Juvenile mycosis fungoides: Cutaneous T-cell lymphoma with frequent follicular involvement

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• 作者：Emmilia Hodak ; MD^a ; ^f ; ^{hodake@post.tau.ac.il" class="auth_mail} ; Iris Amitay-Laish ; MD^a ; Meora Feinmesser ; MD^b ; ^f ; Batya Davidovici ; MD ; MSc^a ; Michael David ; MD^a ; ^f ; Alex Zvulunov ; MD ; MHA^c ; ^g ; Felix Pavlotsky ; MD^e ; ^f ; Isaac Yaniv ; MD^d ; ^f ; Gali Avrahami ; MD^d ; ^f ; Dan Ben-Amitai ; MD^c ; ^f
• 关键词：children ; cutaneous lymphoma ; folliculotropic mycosis fungoides ; juvenile ; mycosis fungoides ; pediatric
• 刊名：Journal of the American Academy of Dermatology
• 出版年：June 2014
• 年：2014
• 卷：70
• 期：6
• 页码：993-1001
• 全文大小：1652 K

文摘

The literature on mycosis fungoides (MF) in children/adolescents is sparse.

Objective

We sought to evaluate the characteristics of juvenile MF in a large cohort.

Methods

Data were collected on all patients with MF, aged 18 years or younger at the time of clinicopathologic diagnosis, who attended the Rabin Medical Center Dermatology Department, Petach Tikva, Israel, between 1994 and 2012 and were followed up prospectively.

Results

There were 50 patients (30 male; mean age 11.4 years at diagnosis); 18 (36%) had Fitzpatrick skin type IV or higher. All were given a diagnosis of early-stage disease (IA-IIA) except 1 (tumor stage, IIB). Eight had classic MF lesions only and 42 had other variants, alone or in combination; these were mainly hypopigmented MF (n = 29) and cases with subtle but clear clinicopathologic features of folliculotropic MF (FMF) (n = 18). Among the various skin-targeted therapies, psoralen plus ultraviolet A (systemic/bath) proved beneficial for FMF. During a follow-up period of 0.25 to 15 years (mean 4.5), 2 patients progressed from stage IA to IB or IIA.

Limitations

Relatively short follow-up is a limitation.

Conclusions

This case series shows that FMF is not uncommon in children and adolescents. It is characterized by more superficial clinical features and less heavy perifollicular lymphocytic infiltrates than adult FMF, and responds well to psoralen plus ultraviolet A. The prognosis of childhood FMF remains unclear.