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Original Investigation |

Clinical Staging and Prognostic Factors in Folliculotropic Mycosis Fungoides

Suzanne van Santen, MD1; Rachel E. J. Roach, MD, PhD1; Remco van Doorn, MD, PhD1; Barbara Horváth, MD, PhD2; Marjolein S. Bruijn, MD, PhD2; Cornelus J. G. Sanders, MD3; Jacco C. de Pooter, MD3; Michelle M. van Rossum, MD, PhD4; Ellen R. M. de Haas, MD, PhD5; Joep C. J. M. Veraart, MD, PhD6; Marcel W. Bekkenk, MD, PhD7; Maarten H. Vermeer, MD, PhD1; Rein Willemze, MD, PhD1
[+] Author Affiliations
1Department of Dermatology, Leiden University Medical Center, the Netherlands
2Department of Dermatology, University Medical Center of Groningen, the Netherlands
3Department of Dermatology, University Medical Center Utrecht, the Netherlands
4Department of Dermatology, Radboud University Medical Center, Nijmegen, the Netherlands
5Department of Dermatology, Erasmus Medical Center, Rotterdam, the Netherlands
6Department of Dermatology, Maastricht University Medical Center, the Netherlands
7Department of Dermatology, Academic Medical Center and Vrije University Medical Center, Amsterdam, the Netherlands
JAMA Dermatol. 2016;152(9):992-1000. doi:10.1001/jamadermatol.2016.1597.
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Importance  Large case series suggest that patients with folliculotropic mycosis fungoides (FMF) have a worse prognosis than patients with classic mycosis fungoides (MF). However, recent studies described a subgroup of patients with FMF with a more favorable prognosis. Distinction between indolent and aggressive FMF may have important therapeutic consequences but is hampered by the inability of the current tumor-node-metastasis-blood (TNMB) staging system to classify patients with FMF in a clinically meaningful way.

Objective  To differentiate between indolent and aggressive FMF using clinicopathological criteria and to define prognostic factors in patients with FMF.

Design, Setting, and Participants  In this prospective cohort study, we followed 203 patients with FMF, included in the Dutch Cutaneous Lymphoma Registry between October 1985 and May 2014 at a tertiary referral center hosting the Dutch Cutaneous Lymphoma Registry. Overall, 220 patients with FMF had been registered, but 17 patients with incomplete follow-up data or a history of classic MF were excluded.

Main Outcomes and Measures  Main outcomes included clinical and histological characteristics, disease progression, and survival. Prognostic factors were investigated using Cox proportional hazard regression analysis. Distinction between early plaque-stage FMF and advanced plaque-stage FMF was made by a blinded review of skin biopsy specimens from patients presenting with plaques.

Results  In a cohort of 147 men and 56 women (median [range] age, 59 [15-93] years), patients with histologically early plaque-stage FMF had a very similar overall survival (OS) rate to patients with only patches and/or follicular papules (10-year OS, 71% vs 80%), while the survival rate of patients with histologically advanced plaque-stage FMF was almost identical to that of patients presenting with tumors (10-year OS, 25% vs 27%). Subsequently, 3 clinical subgroups with significantly different survival data were distinguished: early skin-limited FMF (group A; n = 84; 5-year and 10-year OS, 92% and 72%); advanced skin-limited FMF (group B; n = 102; 5-year and 10-year OS, 55% and 28%); and FMF presenting with extracutaneous disease (group C; n = 17; 5-year and 10-year OS, 23% and 2%). Age at diagnosis, large cell transformation and secondary bacterial infection were independent risk factors for disease progression and/or poor survival.

Conclusions and Relevance  The results of this study provide useful criteria to differentiate between indolent and aggressive FMF and confirm the existence of a subgroup of FMF with a favorable prognosis.

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Figure 1.
Clinical Appearances of FMF

A, A solitary patch with associated alopecia in left eyebrow; B, grouped follicular papules on the trunk; C, alopecia and ulcerating tumors on the scalp; and D, plaques in the neck showing follicular accentuation and extensive secondary bacterial infection (arrows). FMF indicates folliculotropic mycosis fungoides.

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Figure 2.
Clinicopathologic Classification in 2 Patients With FMF Presenting With Similar Plaques on the Face

A, Histologic examination of a plaque on the left cheek of the first patient shows mucin depositions (follicular mucinosis) and (B) a sparse perifollicular and intrafollicular infiltrate. C, Detail of the infiltrate shows small neoplastic T cells with pleomorphic nuclei. D, Histologic examination of a plaque on the left temple of the second patient shows dense intrafollicular infiltrates with (E,F) a predominance of medium-sized to large neoplastic T-cells. FMF indicates folliculotropic mycosis fungoides.

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Figure 3.
OS and DSS of Patients With FMF

A and B, OS and DSS of patients with early and advanced plaque-stage FMF. C and D, OS and DSS of patients with early skin-limited FMF (group A), advanced skin-limited FMF (group B), and FMF presenting with extracutaneous disease (group C). DSS indicates disease-specific survival; FMF, folliculotropic mycosis fungoides; OS, overall survival.

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