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Follicular Mycosis Fungoides, a Distinct Disease Entity With or Without Associated Follicular Mucinosis
A Clinicopathologic and Follow-up Study of 51 Patients
Remco van Doorn, MD;
Erik Scheffer, MD;
Rein Willemze, MD;
for the Dutch Cutaneous Lymphoma Group
Arch Dermatol. 2002;138:191-198.
Objective To determine the clinicopathologic features and the disease course of
patients with follicular mycosis fungoides (MF).
Design A multicenter, 14-year, retrospective cohort analysis.
Setting Dutch Cutaneous Lymphoma Group.
Patients Fifty-one patients with the clinicopathologic features of follicular
MF with (n = 49) or without (n = 2) associated follicular mucinosis. Follow-up
data were compared with those of 158 patients with the classic epidermotropic
type of MF, including 122 patients with generalized plaque-stage MF (T2 N0
M0) and 36 patients with tumor-stage MF (T3 N0 M0).
Observations Characteristic clinical features not or rarely observed in classic MF
were the preferential localization of the skin lesions in the head and neck
region (45 of 51 patients), the presence of follicular papules, alopecia,
acneiform lesions, mucinorrhoea, and often severe pruritus. Characteristic
histologic findings were the presence of perifollicular neoplastic infiltrates
with a variable degree of folliculotropism, but generally no epidermotropism,
follicular mucinosis (49 of 51 cases), and often a considerable admixture
of eosinophils and plasma cells. Response on initial treatment, risk of disease
progression (development of extracutaneous disease and/or death from lymphoma),
and disease-specific and overall survival of patients with follicular MF were
worse than in classic MF patients. The actuarial disease-specific survival
was 68% at 5 years and 26% at 10 years.
Conclusions Follicular MF shows distinctive clinicopathologic features, is more
refractory to treatment, and has a worse prognosis than the classic type of
MF; it should be considered a distinct type of cutaneous T-cell lymphoma.
Based on these results and those of other studies, we suggest the term follicular MF for cases with or without associated follicular
mucinosis.
From the Departments of Dermatology (Dr van Doorn) and Pathology (Dr
Scheffer), Vrije Universiteit Medical Center, Amsterdam, and Department of
Dermatology (Dr Willemze), Leiden University Medical Center, the Netherlands.
A complete list of the participants in the Dutch Cutaneous Lymphoma Group
is available from the authors.
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