Primary cutaneous lymphomas are a heterogeneous group of non‐Hodgkin lymphomas that affect the skin with no evidence of extracutaneous involvement at the time of diagnosis. Cutaneous T‐cell lymphomas (CTCL) are the most common cutaneous lymphoma with an overall incidence of 8.55 per million persons. 1
CTCL rather affects patients in later decades of life, but children and young adults can get affected too, with an estimated annual incidence of 0.1 and 0.3 per million persons in the age groups 0–9 and 10–19 years, respectively. 2
Due to its rarity and the fact that childhood CTCL can mimic benign dermatosis, establishing the right diagnosis is often challenging and does warrant a high level of suspicion for the disease. Alberti‐Violetti et al. 3 share their collected data on childhood CTCL of over 40 years of experience as a tertiary referral centre in Europe. The group claims to have published the largest cohort of paediatric CTCL patients (n = 101) to date and underline epidemiologic findings from previous studies. 4 Lymphomatoid papulosis (Lyp) and mycosis fungoides (MF) were the most common CTCL types identified. Interestingly, classic MF was the most common MF subtype in their cohort. 3 This stands in contrast to published data from other groups that included a high percentage of children with skin of colour where hypopigmented MF was the most prevalent subtype. 4 In the Middle East and Far Middle East, children with folliculotropic MF make up the largest group of paediatric MF patients. 5 Alberti‐Violetti et al. reported only a small number of non‐Caucasian children, which might explain why classic MF was the most prevalent variant in their cohort. 3 These data highlight the geographic differences one encounters when treating different ethnic groups. In a globalized world it is our duty as dermatologists to broaden our diagnostic horizon as a ‘one fits all approach’ is outdated and will leave certain patient groups behind. Furthermore, a timely diagnosis is important to ensure patient counselling and to enable treatment decisions.
Childhood MF usually carries an excellent prognosis, especially in those children that present with hypopigmented patch lesions and early disease stages. 4 Nevertheless, disease progression to tumour stage MF with lethal outcome does occur in childhood and adolescence.
In the present study by Alberti‐Violetti et al., 3 two patients had tumour stage MF at the time of diagnosis and three children died of MF during the follow‐up period underlining that although paediatric MF seems to be more indolent compared with adult‐onset MF, progressive disease is possible and warrants aggressive treatment.
The group also reports their data on juvenile LyP with a total of 48 paediatric onset cases and other rare and very rare CTCL types including an extremely rare case of paediatric Sezary syndrome in a 17‐year‐old patient, who achieved complete remission after a successful allogenic stem cell transplantation. 3
In summary, CTCL is rare during childhood. Although we were all taught the wise words ‘When you hear hoof beats, think horses, not zebras’, we do need to know what to look for to establish an early diagnosis when occasionally a zebra does stop by in our clinic. No need to say that these rare cases should be managed in referral centres with expertise in CTCL.
The important study by Alberti‐Violetti et al. in this issue confirms previous findings in a large cohort and provides insights into the epidemiology of juvenile CTCL in Europe. It is well written and provides a precise overview of the topic.
CONFLICT OF INTEREST STATEMENT
Author of the ISCL/USCLC/EORTC consensus recommendations for paediatric Mycosis fungoides. Advisory board and/or speakers honorary from Takeda and Kyowa Kirin.
Wohlmuth I. Skin lymphomas in childhood: We only see what we know, we only know what we see. J Eur Acad Dermatol Venereol. 2025;39:25–26. 10.1111/jdv.20445
Linked article: S. Alberti‐Violetti et al. J Eur Acad Dermatol Venereol 2025;39:161–170. https://doi.org/10.1111/jdv.20028.
DATA AVAILABILITY STATEMENT
Data sharing is not applicable to this article as no new data were created or analysed in this study.
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Data Availability Statement
Data sharing is not applicable to this article as no new data were created or analysed in this study.