Folliculotropic mycosis fungoides in a pediatric patient with response to psoralen‐ultraviolet A phototherapy

Abstract

Mycosis Fungoides is the most common cutaneous T-cell lymphoma however, it is rare in children. We present the case of a Folliculotropic Mycosis Fungoides in a 13-year-old boy which initially presented as a plaque on his face and was treated with local PUVA therapy. Afterwards the lesions spread so the treatment was changed to systemic PUVA with good response. Although the experience in the treatment of Mycosis Fungoides in pediatric patients is limited, PUVA therapy seems to be an effective and safe option.

Folliculotropic Mycosis Fungoides: Current Guidance and Experience from Clinical Practice

“…Disease stages exhibited in the literature ranged from stage IA to IVA2; in 8 studies only earlier stages (maximum IIA) were evaluated, in 7 studies more advanced stages (from IIB) were also included. The most widely used treatment was Psoralen Ultra-Violet A (PUVA), reported in 8 studies, in 3 as monotherapy (1 case report with CR, 1 original article with CR 70% and PR 26%, 1 original article with ORR of 76%) 5 , 24 , 25 and in 5 studies in association with other drugs: in 2 case reports PUVA was combined with bexarotene, with CR in both cases and subsequent stem cell transplantation following progression in one of them, 27 , 28 whilst in another report it was associated with Interferon, with CR. 31 This treatment, among others, was mentioned in the Japanese case series by Kamijo et al, yet it was less commonly used compared to UVB therapy (ie, 10% vs 50% of the patients in the cohort).…”

“…A total of 38 records was initially identified through a literature search, 19 of which were duplicates. After screening for eligibility and inclusion criteria, 15 articles were ultimately included [20][21][22][23][24][25][26][27][28][29][30][31][32] (Table 1). Most publications were case reports (n=7), followed by case series (n=4), original articles (n=3), and clinical image (n=1).…”

“…4 Looking at the recent data reported in the literature, more than half of the analyzed papers did not include advanced disease stages (≥IIB), thus showing generally higher ORR rates for the therapies reported. [20][21][22][23][24][25][26][27][28][29][30][31][32] On the contrary, looking at those studies which included more advanced stages, such as the papers by van Santeen et al and Laggis et al, a significantly higher fluctuation of ORR could be observed (ie, PR and CR ranging from 25-24% to 48-51%, respectively). 5,32 Our case history shows quite similar responses, with CR achieved mainly in early stages.…”

“…Regarding the treatments used in FMF, in the absence of specific guidelines and clinical prospective studies, a high number of treatments are described in clinical practice, with variable results. [20][21][22][23][24][25][26][27][28][29][30][31][32] Phototherapy, retinoids (ie acitretin, bexarotene, isotretinoin) and topical steroids represent the most widely described non-ionizing therapies (Figure 3), with different results. 3,5,[24][25][26][27][28][29][30][31][32] Phototherapy in all its forms, especially as PUVA, appears to have the greatest initial therapeutic success, although it is more commonly proposed in the early stages of the disease.…”

“…[20][21][22][23][24][25][26][27][28][29][30][31][32] Phototherapy, retinoids (ie acitretin, bexarotene, isotretinoin) and topical steroids represent the most widely described non-ionizing therapies (Figure 3), with different results. 3,5,[24][25][26][27][28][29][30][31][32] Phototherapy in all its forms, especially as PUVA, appears to have the greatest initial therapeutic success, although it is more commonly proposed in the early stages of the disease. Both in the literature and in our case history, the first line of treatment is topical steroid therapy, often already initiated by the patient before the actual diagnosis of FMF.…”