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Case Reports

Abstract

A 63 year old male presented with three months of an intermittently pruritic 8x3 cm hyperpigmented, reticular, scaly plaque with underlying erythema on the left posterolateral neck.  Punch biopsy revealed patchy, band-like, dermal and perifollicular infiltrate of small-to-medium sized lymphocytes with hyperchromatic and cerebriform nuclei with epidermotropism and folliculotropism. In addition, there was exuberant perieccrine lymphocytic inflammation in the mid and deep dermis with extensive syringotropism. The infiltrate was primarily CD3+ T cells, with CD4:CD8 ratio of 3:1. CD5 expression was retained; CD7 expression was diminished in epidermotropic and adnexotropic lymphocytes. T-cell receptor gene rearrangement studies of the tissue revealed no clonal populations. Peripheral blood flow cytometry did not identify an abnormal CD4+ T cell population. A diagnosis of adnexotropic mycosis fungoides (MF) with marked syringotropism and folliculotropism was made.   MF is the commonest cutaneous T cell lymphoma. Unusually, this case demonstrated extensive combined features of syringotropic MF (STMF) and folliculotropic MF (FMF). FMF accounts for 10% of MF and presents as follicular papules, acneiform lesions, and alopecia, most commonly on the head and neck. STMF favors the palms, soles, and extremities. Whether STMF is on the spectrum of FMF or a separate entity is unclear. The largest SMF case series found 100% 5-year disease-specific survival (n=19) compared to 74% for FMF without syringotropism (n=54), suggesting the prognostic importance of this distinction.  Cases with combined features are rare in the literature, though perhaps underreported. Our patient was started on topical bexarotene and pending further follow-up.