Follicular Mycosis Fungoides, a Distinct Disease Entity With or Without Associated Follicular Mucinosis

Abstract

Objective: To determine the clinicopathologic features and the disease course of patients with follicular mycosis fungoides (MF).

Folliculotropic Mycosis Fungoides as a Posttransplant Lymphoproliferative Disorder

“…15 In fact, it has been suggested that patients with folliculotropic MF of any presentation should all be treated as though they have tumor-stage disease. 18 However, a recent case series found that folliculotropic MF as PTLD has a more indolent course, with all of the patients in the series alive at a median follow-up of 87 months. 5 Our patient has been followed for 37 months and despite near complete remission of his CTCL, his recent course has been complicated by episodes of acute graft rejection.…”

Cutaneous T‐cell lymphoma: 2011 update on diagnosis, risk‐stratification, and management

“…Continued role in risk stratification, TNMB (tumor, node, metastasis, and blood) staging remains an important prognostic factor in MF/SS and forms the basis for a ''risk-adapted'' approach to treatment. In 2007, the ISCL and EORTC revised the TNMB staging of MF/SS [143]. Patients with only patches and plaques have stage I disease, but may be further divided into stage IA (<10% body surface area involved or T1) or stage IB (>10% body surface area involved or T2) based on the extent of skin involvement.…”

“…In contrast to previous reports highlighting the aggressive clinical course associated with large cell transformation [189][190][191][192][193], defined as the presence of large, atypical lymphocytes comprising at least 25% of the total lymphoid infiltrate, large cell transformation was not an independent predictor of overall or disease-specific survival but was associated with a higher risk (hazard ratio 3.32) of disease progression [7]. Given the importance of the TNMB classification in risk stratification and defining disease burden, the ISCL/EORTC recommends its use in defining the initial, maximum and current burden of disease, which will ultimately play an important role in the selection of either skin-directed or systemic therapies [143].…”

“…In patients with erythroderma, criteria recommended for the diagnosis of SS by the ISCL include the following: absolute sezary count 1,000/ll, a CD4/CD8 ratio 10 (due to the clonal expansion of CD4 1 cells), aberrant expression of pan-T-cell antigens, demonstration of T-cell clonality in the presence of a lymphocytosis by Southern blot or PCR-based methods, or cytogenetic demonstration of an abnormal clone [143]. At a minimum, the WHO-EORTC recommends the demonstration of T-cell clonality in combination with the aforementioned criteria for the diagnosis of SS, as summarized in Table II [2].…”

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

“…Der Hautbefund bei der follikulären Mykosis fungoides unterscheidet sich wesentlich von dem der klassischen Mykosis fungoides. Charakteristisch ist bei 86 % eine Beteiligung der Gesichtshaut, nur bei etwa 14 % bleibt die Erkrankung auf Stamm und Extremitäten beschränkt [1,4]. Das morphologische Spektrum der Läsionen ist breit und umfasst erythemathöse Papeln und Plaques mit follikulärer Betonung, Komedonen, akneiforme und zystische Läsionen, aber auch noduläre bis hin zu prurigoartigen Formen [1,3].…”