Medizinische Universität Graz Austria/Österreich - Forschungsportal - Medical University of Graz
Gewählte Publikation:
Cerroni, L; Fink-Puches, R; Bäck, B; Kerl, H.
Follicular mucinosis: a critical reappraisal of clinicopathologic features and association with mycosis fungoides and Sézary syndrome.
Arch Dermatol. 2002; 138(2):182-189
Doi: 10.1001%2Farchderm.138.2.182
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- Führende Autor*innen der Med Uni Graz
- Cerroni Lorenzo
- Co-Autor*innen der Med Uni Graz
- Fink-Puches Regina
- Kerl Helmut
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- Abstract:
- 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 gamma 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.
- Find related publications in this database (using NLM MeSH Indexing)
- Adolescent -
- Adult -
- Female -
- Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor -
- Humans -
- Immunohistochemistry -
- Male -
- Middle Aged -
- Mucinosis, Follicular - complications
- Mycosis Fungoides - complications
- Polymerase Chain Reaction - complications
- Sezary Syndrome - complications
- Skin Neoplasms - complications