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[Lymphomatoid papulosis in childhood: six case reports and a literature review]
1Service de dermatologie, hôpital Hôtel-Dieu, 1 place de l'Hôpital, Lyon cedex 02, France.
Insights
Lymphomatoid papulosis (LyP) in children, a rare condition, presents similarly to adult forms. Diagnosis relies on histopathology and immunolabelling, with no consensus on treatment, though watchful waiting or corticosteroids are common first-line options.
Area of Science:
- Dermatology
- Pediatric Oncology
- Hematology
Background:
- Lymphomatoid papulosis (LyP) is a rare lymphoproliferative disorder classified under cutaneous lymphomas.
- While common in adults, LyP is infrequently reported in children, posing diagnostic and management challenges.
- This study presents six pediatric cases of LyP, contributing to understanding this rare condition in a younger population.
Observation:
- Six pediatric patients aged 2-11 years presented with episodic papulonodular skin lesions, some necrotic, on limbs and trunk.
- Histological and immunohistochemical analyses confirmed LyP type A in all cases.
- Key findings included CD30+ and CD15- cells within a dermal infiltrate of lymphocytes, neutrophils, eosinophils, and histiocytes.
Findings:
- Pediatric LyP, predominantly type A, shares clinical and histological features with adult presentations.
- The study highlights the diagnostic confirmation through histopathology and immunolabelling.
- A significant association with malignant lymphoma exists, posing a higher risk in children compared to the general population.
Implications:
- Early and accurate diagnosis of pediatric LyP is crucial, relying on specialized histopathological and immunolabelling techniques.
- While no treatment consensus exists, options range from observation and topical corticosteroids to methotrexate and phototherapy for severe cases.
- Regular clinical monitoring is advised for pediatric LyP patients due to the risk of associated lymphoma, but routine laboratory testing is not recommended.
Background:
Lymphomatoid papulosis (LyP) is a rare lymphoproliferative disorder. It is now included in the World Health Organisation (WHO) classification of cutaneous lymphomas. Although frequently described in adults, there have been only a few reported cases of LyP in children; diagnosis is often difficult in this population and no clear guidelines have been established regarding management or monitoring. In this article we report six new cases of LyP in children.
Patients And Methods:
This is a retrospective study of six children, aged between two and 11 years, seen at the Hôtel-Dieu Hospital in Lyon and at the Grenoble Hospital Centre between 2005 and 2008. Each child underwent skin biopsy for histological and immunohistochemical analysis.
Results:
All six children presented papulonodular lesions on the limbs and trunk, in some cases necrotic, present for different times and developing in episodes. Histology and immunolabelling announces in all six cases militated in favour of LyP type A, with large CD30+ and CD15- cells dispersed in an inflammatory dermal infiltrate made up for the most part of lymphocytes, polynuclear neutrophils, eosinophils and a small number of histiocytes.
Discussion:
Only around 60 cases of LyP have so far been reported in children, principally type A. Association with malignant lymphoma occurs, with high risk in relation to the general population of the same age. Clinical diagnosis is confirmed histopathology and immunolabelling. There is currently no consensus regarding therapeutic management. First-line treatment generally comprises therapeutic abstention or dermal corticosteroids. Methotrexate and phototherapy constitute possible alternatives but should be used only in very disseminate or debilitating forms of the disease.
Conclusion:
The presentation and course of LyP of childhood differs very little from the adult form. Cases of associated lymphoma have been reported. Although regular clinical monitoring is recommended, there is no call for routine laboratory testing.
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