Pseudotumorous Folliculotropic Mycosis Fungoides

Kossard, Steven; Weller, Paul

doi:10.1097/01.dad.0000159213.32066.22

Cited by 20 publications

(14 citation statements)

References 17 publications

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“…Entsprechende Läsionen wurden bereits in der Literatur beschrieben und histologisch einer follikulären Hyperplasie sowie granulomatösen Veränderungen zugeschrieben [ 18,21 ] . Während diese Läsionen bei der histologischen Untersuchung follikulotrope, neoplastische Infi ltrationen aufwiesen, fehlte ihnen die tieferlegende Infi ltration wie sie für die FMF im Tumorstadium typisch ist.…”

Section: Geschlecht/alter Stadium * Verwendete Behandlungen Effektivsunclassified

Underrecognized clinical features of folliculotropic mycosis fungoides: a large clinical series

Baykal

Atcı

Sarı

et al. 2017

J Deutsche Derma Gesell

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Hintergrund und Ziel: Als seltene Form der Mycosis fungoides (MF), ist die follikulotrope MF (FMF) durch ein breites Spektrum klinischer Symptome gekennzeichnet. Dazu gehören, neben den vorherrschenden follikulären Läsionen, auch viele atypische Manifestationen. Das Ziel der vorliegenden Studie war eine klinische Bewertung von FMF-Patienten, unter besonderer Berücksichtigung von vernachlässigten dermatologischen Merkmalen. Patienten und Methodik: Insgesamt wurden 27 FMF-Patienten aus dem 572 Patienten umfassenden MF-Register unserer Abteilung retrospektiv bezüglich ihrer Demographie sowie der klinischen Merkmale, Behandlungsformen, Nachsorge und Therapieergebnisse bewertet. Ergebnisse: Neben den bekannten klinischen Symptomen der FMF fanden wir Lichen-spinulosus-artige Läsionen mit begleitender Hypopigmentierung (n = 3) und Alopezie (n = 2), infiltrierte/erhabene, erythematöse Plaques im Gesicht, die zunächst als Lupus tumidus angesehen wurden (n = 2), pseudotumorale Läsionen, die klinisch eine MF im Tumorstadium vortäuschten (n = 1), dauerhafte Exkoriationen (n = 1), erythematöse, Rosazea-artige Papeln im Gesicht (n = 1) sowie kuppelförmige, asymptomatische, mit Muzin gefüllte (in der Histologie) Papeln/Knoten (n = 2), die andere krankheitsbedingte Läsionen überlagerten. Es kamen mehrere Therapieansätze mit unterschiedlichem Ergebnis zur Anwendung. Acht (29,6 %) Patienten hatten FMF im Spätstadium. Schlussfolgerungen: Das Bewusstsein für vernachlässigte klinische Symptome kann wesentlich dazu beitragen, verspätete Diagnosen dieser aggressiven MF-Variante zu verringern. SummaryBackground and Objective: A rare variant of mycosis fungoides (MF), folliculotropic MF (FMF) is characterized by a broad clinical spectrum that primarily includes follicle-based lesions but also many atypical clinical manifestations. The objective of the present study was to conduct a clinical analysis of patients with FMF, with a particular focus on highlighting underrecognized dermatological features.

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Section: Geschlecht/alter Stadium * Verwendete Behandlungen Effektivsunclassified

Underrecognized clinical features of folliculotropic mycosis fungoides: a large clinical series

Baykal

Atcı

Sarı

et al. 2017

J Deutsche Derma Gesell

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“…The authors explained that although clinical examination of their patient was compatible with advanced tumor stage of MF, the histopathological examination was not compatible with the advanced stage; the bulk of his nodules were due to follicular hyperplasia. [4] Another study about this particular association was reported by Scarisbrick et al In this study 3 patients with CD30+large cell anaplastic lymphoma and 3 patients with lymphomatoid papulosis with histologic evidence of PEH were described. Initial clinicopathologic diagnosis was squamous cell carcinoma in 4 of these patients and eruptive keratoacanthoma in 1 patient.…”

Section: Discussionmentioning

confidence: 62%

“…[5] PEH, has occasionally been reported in the literature as a manifestation of cutaneous lymphomas. It has been associated especially with CD30+ anaplastic large cell lymphoma in the majority of cases [3,4,6] and rarely with lymphomatoid papulosis type A [6] and MF. [3,4] The first comprehensive study describing the clinical and histopathological features of PEH in cutaneous T-cell lymphomas was reported by Courville et al in 1999, although this particular association had rarely been reported previously.…”

Section: Discussionmentioning

confidence: 99%

“…It has been associated especially with CD30+ anaplastic large cell lymphoma in the majority of cases [3,4,6] and rarely with lymphomatoid papulosis type A [6] and MF. [3,4] The first comprehensive study describing the clinical and histopathological features of PEH in cutaneous T-cell lymphomas was reported by Courville et al in 1999, although this particular association had rarely been reported previously. [7,8] The authors studied 11 cases with PEH which were collected from a series of 353 cutaneous lymphomas registered between 1990 and 1996 by the French Study Group of Cutaneous Lymphomas in their study.…”

Section: Discussionmentioning

confidence: 99%

“…[2] There are also some unusual histological types of MF such as MF with pseudoepitheliomatous hyperplasia (PEH) [3] and pseudotumorous folliculotropic MF. [4] These patients present with exophytic scaling plaques, nodules [4] or seborrhoeic keratosis-like lesions. [3] These unusual clinical and histopathological types can cause problems either during the diagnosis or in staging of patients with MF.…”

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confidence: 99%

See 2 more Smart Citations

Pseudoepitheliomatous Hyperplasia In Mycosis Fungoides

Bayramgürler¹,

Yıldız²,

Demirsoy³

et al. 2008

TUTFD

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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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Pseudotumorous Folliculotropic Mycosis Fungoides

Cited by 20 publications

References 17 publications

Underrecognized clinical features of folliculotropic mycosis fungoides: a large clinical series

Underrecognized clinical features of folliculotropic mycosis fungoides: a large clinical series

Pseudoepitheliomatous Hyperplasia In Mycosis Fungoides

Folliculotropic Mycosis Fungoides

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