Mastocytosis – pathogenesis, clinical manifestation and treatment

Wagner, Nicola; Staubach, Petra

doi:10.1111/ddg.13418

Cited by 12 publications

(22 citation statements)

References 66 publications

(87 reference statements)

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“…In these patients, CM often presents as maculopapular brown skin lesions (MPCM), but may also present as erythroderma and pachyderma typical for diffuse cutaneous mastocytosis (DCM) or up to four nodular or plague lesions termed mastocytoma [ 8 ]. Regardless of the subform, CM may be associated with MC mediator-related symptoms in the skin such as pruritus, flushing, and blistering [ 34 , 35 , 36 ]. Generally, the probability for having these symptoms is more likely in patients with indurated skin lesions containing packed accumulations of MCs typically seen in DCM.…”

Section: Mediator-related Symptoms In Children With Mastocytosismentioning

confidence: 99%

“…Interestingly, patients with severe MC mediator-related symptoms or MCAS do not always have an elevated baseline serum tryptase level. Moreover, worsening of symptoms does not always correlate with an increase of tryptase level [ 4 , 35 ]. In children with mastocytosis, baseline level should be determined at least 24–48 h after the complete resolution of severe mediator-related symptoms [ 7 , 70 ].…”

Section: Diagnostic Testingmentioning

confidence: 99%

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Mediator-Related Symptoms and Anaphylaxis in Children with Mastocytosis

Brockow

Plata-Nazar

Lange

et al. 2021

IJMS

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Mastocytosis is characterized by the pathological accumulation of mast cells (MC) in various organs. In these patients, MC may degranulate and thereby contribute to clinical symptoms, especially when a concomitant allergy is present. However, MC activation can not only be induced by high-affinity receptors for IgE, but also by anaphylatoxins, neuropeptides, IgG immune complexes, complement-components, drugs, products of bacteria or parasites, as well as physical factors such as heat, cold, vibration, stress, sun, or physical effort. Symptoms due to mediators released by activated MC may develop in adults suffering from systemic mastocytosis, but also evolve in children who usually have cutaneous mastocytosis (CM). Clinically, CM is otherwise characterized by typical brown, maculopapular skin lesions or mastocytoma associated with a positive Darier’s sign. Pruritus and flushing are common and blistering may also be recorded, especially in diffuse CM (DCM). Pediatric patients with mastocytosis may also have gastrointestinal, respiratory, and neurologic complaints. Although anaphylaxis is not a typical finding, pediatric patients with massive skin involvement and high tryptase levels have a relatively high risk to develop anaphylaxis. This paper reviews MC mediator-related symptoms and anaphylaxis in children with mastocytosis, with special emphasis on risk factors, triggers, and management.

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Section: Mediator-related Symptoms In Children With Mastocytosismentioning

confidence: 99%

Section: Diagnostic Testingmentioning

confidence: 99%

Mediator-Related Symptoms and Anaphylaxis in Children with Mastocytosis

Brockow

Plata-Nazar

Lange

et al. 2021

IJMS

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“…Depending on the age of initial presentation, all cases can be divided into pediatric mastocytosis (65%) and adult mastocytosis (35%) (9). This classification has an important prognostic value (10).…”

Section: Epidemiology Of MCmentioning

confidence: 99%

Laboratory Tests in Diagnosis of Mastocytosis: Literature Review and Case Report

Орлова

Шкурлатовская

Silina

et al. 2020

Serbian Journal of Experimental and Clinical Research

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Mastocytosis is a heterogeneous group of disorders characterized by abnormal growth and accumulation of mast cells (MCs) in the skin and/or other organ systems. Mastocytosis is a rare disease. The annual incidence is 5–10 cases per 1 million people. However, the majority of cases stay undiagnosed due to the lack of specific tests and a wide variety of clinical features of the disease. In mastocytosis, somatic mutations of KIT gene lead to autocrine dysregulation and constitutive c-KIT activation in the absence of its ligand SCF. Clinical symptoms of the disease are determined by MC mediator release and/or infiltration of tissues by MCs. According to the World Health Organisation classification updated in 2016 mastocytosis is divided to cutaneous mastocytosis (CM), indolent systemic mastocytosis (ISM), smoldering systemic mastocytosis (SSM), SM with an associated hematologic (non-MC-lineage) neoplasm (SMAHN), aggressive SM (ASM), MC leukemia (MCL) and MC sarcoma (MCS). The CM and ISM prognosis is excellent with (almost) normal life expectancy, unlike aggressive forms (ASM and MCL) with poor prognosis. In this paper the key aspects of clinical features and diagnostic criteria of mastocytosis are discussed. We present a case report of a patient with mastocytosis in the skin following psoralen plus ultraviolet A (PUVA) therapy with good response.

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“…In der dermatologischen Praxis sollten Mastozytome als seltene Differenzialdiagnose von verrukösen bräunlichen Tumoren im Erwachsenenalter bedacht werden [ 10 ] . Diese können bei fehlender Diagnosestellung gigantische Ausmaße annehmen und eine interdisziplinäre therapeutische Herausforderung darstellen [ 11 ] .…”

Section: Ihre Diagnose? …unclassified