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[Mycosis fungoides in the child. Three cases]
E Vasseur1, M Rybojad, M D Vignon
1Service de Dermatologie, Hôpital Saint-Louis, Paris.
Introduction:
Cutaneous lymphoma is unusual in children but according to data in the literature, approximated 5 p. 100 of the cases observed would begin in childhood.
Case Report:
We retrospectively studied 3 cases of mycosis fungoides where the first manifestations occurred before 10 years of age. In one of the patients, the diagnosis was not definitively confirmed until adulthood.
Discussion:
Diagnosis in these forms which begin in childhood is usually achieved after a long delay. Clinically, these lymphomas form a homogeneous group. In approximately 15 p. 100, guttate parapsoriasis occurs before mycosis fungoides. Biopsy is indicated if the lesions change in aspect or become atypical. The most frequent presentation in children or young adults is vitiligoid hypo-pigmented macules. Histologically, childhood forms do not differ from the adult forms and also respond to local treatment as in adults. Prospective studies conducted conjointly by paediatricians and dermatologists would be needed to describe the natural history of these cutaneous lymphomas in light of progression to aggressive lymphoma of Hodgkin's disease described in certain cases.
Insights
Pediatric cutaneous lymphoma, specifically mycosis fungoides, often presents with delayed diagnosis. Early signs like hypopigmented macules require biopsy for accurate diagnosis and timely treatment.
Area of Science:
- Pediatric Dermatology
- Oncology
- Dermatopathology
Background:
- Cutaneous lymphoma is rare in children, accounting for approximately 5% of cases.
- Mycosis fungoides is the most common type of cutaneous lymphoma in pediatric patients.
Observation:
- Retrospective study of 3 mycosis fungoides cases with onset before age 10.
- One case required diagnosis in adulthood due to delayed identification.
Findings:
- Childhood cutaneous lymphoma diagnosis is often delayed, with 15% presenting as guttate parapsoriasis preceding mycosis fungoides.
- Vitiligoid hypopigmented macules are the most frequent presentation in children and young adults.
- Histological features of childhood mycosis fungoides are similar to adult forms and respond to local treatments.
Implications:
- Biopsy is crucial for atypical or changing lesions to ensure accurate diagnosis.
- Further prospective studies involving pediatricians and dermatologists are needed to understand the natural history and progression to aggressive lymphomas.
- Early recognition and management are vital for improving outcomes in pediatric cutaneous lymphoma.