Case report
Two cases of syringotropic cutaneous T-cell lymphoma and review of the literature

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Syringotropic cutaneous T-cell lymphoma (CTCL) is a rare form of CTCL characterized histologically by infiltrates of atypical lymphocytes located primarily in and around hyperplastic eccrine glands and ducts. Currently, syringotropic CTCL is classified as a histopathologic variant of folliculotropic mycosis fungoides (MF); however, the relationship between these two entities remains tenuous. We report two additional cases of syringotropic CTCL and review the differences between these two subtypes of MF with regard to epidemiology, clinical features, prognosis, and treatment. Based on these data, we conclude that syringotropic CTCL should be classified as a distinct variant of MF separate from folliculotropic MF.

Section snippets

Case 1

A 65-year-old Caucasian woman was referred to our dermatology clinic with a 6-month history of hair loss and erythematous, pruritic lesions on her scalp, neck, abdomen, groin, and back, which had been previously diagnosed as granulomatous dermatitis. She had received treatment with topical corticosteroids with little improvement. The patient's medical history was significant for mitral valve prolapse, depression, and stasis dermatitis as a result of chronic lower extremity edema.

On physical

Discussion

Although SLHA and syringotropic CTCL were previously considered separate conditions, the current literature suggests that they are varying presentations of the same disease. A correlation between syringotropic CTCL and SLHA was originally proposed by Burg and Schmockel4 in 1992. SLHA shares many of the same histopathologic features of syringotropic CTCL, such as hyperplastic eccrine glands surrounded by a lymphocytic infiltrate, but without the presence of heavy infiltrate of atypical

Conclusion

Currently syringotropic CTCL is categorized by the WHO-EORTC as a subtype of folliculotropic MF1; however, emerging data suggest that this classification system may need to be revisited. Although there is some histologic overlap between these two variants with some cases of folliculotropic CTCL having involvement of the eccrine glands and vice versa, the cumulative data regarding differences in epidemiology, pathogenesis, and prognosis suggest that syringotropic CTCL may merit classification as

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Cited by (22)

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    2017, Actas Dermo-Sifiliograficas
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    The second was the generalized syringotropic MF responsive to extracorporeal photophoresis and maintenance use of oral bexarotene.4 In other cases of syringotropic MF, patients had variable response to PUVA treatment,10 narrow-band ultraviolet-B phototherapy, and topical treatments with bexarotene gel and clobetasol ointment.11 In our case, TSEBT is a good treatment modality for generalized syringotropic MF without the evidence of internal organ involvement.

  • Syringotropic mycosis fungoides: Clinical and histologic features, response to treatment, and outcome in 19 patients

    2014, Journal of the American Academy of Dermatology
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    Palmar and plantar involvement by STMF was not reported in the previous studies on STMF by Pileri et al,5 but has been described in case reports.10,17,18 This, along with the deep location of the infiltrate, may explain the low rates of complete responses associated with topical treatments in STMF observed in the current study and others.7,9,10,19 Ulceration, which is not a typical presentation of MF, occurred in 4 cases, and had been previously described in 4 additional case reports of STMF,20-23 which suggests that it could be more frequent in STMF than in classic MF.

  • Syringotropic cutaneous T-cell lymphoma mimicking dermatomycosis

    2012, Annales de Dermatologie et de Venereologie
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Funding sources: None.

Conflicts of interest: None declared.

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