Objective: To characterize the clinical features, response to therapy, evolution and prognosis of cutaneous mastocytosis in children.
Background: Mastocytosis in children, instead of being induced by a potentially oncogenic c-kit mutation, is probably a clonal disease with benign prognosis.
Methods: The clinicopathological features, evolution and response to treatment were analysed in 71 children with mastocytosis.
Results: There were 53 (75%) cases of urticaria pigmentosa, 12 (17%) cases of mastocytoma, and six (8%) cases of diffuse cutaneous mastocytosis. In 92% of cases disease onset was in the first year of life. There was a male predominance 1.8 : 1. Treatment did not modify the disease evolution. Eighty per cent of patients improved or had spontaneous resolution of the disease.
Conclusion: The most frequent clinical form of mastocytosis was urticaria pigmentosa followed by mastocytoma and diffuse cutaneous mastocytosis. Darier's sign was present in 94% of cases. A negative Darier's sign does not rule out mastocytosis. In contrast to adults, mastocytosis in children usually has a benign course making sophisticated or invasive diagnostic tests unnecessary. A classification of paediatric cutaneous mastocytosis is proposed.
Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.1111/j.1468-3083.2004.00830.x | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Eruptive Syringoma-Clinical, Dermoscopic, and Reflectance Confocal Microscopy Features.
Diagnostics (Basel)
January 2025
Department of Dermatology, Venereology and Allergology, Medical University of Gdańsk, 80-214 Gdańsk, Poland.
We present an interesting image of eruptive syringoma confirmed by histopathological assessment in a 37-year-old male who was consulted due to numerous brownish small macules and papules resembling maculopapular cutaneous mastocytosis (MPCM). We show difficulties in diagnosing ES, given its rare occurrence and resemblance to other dermatological disorders. Moreover, we discuss the role of dermoscopy and reflectance confocal microscopy in the differential diagnosis of syringoma.
View Article and Find Full Text PDFExploring Pediatric Dermatology in Skin of Color: Focus on Dermoscopy.
Life (Basel)
December 2024
Institute of Dermatology, Department of Medical Area, University of Udine, 33100 Udine, Italy.
This literature review aims to comprehensively evaluate the clinical and dermoscopic presentations of common pediatric diseases among children with skin of color (SoC) while also addressing potential variations based on racial backgrounds. This review encompasses various conditions, such as nevi subtypes, viral infections, infestations, and inflammatory dermatoses, as well as hair diseases and abnormal vascular formations, occurring in pediatric populations. Overall, we identified 7 studies on nevi subtypes, 24 studies on skin infections, 6 on inflammatory dermatoses, 10 on hair diseases and disorders, and 14 on miscellaneous disorders that also satisfied our SoC- and race-specific criteria.
View Article and Find Full Text PDFEvaluation of allergic diseases in children diagnosed with cutaneous mastocytosis.
Allergy Asthma Proc
January 2025
Department of Pediatric Allergy and Immunology, Health Sciences University, Ankara City Hospital, Ankara, Turkey.
Cutaneous mastocytosis (CM) is the most common type of mastocytosis in children. The atopy frequency in these patients is typically similar to that in the general population, but a higher incidence of anaphylaxis is reported. This study aimed to evaluate the presence of allergic diseases in children diagnosed with CM and its impact on clinical manifestations.
View Article and Find Full Text PDFRecalcitrant Pustular Dermatosis Successfully Treated With Prolonged Isotretinoin Therapy.
Cureus
November 2024
Dermatology, Imperial Dermatology, Hollywood, USA.
Isotretinoin (13-cis-retinoic acid) is a well-established systemic treatment for moderate to severe acne vulgaris, renowned for its ability to target multiple contributors to acne pathogenesis. However, its therapeutic potential extends beyond conventional acne management. This case report highlights its efficacy in treating recalcitrant pustular dermatosis, a condition that proved resistant to standard therapies and posed significant diagnostic challenges.
View Article and Find Full Text PDFThe Mastocytosis Control Test: A Patient-Reported Outcome Measure Assessing Disease Control.
J Allergy Clin Immunol Pract
December 2024
Institute of Allergology, Charité-Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany; Fraunhofer Insititute for Translational Medicine and Pharmacology ITMP, Immunology and Allergology, Berlin, Germany.
Background: Mastocytosis is characterized by expanding neoplastic mast cells in organs such as the skin, bone marrow, and gastrointestinal tract. The release of mast cell mediators triggers cutaneous, gastrointestinal, and other symptoms. Currently, no validated mastocytosis-specific patient-reported outcome measure (PROM) exists to assess disease control.
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!