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Follicular Mucinosis
A Critical Reappraisal of Clinicopathologic Features and Association With Mycosis Fungoides and Sézary Syndrome
Lorenzo Cerroni, MD;
Regina Fink-Puches, MD;
Barbara Bäck;
Helmut Kerl, MD
Arch Dermatol. 2002;138:182-189.
Context Beginning in 1957, patients have been described with localized alopecia
characterized histopathologically by mucin deposition within hair follicles
(follicular mucinosis [FM]). At least 2 distinct diagnostic entities have
been proposed: one occurring in children and young adults without association
with other diseases ("idiopathic" FM), the other occurring in elderly patients
and associated with mycosis fungoides or Sézary syndrome ("lymphoma-associated"
FM).
Objective To determine whether idiopathic and lymphoma-associated FM are distinct
or related entities.
Design Case series.
Setting Department of Dermatology, University of Graz, Graz, Austria.
Patients Forty-four patients with FM were divided into 2 groups. Group 1 comprised
16 patients (mean age, 37.5 years) with no associated mycosis fungoides or
Sézary syndrome; group 2 was made up of the other 28 (mean age, 52.2
years), who had clinicopathologic evidence of cutaneous T-cell lymphoma.
Results Mean age was lower in patients with idiopathic FM, but a considerable
overlapping among the 2 groups was present. Location on the head and neck
region was common in both groups, but most patients with lymphoma-associated
FM had lesions also on other body sites. In fact, solitary lesions at presentation
were common in patients with idiopathic FM (11 [68.8%] of 16 patients), but
uncommon in those with lymphoma-associated FM (2 [7.1%] of 28 patients). Histopathologic
findings did not allow clear-cut differentiation of the 2 groups. Finally,
a monoclonal rearrangement of the T-cell receptor  gene was demonstrated
by polymerase chain reaction analysis in about 50% of tested cases from each
group.
Conclusions Criteria previously reported to differentiate idiopathic from lymphoma-associated
FM proved ineffective. In analogy to localized pagetoid reticulosis (Woringer-Kolopp
disease), small-plaque parapsoriasis, and so-called solitary mycosis fungoides,
idiopathic FM may represent a form of localized cutaneous T-cell lymphoma.
From the Department of Dermatology, University of Graz, Austria.
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