Syringolymphoid hyperplasia and follicular mucinosis in a patient with cutaneous T-cell lymphoma

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

“…11 Alopecia, pruritus, hyperesthesia, and anhidrosis of affected areas of skin have been reported, but lesions may also be entirely asymptomatic. 12 In patients with more extensive disease, the affected skin may show hyperkeratosis, poikiloderma atrophicans vasculare, erythroderma, or palmoplantar keratoderma. 13 Histologically, the classic features of syringotropic CTCL are hyperplastic eccrine glands and eccrine ducts surrounded by a dense, syringotropic infiltrate of atypical lymphocytes with characteristic cerebriform nuclei.…”

“…Whereas all of the previously reported cases of syringotropic CTCL requiring systemic therapy responded to treatment (including our patient), only a minority of patients with folliculotropic MF achieved remission after systemic therapy. 7,12,14,15,21 CONCLUSION Currently syringotropic CTCL is categorized by the WHO-EORTC as a subtype of folliculotropic MF 1 ; 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 a distinct entity separate from folliculotropic MF.…”

A Case of Syringolymphoid Hyperplasia with Follicular Mucinosis

“…Burg and Schmöckel [6], Tannous et al [4], Thein et al [1], and Pileri et al [2] speculated that SLH represents a syringotropic variant of cutaneous T-cell lymphoma. However, Haller et al [3] hypothesized that SLH is a syringotropic variant of FM and should be viewed as a facultative precursor lesion of mycosis fungoides.…”

“…SLH have been seen as both benign lesions and in association with T-cell lymphoproliferative lesions [1,2,3,4,5,6,7,8,9]. Long-term clinical and histopathological follow-up may be necessary to more accurately classify this rare lesion and assess its prognostic features.…”

“…First reported in 1969, SLH remains a very rare condition with less than 40 cases reported in the literature [1,2]. A subset of cases is associated with follicular mucinosis (FM); 12 cases of SLH with associated FM have been reported in the literature including this current case [1,2,3,4,5,6,7]. …”