Tags

Type your tag names separated by a space and hit enter

Follicular mycosis fungoides, a distinct disease entity with or without associated follicular mucinosis: a clinicopathologic and follow-up study of 51 patients.
Arch Dermatol. 2002 Feb; 138(2):191-8.AD

Abstract

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.

Authors+Show Affiliations

Department of Dermatology, B1-Q-93, Leiden University Medical Center, Albinusdreef 2, 2333 ZA Leiden, The Netherlands.No affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Multicenter Study

Language

eng

PubMed ID

11843638

Citation

van Doorn, Remco, et al. "Follicular Mycosis Fungoides, a Distinct Disease Entity With or Without Associated Follicular Mucinosis: a Clinicopathologic and Follow-up Study of 51 Patients." Archives of Dermatology, vol. 138, no. 2, 2002, pp. 191-8.
van Doorn R, Scheffer E, Willemze R. Follicular mycosis fungoides, a distinct disease entity with or without associated follicular mucinosis: a clinicopathologic and follow-up study of 51 patients. Arch Dermatol. 2002;138(2):191-8.
van Doorn, R., Scheffer, E., & Willemze, R. (2002). Follicular mycosis fungoides, a distinct disease entity with or without associated follicular mucinosis: a clinicopathologic and follow-up study of 51 patients. Archives of Dermatology, 138(2), 191-8.
van Doorn R, Scheffer E, Willemze R. Follicular Mycosis Fungoides, a Distinct Disease Entity With or Without Associated Follicular Mucinosis: a Clinicopathologic and Follow-up Study of 51 Patients. Arch Dermatol. 2002;138(2):191-8. PubMed PMID: 11843638.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Follicular mycosis fungoides, a distinct disease entity with or without associated follicular mucinosis: a clinicopathologic and follow-up study of 51 patients. AU - van Doorn,Remco, AU - Scheffer,Erik, AU - Willemze,Rein, PY - 2002/2/15/pubmed PY - 2002/4/3/medline PY - 2002/2/15/entrez SP - 191 EP - 8 JF - Archives of dermatology JO - Arch Dermatol VL - 138 IS - 2 N2 - 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. SN - 0003-987X UR - https://www.unboundmedicine.com/medline/citation/11843638/Follicular_mycosis_fungoides_a_distinct_disease_entity_with_or_without_associated_follicular_mucinosis:_a_clinicopathologic_and_follow_up_study_of_51_patients_ L2 - https://jamanetwork.com/journals/jamadermatology/fullarticle/vol/138/pg/191 DB - PRIME DP - Unbound Medicine ER -