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Variants of mycosis fungoides

Emmilia Hodak, MD
Iris Amitay-Laish, MD
Section Editor
John A Zic, MD
Deputy Editor
Rosamaria Corona, MD, DSc


Mycosis fungoides (MF) is the most common cutaneous T cell lymphoma (CTCL) [1]. Patients with classic MF, as originally described by Alibert and Bazin two centuries ago, initially present with erythematous patches and plaques (picture 1A-B), which represent the early stage of MF. Clinically, the patches and plaques vary in size and shape, have a scaly atrophic surface, and are often located on sun-protected areas of the body. Histologically, they are characterized by a superficial lymphoid infiltrate and epidermotropism of lymphocytes with various degree of atypia. Although in many cases the diagnosis of MF is based upon the combination of clinical and histologic features, immunohistochemical and molecular studies are usually needed to confirm the diagnosis in equivocal cases [2,3].

It has long been recognized that early MF can mimic common dermatoses, such as psoriasis and atopic dermatitis. However, in the last few decades, the list of differential diagnoses has considerably widened, due to the description of numerous atypical variants of MF that show the clinical features and/or histopathologic patterns of all the major inflammatory skin diseases [4-7]. Among them, the 2005 World Health Organization-European Organization for Research and Treatment of Cancer (WHO-EORTC) classification of primary cutaneous T cell lymphomas recognizes only three variants/subtypes of MF with distinctive clinicopathologic features, clinical behavior, and prognosis: folliculotropic MF, pagetoid reticulosis, and granulomatous slack skin [1].

In this topic, we will review the clinical features, diagnosis, and management of these three well-defined subtypes of MF as well as of a number of rare MF variants. Classic MF and Sézary syndrome are discussed separately.

(See "Clinical manifestations, pathologic features, and diagnosis of mycosis fungoides".)

(See "Staging and prognosis of mycosis fungoides and Sézary syndrome".)

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Literature review current through: Dec 2017. | This topic last updated: Jun 08, 2017.
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