Follicular Mycosis Fungoides: Variability of a Rare Entity

Roeschm, Alexander; Schleyer, Verena; Landthaler, Míchael; Vogt, Thomas

doi:10.1111/j.1540-9740.2005.03389.x

Cited by 7 publications

(4 citation statements)

References 17 publications

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“…Thus, it is not surprising that the disease runs a more aggressive course in these more advanced stages. However, in some patients, FMF run a less aggressive and/or different course, challenging the concept of FMF as an entity in its own right with an intrinsically worse prognostic factor …”

Section: Discussionmentioning

confidence: 99%

Spiky follicular mycosis fungoides: a clinicopathologic study of 8 cases

et al. 2015

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Section: Discussionmentioning

confidence: 99%

Spiky follicular mycosis fungoides: a clinicopathologic study of 8 cases

et al. 2015

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“…3 There is scant information in the literature regarding its clinical manifestations. [4][5][6][7][8][9][10][11][12][13][14][15][16] Data regarding optimal therapies in FMF are similarly limited. In addition, only 2 other studies have provided any survival data for patients with FMF.…”

Section: Discussionmentioning

confidence: 99%

Folliculotropic Mycosis Fungoides

Gerami

Rosen²,

Kuzel³

et al. 2008

Arch Dermatol

196

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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.

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“…4). Clinically it presents follicular papules (often grouped), alopecia and acneiform lesions 48,49 . Patients often complain about severe pruritus 48 .…”

Section: Folliculotropic Mycosis Fungoidesmentioning

confidence: 99%

Mycosis fungoides: a dermatological masquerader

et al. 2007

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Mycosis fungoides (MF), a low-grade lymphoproliferative disorder, is the most common type of cutaneous T-cell lymphoma. Typically, neoplastic T cells localize to the skin and produce patches, plaques, tumours or erythroderma. Diagnosis of MF can be difficult due to highly variable presentations and the sometimes nonspecific nature of histological findings. Molecular biology has improved the diagnostic accuracy. Nevertheless, clinical experience is of substantial importance as MF can resemble a wide variety of skin diseases. We performed a literature review and found that MF can mimic >50 different clinical entities. We present a structured framework of clinical variations of classical, unusual and distinct forms of MF. Distinct subforms such as ichthyotic MF, adnexotropic (including syringotropic and folliculotropic) MF, MF with follicular mucinosis, granulomatous MF with granulomatous slack skin and papuloerythroderma of Ofuji are delineated in more detail.

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Follicular Mycosis Fungoides: Variability of a Rare Entity

Abstract: Because it was previously suggested that FMF may have a worse prognosis compared with classic mycosis fungoides, this impressive response to bexarotene is of great clinical interest and may spark new studies employing this novel retinoid for FMF treatment.

Cited by 7 publications

References 17 publications

Spiky follicular mycosis fungoides: a clinicopathologic study of 8 cases

Spiky follicular mycosis fungoides: a clinicopathologic study of 8 cases

Folliculotropic Mycosis Fungoides

Mycosis fungoides: a dermatological masquerader

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