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

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

“…4 The clinically noticeable impact on hair growth in STMF reflects the 75% incidence of concomitant follicular involvement observed over all literature-reported cases. Importantly, the clinical behavior of STMF with follicular epithelium involvement is significantly more benign than that of FMF, 6 suggesting that despite similar early clinical presentations and frequent histopathologic overlaps, STMF and FMF are molecularly distinct diseases.…”

“…3,6 Molecular studies demonstrate a high concentration of CD1a cells localized to the infundibulum and isthmus exceeding a 10:1 ratio in 70% of lesional hair follicles of FMF analyzed, 19 whereas this is not so for STMF even in instances of brisk follicular involvement indicating an intrinsic difference between the two entities. 4 This variation in T-cell subtypes may account for the significant divergence clinical behavior. Additionally, there have been no cases of transformation from one form of MF to another.…”

“…1,2 Syringotropic mycosis fungoides (STMF) is a rare form of MF, first identified by Sarkany in 1969. 3–4, 7–19 It is characterized histopathologically by hyperplastic eccrine glands and ducts surrounded and permeated by a dense infiltrate of atypical lymphocytes in the deep dermis. 4 Although the clinical presentation of STMF is similar to folliculotropic mycosis fungoides (FMF), STMF is much less aggressive.…”

“…3–4, 7–19 It is characterized histopathologically by hyperplastic eccrine glands and ducts surrounded and permeated by a dense infiltrate of atypical lymphocytes in the deep dermis. 4 Although the clinical presentation of STMF is similar to folliculotropic mycosis fungoides (FMF), STMF is much less aggressive. 5 In the largest case-series of STMF published to date, de Masson et al found five-year disease specific survival approached 100% for STMF (n = 19) versus 74% individuals with FMF (n = 54).…”

A Case of Rapidly Progressive, Fatal Mycosis Fungoides Presenting as a Haematoma-like Lesion

“…"Syringotropic MF", a rare variant of MF with a prognosis similar to classic MF, is characterized by hyperplastic eccrine glands and eccrine ducts surrounded by a dense infiltrate of atypical lymphocytes with cerebriform nuclei (1,(20)(21)(22). Folliculotropic MF is considered to be a closely related condition, as some cases of syringotropic MF demonstrate lymphocytic infiltration involving both eccrine glands and hair follicles (23,24).…”