary cutaneous CD4+ small/medium sized T cell lympho-proliferative disorder (provisional) Primary cutaneous acral CD8+ T cell lymphoma (provisional) Primary cutaneous peripheral T cell lymphoma This article is available in: http://neoplasm.imedpub.com/archive.php 2 ARCHIVOSJournal of DE Neoplasm MEDICINA 2018 ISSNISSN 2254-6758 1698-9465 Vol.3 No.2:33 Numerous intra-epidermal lymphocytes may be elucidated with concomitant, mild epidermal spongiosis (disproportionate epidermotropism). The aforementioned features may provide crucial evidence in the diagnosis of the preliminary disorder [8]. Atypical histological manifestations of preliminary mycosis fungoides may be 1) the occurrence of significant spongiosis 2) modifications of dermo-epidermal junction with the emergence of numerous necrotic keratinocytes, thus simulating the histology of erythema multiforme 3) prominent incontinence of the melanin pigment with concurrent