The Spectrum of Histopathologic and Immunohistochemical Findings in Folliculotropic Mycosis Fungoides

Gerami, Pedram; Guitart, Joan

doi:10.1097/pas.0b013e3180439bdc

Cited by 127 publications

(153 citation statements)

References 26 publications

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“…In our group's previous studies, we have seen high densities of CD1a ϩ Langerhans cells in the follicular epithelium. 1 It is unclear whether folliculotropism is a function of specific cell-surface antigens of the T cells or an abnormality within the follicular epithelium.…”

Section: Discussionmentioning

confidence: 99%

“…We have previously published an in-depth analysis of the histologic patterns seen with this disease. 1,18 The present study is a comprehensive analysis of the clinical features of FMF and offers, to our knowledge, the first major US study of survival data in FMF.…”

Section: Discussionmentioning

confidence: 99%

“…Histologic criteria re- quired a predominance of 1 of the 5 patterns previously established as characteristic of FMF. 1 In each case, at least focal areas of the prototypical pattern with atypical folliculotropic lymphocytes was required. All cases were reviewed by our lymphoma group who have focused expertise in dermatology, dermatopathology, and oncology of cutaneous lymphomas.…”

Section: Methodsmentioning

confidence: 99%

“…1,19 In 22 of the 26 patients with true tumor lesions, the tumors involved the head and neck region. Of the 26 cases of true tumors, 22 involved the face or scalp.…”

Section: Clinical Characteristicsmentioning

confidence: 99%

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Folliculotropic Mycosis Fungoides

Gerami

Rosen²,

Kuzel³

et al. 2008

Arch Dermatol

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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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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Methodsmentioning

confidence: 99%

“…1,19 In 22 of the 26 patients with true tumor lesions, the tumors involved the head and neck region. Of the 26 cases of true tumors, 22 involved the face or scalp.…”

Section: Clinical Characteristicsmentioning

confidence: 99%

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Folliculotropic Mycosis Fungoides

Gerami

Rosen²,

Kuzel³

et al. 2008

Arch Dermatol

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196

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“…Zur Diagnosestellung sind neben der Klinik histologische und immunhistologische Untersuchungen entscheidend. Es zeigen sich insbesondere perifollikulär monomorphe T-Zell-Infiltrate [2,3,8]. Der Nachweis der Monoklonalität mittels PCR ist von großer diagnostischer Bedeutung.…”

Section: Kasuistik 123unclassified

Follikuläre Mykosis fungoides – Behandlung mit Methotrexat, Interferon-alpha, PUVA und Dermabrasio

Hofer¹,

Driesch²

2009

Akt Dermatol

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Folliculotropic Mycosis Fungoides

et al. 2010

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To clarify clinicopathologic features and reconcile discrepancies in previous studies of folliculotropic mycosis fungoides (FMF).Design: A single-center retrospective clinicopathologic study and a systematic review of FMF.Setting: Tertiary referral center in the midwestern United States.Patients: Patients with clinical and histopathologic evidence of FMF seen at the tertiary referral center during a 12 1 ⁄2-year period.Main Outcome Measures: Clinicopathologic features of FMF.Results: Fifty patients (32 male [64%] and 18 female [36%]) met study criteria for the clinicopathologic review. Pruritic patches, plaques, and folliculocentric lesions (milia, cysts, and alopecia) on the head, neck, and

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The Spectrum of Histopathologic and Immunohistochemical Findings in Folliculotropic Mycosis Fungoides

Cited by 127 publications

References 26 publications

Folliculotropic Mycosis Fungoides

Folliculotropic Mycosis Fungoides

Follikuläre Mykosis fungoides – Behandlung mit Methotrexat, Interferon-alpha, PUVA und Dermabrasio

Folliculotropic Mycosis Fungoides

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