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[Classification and management of mastocytosis in the child]
S Loubeyres1, C Léaute-Labrèze, S Roul
1Unité de dermatologie pédiatrique, Hôpital Pellegrin-Enfants, Bordeaux.
Insights
Childhood mastocytosis, presenting as urticaria pigmentosa or mastocytoma, typically resolves during growth. Bullae or xanthelasmoid lesions do not indicate a poor prognosis, and codeine avoidance is advised.
Area of Science:
- Pediatric dermatology
- Hematology
Context:
- Mastocytosis is a common pediatric skin condition.
- Understanding its varied presentations and prognosis is crucial for management.
Purpose:
- To analyze the initial manifestations and clinical course of childhood mastocytosis.
- To identify prognostic indicators and inform management strategies.
Summary:
- Retrospective study of 49 pediatric mastocytosis cases (urticaria pigmentosa and mastocytoma).
- Typical presentations included pigmentary urticaria and mastocytoma; bullae and xanthelasmoid changes were also observed.
- Most cases showed spontaneous improvement and clinical cure during growth, with one duodenal infiltration case noted.
Impact:
- Dermal atopy and bullae development do not negatively impact the clinical course.
- Xanthelasmoid lesions suggest a potential link with juvenile xanthogranuloma.
- Advising against codeine-containing cough medicines and using antihistamines for pruritus are key management recommendations.
Objective:
Mastocytosis is a frequently observed condition in children. We analyzed the initial manifestations and clinical course.
Patients And Methods:
We restrospectively studied 49 cases of mastocytosis in children (29 boys and 20 girls) managed in our unit between 1985 and 1995. All of the children had typical manifestations. Photographic documents were available in all cases.
Results:
There was pigmentary urticaria in 32 cases and a mastocytoma in 17. Axanthelasmoid aspect and bullae were observed in some cases in both of these clinical forms. Complementary explorations demonstrated one case of duodenal mast cell infiltration. Excepting the case with skin and duodenal manifestation, all of our patients improved and clinical cure was obtained during growth.
Discussion:
The association of dermal atopia and mastocytosis does not influence the clinical course of these two conditions. The development of bullae does not appear to be a factor of poor prognosis. The xanthelasmoid aspect of the lesions and the similar course in childhood mastocytosis and juvenile xanthogranulma would suggest that a common process with a histological spectrum including mastocytoma and xanthoma is involved. In our experience, counselling against the use of anti-cough medicines containing codeine is an essential part of management. Antihistamine agents may be prescribed for pruritus.