Syringotropic Mycosis Fungoides: A Variant of Folliculotropic or a Distinct Subtype of Adnexotropic Mycosis Fungoides?

December 2022 | Volume 21 | Issue 12 | 1362 | Copyright © December 2022


Published online December 1, 2022

Dillon Nussbaum BSa, Misty G. Eleryan MD MSb, Christen B. Samaan MDc, Frank Glass MDd, Ronald Prussick MDa,e

aDepartment of Dermatology, George Washington University School of Medicine & Health Sciences, Washington, D.C.
bDivision of Dermatology, UCLA David Geffen School of Medicine, Los Angeles, CA
cDepartment of Dermatology Penn State School of Medicine, Hershey, PA
dDepartment of Dermatology, University of South Florida, Tampa, FL
eWashington Dermatology Center, Rockville, MD

Abstract
Mycosis fungoides (MF) is a slowly progressive form of cutaneous T-cell lymphomas (CTCL). MF is classified into 4 subtypes including folliculotropic MF (FMF). Infiltration of both hair follicles and eccrine glands is included in the FMF classification and designated as syringotropic MF (STMF), an exceedingly rare form of CTCL. We report an additional case of STMF. The clinical course of syringotropic CTCL is more benign than FMF, suggesting that despite similar clinical presentations, they are molecularly distinct diseases. Clinical characteristics can help differentiate STMF from FMF. Skin-directed therapies are less effective in STMF than FMF. With distinct clinical characteristics, histopathologic findings, and disease course, syringotropic CTCL should be considered a subtype entity in the spectrum of adnexotropic MF.

J Drugs Dermatol. 2022;21(12):1362-1364. doi:10.36849/JDD.6779

INTRODUCTION

Mycosis fungoides (MF) is a slowly progressive form of cutaneous T-cell lymphomas (CTCL), a group of malignancies of skin-homing lymphocytes. MF is the most common form of CTCL with 1260 newly diagnosed cases per year and typically affects adults in their 6th decade of life.1 The World Health Organization (WHO)-European Organization for Research and Treatment of Cancer (EORTC) classifies MF into 4 subtypes, which are classic MF, Woringer-Kolopp disease, granulomatous slack-skin disease, and folliculotropic MF (FMF).

FMF is characterized by atypical lymphocytes infiltrating the hair follicles with preferential clinical presentation involving the head and neck with associated alopecia and mucinorrhea.1 Infiltration of both hair follicles and eccrine glands is included in the FMF classification by the WHO-EORTC and designated as syringotropic MF (STMF).2 STMF is an exceedingly rare form of CTCL with less than 40 cases reported in the literature. We report an additional case of STMF with a distinguishable presentation of livedoid lesions and ulceration.

CASE REPORT



A 68-year-old Caucasian male with history of psoriasis and herpes simplex virus infection presented to his local dermatologist with a 1-year history of worsening, painful plaques on the left ankle despite prolonged use of high-potency topical steroids. He reported a stabbing and burning pain in the medial arch of his left foot. As time progressed, the left ankle lesion became ulcerated and he developed several satellite papules on the left plantar foot. He was referred to wound care clinic for treatment of a persistent non-healing ulcer.
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