Variants of Mycosis Fungoides

(1)

Department of Health Management, New York Medical College, Valhalla, New York, USA

(2)

University of British Columbia, Vancouver, British Columbia, Canada

(3)

Misdiagnosis Association, Seattle, Washington, USA

Keywords

Sézary syndromeLeukemicErythrodermaLymphadenopathyNeoplastic Sézary cellsFollicularPagetoid reticulosisGranulomatous slack skin

Clinical and histologic variants of mycosis fungoides (MF) have been reported. Sézary syndrome (SS) is considered to be the leukemic variant of MF and is defined as the triad of erythroderma , generalized lymphadenopathy , and the presence of neoplastic Sézary cells in the blood. In addition to SS, distinct variants of MF include follicular MF, pagetoid reticulosis, and granulomatous slack skin, which each individually have distinctive clinical and histological featured and are classified as separate entities.

Follicular Mycosis Fungoides

Other names of this condition include folliculotropic, follicular, pylotropic, folliculocentric, and follicular mucinosis [1]. The lesions of folliculotropic MF appear to preferentially affect the face, neck, and upper trunk regions [1]. A highly characteristic finding of follicular MF includes infiltrated plaques in the eyebrow region with alopecia. Histologically, follicular MF is characterized by dense dermal lymphocytic infiltrates localized to the perivascular and periadnexal areas. Folliculotropism is a dominant feature (as opposed to epidermotropism in the case of MF) [2]. The infiltrate contains small- and medium-sized lymphocytes that have irregular and cerebriform nuclei.

Overall survival is poor, with 15-year survival rates of 41 % even in early stage disease [3]. Patients with follicular MF patients are typically less responsive to the standard treatments used for patients with classical MF due to the deep, perifollicular or intrafollicular location of the neoplastic infiltrate [2].

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