Clinical features and course of cutaneous mastocytosis in 133 children

Schena, Donatella; Galvan, Arturo; Tessari, Gianpaolo; Girolomoni, Giampiero

doi:10.1111/bjd.14004

Cited by 14 publications

(13 citation statements)

References 13 publications

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“…We discovered that 62% of all pediatric patients with mastocytosis were diagnosed before 2 years of age. This percentage was considerably lower than the 90% reported by Meni et al (2015) , but because our results are based on unselected register-based information, we believe that the percentage of infantile onset is closer to 60% than to 90% of all pediatric cases, which is in line with the findings of Schena et al (2016) .…”

Section: Discussionsupporting

confidence: 82%

How “benign” is cutaneous mastocytosis? A Danish registry-based matched cohort study

Kibsgaard

Deleuran

Flohr

et al. 2020

International Journal of Women's Dermatology

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Background There are limited estimates of the incidence rates (IRs) of mastocytosis, and only a few studies have addressed the long-term consequences of living with these diagnoses. Previous reports have shown that systemic mastocytosis is associated with leukemic transformations and an increased risk of death as opposed to cutaneous mastocytosis (CM) and indolent systemic mastocytosis (ISM), which have benign diagnoses with life expectancy rates similar to those of the background population. Objective This study aimed to analyze the incidence and mortality of mastocytosis. Methods A population-based matched cohort study of patients with mastocytosis between 1 January 1, 1977 and 31 December 31, 2014 was identified from the Danish National Health Registries. IRs of CM, ISM, and pediatric mastocytosis were highlighted. Survival estimates were compared with those of a healthy background population, using a Cox proportional hazard model. Results A total of 1461 patients with mastocytosis were identified. The annual IR of overall mastocytosis was 1.1 per 100,000 person years (95% confidence interval [CI], 1.0–1.2). Among children, the IR was 1.8 per 100,000 person years (95% CI, 1.6–2.1). The prevalence of any comorbidity was twice as high among patients with mastocytosis compared with the population without mastocytosis (odds ratio: 2.1; 95% CI, 1.8–2.5). The Charlson Comorbidity Index–adjusted mortality among adult patients with mastocytosis was HR Cutaneous Mastocytosis 1.2 (95% CI, 0.8–1.9), HR Indolent Systemic Mastocytosis 1.9 (95% CI 1.4–2.5), and HR Systemic Mastocytosis 4.2 (95%, CI 1.9–9.4), respectively. Conclusion Based on an entire nation, with free health care at the point of access, we estimated an annual IR of mastocytosis and its subgroups. We discovered that patients with ISM had an increased risk of death compared with the general population. Our data supported the overall benign nature of CM diagnosed after age 2 years.

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Section: Discussionsupporting

confidence: 82%

How “benign” is cutaneous mastocytosis? A Danish registry-based matched cohort study

Kibsgaard

Deleuran

Flohr

et al. 2020

International Journal of Women's Dermatology

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“…Six of these reactions were due to b-lactam antibiotics, 3 to acetaminophen, and 2 to the measles, mumps, and rubella (MMR) vaccine. 109 In another study 111 children with MIS, increased basal serum tryptase levels, and extensive cutaneous involvement were identified as predictors for severe MC mediator release symptoms. However, only 4 had immediate hypersensitivity reactions to drugs with pruritus only (n 5 1) or together with hives (n 5 2) or without angioedema (n 5 1).…”

Section: Adverse Reactions To Antibiotics and Other Drugsmentioning

confidence: 97%

Adverse reactions to drugs and biologics in patients with clonal mast cell disorders: A Work Group Report of the Mast Cells Disorder Committee, American Academy of Allergy, Asthma & Immunology

Carter

Metcalfe

Matito

et al. 2019

Journal of Allergy and Clinical Immunology

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AAAAI Position Statements,Work Group Reports, and Systematic Reviews are not to be considered to reflect current AAAAI standards or policy after five years from the date of publication. The statement below is not to be construed as dictating an exclusive course of action nor is it intended to replace the medical judgment of healthcare professionals. The unique circumstances of individual patients and environments are to be taken into account in any diagnosis and treatment plan. The statement reflects clinical and scientific advances as of the date of publication and is subject to change.

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“…İleri yaş başlangıçlı olguların daha uzun süre semptomatik olduğu, daha geç remisyona girdiği ya da erişkin hayatta devam ettiği düşünülmektedir. 5,8,9 Çalışma grubumuzda da 1 olguda döküntü başlangıç yaşı 11 olup, tanı anında sistemik hastalık lehine bulgusu olmasa da geç tanı yaşı nedeni ile erişkin mastositoz benzeri seyir olasılığı açısından hasta daha yakın izleme alınmıştır.…”

Section: Gereç Ve Yöntemlerunclassified

“…Özellikle bazı mutasyonların persistan ve uzun süreli hastalıkla ilişkili olabileceği öne sürülmektedir. 4,5,7 Çalışmamızda, her olguda cilt biyopsi örneği olmakla birlikte, c-KIT mutasyon analizi yapılmaması çalışmamızın kısıtlayıcı özelliğidir.…”

Section: Gereç Ve Yöntemlerunclassified

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