Mast cell activation syndrome (MCAS) diagnosed using double-balloon enteroscopy

Mönkemüller, Klaus; Kaßalik, M.; Baraksei, D.; Fry, Lucía C.; Moege, Jürgen

doi:10.1055/s-0031-1291587

Cited by 1 publication

(1 citation statement)

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“…Since these first mentions of non-proliferative MCAD, the published case reports and series studies have accumulated evidence of extremely polymorphous cutaneous manifestations including migratory pruritus [154], dermatographism [126,[155][156][157], hives [153,158], waxing and waning diffuse migratory rash [154], edema/migratory edema [126], migratory abdominal wall cellulitis [155], xerostomia [155,158], telangiectasia, bleeding tendency [159], eczema, Reynaud's syndrome [157] and diaphoresis [160].…”

Section: Cutaneous Manifestationsmentioning

confidence: 99%

Mast Cell Activation Syndrome Update—A Dermatological Perspective

Mihele

Nistor

Bruma

et al. 2023

JPM

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Mast cells (MCs) are infamous for their role in potentially fatal anaphylaxis reactions. In the last two decades, a more complex picture has emerged, as it has become obvious that MCs are much more than just IgE effectors of anaphylaxis. MCs are defenders against a host of infectious and toxic aggressions (their interactions with other components of the immune system are not yet fully understood) and after the insult has ended, MCs continue to play a role in inflammation regulation and tissue repair. Unfortunately, MC involvement in pathology is also significant. Apart from their role in allergies, MCs can proliferate clonally to produce systemic mastocytosis. They have also been implicated in excessive fibrosis, keloid scaring, graft rejection and chronic inflammation, especially at the level of the skin and gut. In recent years, the term MC activation syndrome (MCAS) was proposed to account for symptoms caused by MC activation, and clear diagnostic criteria have been defined. However, not all authors agree with these criteria, as some find them too restrictive, potentially leaving much of the MC-related pathology unaccounted for. Here, we review the current knowledge on the physiological and pathological roles of MCs, with a dermatological emphasis, and discuss the MCAS classification.

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