Syringotropic Mycosis Fungoides: A Rare Form of Cutaneous T-cell Lymphoma Enabling a Histopathologic “Sigh of Relief”

Abstract

Syringotropic mycosis fungoides (STMF) is a very rare variant of cutaneous T-cell lymphoma (CTCL). It follows a much milder disease course than its clinically indistinguishable adnexal counterpart, folliculotropic mycosis fungoides (FMF). We report a case of a 36-year-old male who presented with erythematous, studded papules and plaques on the left upper extremity and right anterior thigh diagnosed as MF Stage 1A on initial superficial shave biopsy. Lesions recurred after initial improvement with narrow band ultraviolet light therapy demonstrating a concentration of abnormal lymphocytes around eccrine sweat glands on repeat biopsy, consistent with STMF. While the deeper, periadnexal infiltrate found in both STMF and FMF confers increased resistance to skin-directed therapies effective in classic MF, these entities diverge with respect to their clinical behavior. Syringotropism is a marker for increased disease specific survival whereas even FMF carries a prognosis worse than conventional MF. Increased awareness amongst the dermatopathology community of the histopathologic distinction between STMF and FMF is essential to guide treatment type, duration, and intensity in adnexal disease.

Rare Case of Syringotropic Mycosis Fungoides Presenting as Poikiloderma-Like Erythroderma Concurrent With Intermittent Fever and a Literature Review

“…As previously reported, male patients are affected twice as often as female patients, 5 which is compatible with classic MF. The age of the affected patients ranges from 19 to 86 years old, with older age group predominant.…”

“…In addition to these features, syringotropism was concomitant with folliculotropism in most cases of STMF. 5,6,13,14 However, our 2 specimens only demonstrated syringotropism without the presence of follicles. Echols et al 13 proposed that it was related to the anatomical sites; except for areas with a high density of follicles on the face and neck, follicles were not easily found in the specimens from other parts.…”

Syringotropic Lichen Planus: A Potential Histopathologic Mimicker of Syringotropic Mycosis Fungoides

“…Clinically, syringotropic MF is usually characterized by infiltrated papules, plaques, or nodules without prominent scaling, sometimes showing a clear-cut follicular accentuation; the lesions are solitary in a distinct proportion of the cases. 3,6,7 Our patient did not show clinical features of conventional or syringotropic MF. In addition, typical histopathologic features of LP were observed in the epidermis and papillary dermis, confirming the diagnosis of LP.…”

“…2,3 Eccrinotropic infiltrates of neutrophils are usually observed in the setting of neutrophilic eccrine hidradenitis, 4 which is regarded as an adverse cutaneous drug reaction (particular chemotherapeutic agents), or of palmoplantar eccrine hidradenitis, 5 an idiopathic condition with a predilection for children. Histologic evidence of lymphocytic syringotropism has been conventionally linked to mycosis fungoides (MFsyringotropic MF), 3,6,7 although rare cases of syringotropism in lichen striatus (LS) 2 or lymphocytic autoimmune hidradenitis 8 have been described as well.…”

Successful treatment ofCD8+ syringotropic mycosis fungoides with bexarotene and local radiotherapy

“…Dear Editor, Syringotropic mycosis fungoides (STMF) is a rare variant of cutaneous T cell lymphoma (CTCL) that is characterized by infiltration of atypical lymphocytes into or around the eccrine sweat ducts, [1][2][3][4] leading to hypohidrosis of affected skin. 4 STMF occurs in a wide range of age groups, but the patients are relatively younger than those with conventional MF.…”