Mastocytosis: oral implications of a rare disease

Abstract

Mastocytosis encompasses a group of rare clinical entities, which are characterized by an abnormal growth and, usually, low accumulation of clonal and morphologically abnormal mast cells (MCs), within one or more organs. Clinical presentations are quite variable and symptoms are usually related to the release of mast cell mediators, tissue infiltration by MC (usually in the aggressive categories of the disease), or both. Mast cells are hematopoietic-derived cells that reach phenotypic maturity in the mucosa and peripheral connective tissues. These cells play an active role both on immunologic and non-immunologic processes. Within the oral cavity, MCs reside in the connective tissues, in physiologic conditions, and their number is elevated in pathologic situations resulting from immunoinflammatory processes, such as pulpal inflammation and periodontal disease. As MCs influence so many phenomena within the oral cavity, mastocytosis may manifest itself in the oral tissues. Patients with mastocytosis should be put under special care by dental professionals, in what concerns not only general patient management, but also drug prescription, as they are particularly prone to anaphylaxis and other peri and post-operative complications. Several allergens or mast cell activation triggers such as local anesthetics, zinc oxide, eugenol, penicilins, metals and oral hygiene products are frequently administered or prescribed by dentists. Patients with mastocytosis may also require stress management, during dental consultation. This review aims to briefly summarize the potential ways in which mast cell disease may affect the oral cavity and the dental management of mastocytosis affected patients.

Top-Down Proteomics of Human Saliva Discloses Significant Variations of the Protein Profile in Patients with Mastocytosis

“…49 It is necessary to underline that activated mast cells release cathepsins, in particular cathepsin C. 50 It can be supposed that an impaired cathepsin regulation in mastocytosis patients might lead to a major inclination through oral inflammation and mucosal lesions, which are frequently associated with the disease. 20,21,51,52 Moreover, because the salivary cystatins can be expressed in several tissues and secreted in other biological fluids, 43 the condition evidenced in saliva might reflect a systemic state, where the altered suppression of cathepsin activity results in a disproportionate inflammatory and allergic responses. Interesting results have been found about thymosin β4 and α-defensins 1 and 4, when the SM patients were divided based on the presence of additional cutaneous symptoms.…”

Dental treatment of a Seven-Year-Old Child with Mastocytosis

“…Children with mastocytosis should be monitored regularly by an allergist and their parents should be trained in how to manage attacks. Given their high propensity anaphylaxis, patients with mastocytosis should be tested for allergies prior to any dental treatment 12 . However, data may be difficult to interpret, and tests may be invaluable.…”

Clinical observations in cutan mastocytosis

“…Korábban mindegyik tünetre volt már példa az irodalomban [14,15,16,17,18,19]. Huszonkét betegünk közül 20 beteg panaszkodott fl ushre, valamint 10 esetben hasmenés és nyolc esetben fejfájás is előfordult a mediátor tünetek közül.…”