Escande H, Bennani I, Bulai Livideanu C, Uthurriague C, Paul C, Nougué J
Service de dermatologie, centre hospitalier de Montauban, 100, rue Léon-Cladel, BP 765, 82013 Montauban cedex, France; Service de dermatologie, hôpital Larrey, université Paul-Sabatier, CHU, 24, chemin de Pouvourville, TSA 30030, 31059 Toulouse cedex 9, France.
Ann Dermatol Venereol. 2013 Oct;140(10):641-4. doi: 10.1016/j.annder.2012.06.054. Epub 2013 Jun 24.
Anaphylaxis is a severe, generalized, life-threatening reaction of rapid onset. We report the case of a patient presenting several systemic anaphylactic reactions over many years, initially ascribed to a cereals allergy but which finally proved to be due to systemic mastocytosis hidden for a long time.
A 53-year-old man consulted for an eruption consisting of monomorphic pigmented maculopapular lesions on the trunk associated with itching and urticaria. He was a farmer and presented severe sensitivity to cereals, with anaphylaxis, which continued despite withdrawal of these allergens. Skin and bone marrow infiltration, abnormal mast cells, positivity for c-kit 816 mutation and the persistent elevation of serum tryptase enabled a diagnosis of indolent systemic mastocytosis to be made.
In systemic mastocytosis anaphylaxis is an expected complication relating to the proliferation of mast cells and a massive increase in mediator release (non-immunological mechanism). All patients with severe and recurrent anaphylaxis should be analyzed for underlying mastocytosis by careful physical examination and assay of baseline tryptase.
过敏反应是一种严重的、全身性的、危及生命的速发型反应。我们报告了一例多年来出现多次全身性过敏反应的患者，最初认为是谷物过敏，但最终证实是由于长期隐匿的系统性肥大细胞增多症所致。
一名53岁男性因躯干出现由单形性色素沉着性斑丘疹组成的皮疹伴瘙痒和荨麻疹前来就诊。他是一名农民，对谷物高度敏感，出现过敏反应，尽管避免接触这些过敏原仍持续发作。皮肤和骨髓浸润、异常肥大细胞、c-kit 816突变阳性以及血清类胰蛋白酶持续升高，从而诊断为惰性系统性肥大细胞增多症。
在系统性肥大细胞增多症中，过敏反应是与肥大细胞增殖和介质释放大量增加相关的预期并发症（非免疫机制）。所有严重且反复发生过敏反应的患者都应通过仔细的体格检查和基础类胰蛋白酶检测来分析是否存在潜在的肥大细胞增多症。
