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Primary follicular mucinosis: long-term follow-up of patients younger than 40 years with and without clonal T-cell receptor gene rearrangement.
J Am Acad Dermatol. 2002 Dec; 47(6):856-62.JA

Abstract

Since the original descriptions of follicular mucinosis, accumulating experience shows that patient age, distribution of lesions, and duration or extent of disease do not reliably distinguish benign primary follicular mucinosis from secondary follicular mucinosis, associated with cutaneous lymphoma. More recently, it has been suggested that individuals with follicular mucinosis demonstrating a clonal T-cell receptor gene rearrangement may be at higher risk for the development of lymphoma. Long-term follow-up of 7 patients younger than 40 years with primary follicular mucinosis are reported. In all cases, there was no clinical or histologic evidence of associated dermatoses or lymphoma at the time of diagnosis. Five of the patients have clonal T-cell gene rearrangement as determined by Southern blot analysis. Clinically, at the time of diagnosis, lesions of primary follicular mucinosis ranged from papules confined to the face to widespread cutaneous plaques. After a mean follow-up of 10 years (range, 5-23 years) from the onset of disease, the majority of patients continue to have cutaneous manifestations of follicular mucinosis despite various treatments. There is no evidence of progression to cutaneous T-cell lymphoma in any patient despite the presence of a clonal T-cell receptor gene rearrangement. Continued prolonged follow-up of patients with clonal primary follicular mucinosis is necessary to determine the significance of infiltrates harboring a T-cell receptor gene rearrangement. However, in our experience with this group of selected patients, primary follicular mucinosis has been a clonal disorder with limited or "benign" cutaneous manifestations.

Authors+Show Affiliations

Department of Dermatology and Section of Dermatopathology, Mayo Clinic, Rochester 55905, USA.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article

Language

eng

PubMed ID

12451369

Citation

Brown, Holly A., et al. "Primary Follicular Mucinosis: Long-term Follow-up of Patients Younger Than 40 Years With and Without Clonal T-cell Receptor Gene Rearrangement." Journal of the American Academy of Dermatology, vol. 47, no. 6, 2002, pp. 856-62.
Brown HA, Gibson LE, Pujol RM, et al. Primary follicular mucinosis: long-term follow-up of patients younger than 40 years with and without clonal T-cell receptor gene rearrangement. J Am Acad Dermatol. 2002;47(6):856-62.
Brown, H. A., Gibson, L. E., Pujol, R. M., Lust, J. A., & Pittelkow, M. R. (2002). Primary follicular mucinosis: long-term follow-up of patients younger than 40 years with and without clonal T-cell receptor gene rearrangement. Journal of the American Academy of Dermatology, 47(6), 856-62.
Brown HA, et al. Primary Follicular Mucinosis: Long-term Follow-up of Patients Younger Than 40 Years With and Without Clonal T-cell Receptor Gene Rearrangement. J Am Acad Dermatol. 2002;47(6):856-62. PubMed PMID: 12451369.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Primary follicular mucinosis: long-term follow-up of patients younger than 40 years with and without clonal T-cell receptor gene rearrangement. AU - Brown,Holly A, AU - Gibson,Lawrence E, AU - Pujol,Ramon M, AU - Lust,John A, AU - Pittelkow,Mark R, PY - 2002/11/27/pubmed PY - 2003/1/10/medline PY - 2002/11/27/entrez SP - 856 EP - 62 JF - Journal of the American Academy of Dermatology JO - J Am Acad Dermatol VL - 47 IS - 6 N2 - Since the original descriptions of follicular mucinosis, accumulating experience shows that patient age, distribution of lesions, and duration or extent of disease do not reliably distinguish benign primary follicular mucinosis from secondary follicular mucinosis, associated with cutaneous lymphoma. More recently, it has been suggested that individuals with follicular mucinosis demonstrating a clonal T-cell receptor gene rearrangement may be at higher risk for the development of lymphoma. Long-term follow-up of 7 patients younger than 40 years with primary follicular mucinosis are reported. In all cases, there was no clinical or histologic evidence of associated dermatoses or lymphoma at the time of diagnosis. Five of the patients have clonal T-cell gene rearrangement as determined by Southern blot analysis. Clinically, at the time of diagnosis, lesions of primary follicular mucinosis ranged from papules confined to the face to widespread cutaneous plaques. After a mean follow-up of 10 years (range, 5-23 years) from the onset of disease, the majority of patients continue to have cutaneous manifestations of follicular mucinosis despite various treatments. There is no evidence of progression to cutaneous T-cell lymphoma in any patient despite the presence of a clonal T-cell receptor gene rearrangement. Continued prolonged follow-up of patients with clonal primary follicular mucinosis is necessary to determine the significance of infiltrates harboring a T-cell receptor gene rearrangement. However, in our experience with this group of selected patients, primary follicular mucinosis has been a clonal disorder with limited or "benign" cutaneous manifestations. SN - 0190-9622 UR - https://www.unboundmedicine.com/medline/citation/12451369/Primary_follicular_mucinosis:_long_term_follow_up_of_patients_younger_than_40_years_with_and_without_clonal_T_cell_receptor_gene_rearrangement_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0190962202003146 DB - PRIME DP - Unbound Medicine ER -