Folliculotropic Mycosis Fungoides

Abstract

To study the clinical features, therapeutic responses, and outcomes in patients with folliculotropic mycosis fungoides (FMF) and to compare our single-center experience of 43 patients with the findings from the Dutch Cutaneous Lymphoma Group.Setting: A single-center experience from the Northwestern University Multidisciplinary Cutaneous Lymphoma Group.Patients: Forty-three patients with FMF were included in the study and compared with 43 age-and stagematched patients with classic epidermotropic mycosis fungoides (MF) with similar follow-up time.Results: Folliculotropic mycosis fungoides showed distinct clinical features, with 37 patients having facial involvement (86%) and only 6 having lesions limited to the torso (14%). The morphologic spectrum of lesions is broad and includes erythematous papules and plaques with follicular prominence with or without alopecia; comedonal, acneiform, and cystic lesions; alopecic patches with or without scarring; and nodular and prurigolike lesions. Sixty-five percent of patients had alopecia, which in 71% of cases involved the face. Severe pruritus was seen in 68% of patients. In general, patients responded poorly to skin-directed therapy and in almost all cases required systemic agents to induce even a partial remission, including patients with early-stage disease. Overall survival was poor. Patients with early-stage disease (ՅIIA) had a 10-year survival of 82%, which took a steep drop off to 41% by 15 years. Patients with late-stage disease (ՆIIB) had an outcome similar to those patients in the control group with conventional epidermotropic MF of a similar stage. Conclusions:The morphologic spectrum of clinical presentation for FMF is broad and distinct from those in conventional MF. This is at least partially attributed to the ability of FMF to simulate a variety of inflammatory conditions afflicting the follicular unit. The disease course is aggressive, and many patients, including those with early disease, show a poor outcome particularly between 10 and 15 years after the initial onset of disease. Response to skindirected therapy is poor even in early-stage disease, and our best results were seen with psoralen plus UV-A (PUVA) therapy with oral bexarotene or PUVA with interferon alfa. These findings corroborate those of the Dutch Cutaneous Lymphoma Group and further validate the classification of FMF as a distinct entity.

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

“…Resistance to skin directed therapies mirrors FMF which, in the rare instance it responds to treatment, requires systemic agents. 20 However, even stubborn STMF lesions do not incur disease specific mortality 6 compared with the FMF, which even when treated aggressively, has an 18% and 59% mortality rate at 10 and 15 years, respectively. 20 …”

Alopecia Universalis Associated with Cutaneous T Cell Lymphoma

“…Patchy alopecia can be a feature of folliculotropic MF, an uncommon variant of MF, characterized histologically by atypical T lymphocytes which infiltrate preferentially the follicular epithelium and may or not be associated with follicular mucinosis [1]. These patients often have alopecic patches, which from a clinical point of view may present as (1) red and scaly inflammatory patches of scarring alopecia, (2) non-inflamed smooth patches resembling alopecia areata or (3) scalp nodular lesions with alopecia [1,2,3,4]. The alopecia areata-like pattern has been described on the scalp, but it is more commonly observed in other body areas, including the limbs and pubic area [5].…”

Two Clinically Unusual Cases of Folliculotropic Mycosis Fungoides: One with and the Other without Syringotropism

“…Therefore, in cases with slight follicular involvement, findings of eccrine gland involvement could be helpful in the diagnosis3. According to the few studies reported on patients with folliculotropic MF, the percentage of accompanying eccrine gland involvement is 4% to 33% (Table 1)3,4,5,6,7,8,9,10. It is unclear whether folliculotropism or syringotropism is a function of specific cell-surface antigens of the T-cells, adhesion molecules, or an abnormality of follicular or syringeal epithelium2.…”

“…Purple, pruritic papules, and brownish purple patches were observed on the intertriginous regions. Gerami et al8 have reported 2 cases having histologically interface dermatitis with lichen planopilaris-like pattern.…”