Cases of Childhood Mastocytosis: A Single Center Experience

Year 2024, , 60 - 64, 30.05.2024

Seda Çevik , Ugur Altas , Fatih Çiçek , Zeynep Meva Altaş , Ayşen Çetemen , Mehmet Yaşar Özkars

https://doi.org/10.56766/ntms.1433524

Abstract

Objective: Cutaneous mastocytosis, primarily affecting children, is confined to the skin and generally carries a good prognosis. In our study, we aimed to evaluate the clinical findings, laboratory values and treatment-related data of 10 patients who were followed up with a diagnosis of mastocytosis in our clinic between 2014 and 2022.
Methods: Age, gender, family history, clinical findings, type of lesions, laboratory values and treatment-related data of the patients were analyzed within the scope of the study. Skin biopsy was taken from clinically suspected patients and the diagnosis was made with histopathologic confirmation. Histopathologic diagnosis was made by demonstration of mast cells showing metachromasia with toluidine blue in full-thickness skin biopsy.
Results: The median age at presentation was 10.0 months (min-max: 1.0-117.0). While rash and pruritus were the most common complaints seen in all patients; erythema was seen in 9 (90%) patients. The most common rash type was maculopapular. One (10.0%) patient had nodules and mastocytoma. When the laboratory findings of the patients were evaluated, no patient had thrombocytopenia or leukopenia. One patient had anemia. The median value of total IgE values was 65.0 IU/ml (8.0-1719.0).
Conclusion: In our study, all patients had symptoms of rash and pruritus. The most common lesion type in our study was maculopapular rash (UP type) seen in 4 patients (40%). Nodules and mastocytoma (NM type) were seen in 1 patient (10%). In our study covering an eight-year period, all of our patients had cutaneous mastocytosis and none of them had systemic involvement.

Keywords

Mastocytosis, rash, urticaria pigmentosa

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