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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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