Mastocytosis and atopy: a study of 33 patients with urticaria pigmentosa

Müller, Ulrich; Helbling, Arthur; Hunziker, T.; Wüthrich, B; Pécoud, A; Gilardi, S.; Beretta, E; Fasel, J.; Messerli, W.; Maurer, P.

doi:10.1111/j.1398-9995.1990.tb00945.x

Cited by 59 publications

(36 citation statements)

References 12 publications

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“…Poor prognosis is associated with aggressive forms (ASM), SM associated with a hematological non-mast cell clonal disorder (SM-AHNMD) and mast cell leukemia (MCL). The incidence of atopic diseases such as asthma and allergic rhinitis is the same as in the general population [3]. …”

Section: Introductionmentioning

confidence: 99%

Mastocytosis: Mediator-Related Signs and Symptoms

Castells

Austen²

2002

Int Arch Allergy Immunol

115

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Patients with systemic mastocytosis present symptoms related to the tissue response to the release of mediators from mast cells and to the local mast cell burden. Such patients often have a history of chronic and acute mediator-related symptoms. Most patients have indolent disease with a good prognosis and a normal life span. Symptoms can include pruritus, flushing, syncope, gastric distress, nausea and vomiting, diarrhea, bone pain and neuropsychiatric symptoms, most of which are controlled by medication. Because there is no current cure for mastocytosis, successful therapeutic interventions rely on the recognition of mediator-related symptoms and their treatment, and established intervention approaches for the relatively uncommon leukemic concomitants. Efforts to link a particular mast cell-derived mediator to some aspect of the symptom complex depend on the known actions of the mediator and the efficacy of target-based interventions.

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Section: Introductionmentioning

confidence: 99%

Mastocytosis: Mediator-Related Signs and Symptoms

Castells

Austen²

2002

Int Arch Allergy Immunol

115

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“…Atopic disease prevalence in mastocytosis is reported to be similar compared to the general population [23,89], and the sBT value inferred no difference in mastocytosis patients in the allergic group vs the non-allergic group even when the two groups demonstrated a significant difference in IgE levels [23].…”

Section: Mastocytosis and Allergymentioning

confidence: 93%

“…In theory, one could speculate that the abundance of MC would make patients with mastocytosis more susceptible to allergic reactions. However, the low serum IgE typically associated with the disease is reflective of the relative low density of the IgE receptor on MC and basophils thereby decreasing the allergen bridge needed for activation [89].…”

Section: Mastocytosis and Allergymentioning

confidence: 99%

Cutaneous and Systemic Mastocytosis in Children: A Risk Factor for Anaphylaxis?

Matito

Carter²

2015

Curr Allergy Asthma Rep

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Childhood mastocytosis is usually a clonal mast cell disease related to activating mutations in KIT. The symptoms in childhood mastocytosis are typically cutaneous in nature although systemic symptoms including anaphylaxis due to the release of mast cells (MC) mediators can also manifest. The prevalence of anaphylaxis reported in childhood mastocytosis is higher than the rates reported in the pediatric general population, but lower than the prevalence of anaphylaxis described in adult mastocytosis. An extensive cutaneous involvement was reported as a risk factor for anaphylaxis, and patients with diffuse cutaneous mastocytosis have been documented to have more severe anaphylaxis symptoms. Anaphylaxis due to unknown causes or idiopathic anaphylaxis was the primary etiology in pediatric mastocytosis, followed by foods as the most relevant identified trigger; however, hymenoptera stings are not a frequent trigger of anaphylaxis in children with mastocytosis in contrast to the adult patients.

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“…On histopathological examination of the cutaneous lesions, mast cell accumulation is observed along the papillary dermis and reticular dermis and inside the subcutaneous adipose tissue (23). Therefore, the incidence of atopic disease is increased compared to the general population (24).…”

Section: Cutaneous Mastocytosismentioning

confidence: 99%

Current approach to cutaneous mastocytosis in childhood

Tamay

Özçeker

2016

Turk Pediatri Ars

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Mastocytosis is a heterogeneous disorder characterized by clonal proliferation and accumulation of mast cells in one of more organs which may lead to different clinical pictures. Pathological increase and activation of mast cells in various tissues can cause different clinical pictures. Cutaneous mastocytosis limited to the skin is the most typical clinical picture observed in children and systemic mastocytosis is very rare in the pediatric age group. The diagnosis of cutaneous mastocytosis is based on clinical findings, but is often delayed due to lack of clinical awareness of the disease and lack of its consideration in the differential diagnosis. This article focuses on the current diagnosis, management and treatment of cutaneous mastocytosis in children in order to increase awareness about this issue.(Turk Pediatri Ars 2016; 51: 123-7)

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Mastocytosis and atopy: a study of 33 patients with urticaria pigmentosa

Cited by 59 publications

References 12 publications

Mastocytosis: Mediator-Related Signs and Symptoms

Mastocytosis: Mediator-Related Signs and Symptoms

Cutaneous and Systemic Mastocytosis in Children: A Risk Factor for Anaphylaxis?

Current approach to cutaneous mastocytosis in childhood

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