Department of Dermatology, University of Cologne, Cologne, Germany; Department of Dermatology, University of Luebeck, Luebeck, Germany.
Servicio Central de Citometria (NUCLEUS), Centro de Investigacion del Cancer (IBMCC; CSIC/USAL) and Department of Medicine and IBSAL, University of Salamanca, Salamanca, Spain.
J Allergy Clin Immunol. 2016 Jan;137(1):35-45. doi: 10.1016/j.jaci.2015.08.034. Epub 2015 Oct 21.
Cutaneous lesions in patients with mastocytosis are highly heterogeneous and encompass localized and disseminated forms. Although a classification and criteria for cutaneous mastocytosis (CM) have been proposed, there remains a need to better define subforms of cutaneous manifestations in patients with mastocytosis. To address this unmet need, an international task force involving experts from different organizations (including the European Competence Network on Mastocytosis; the American Academy of Allergy, Asthma & Immunology; and the European Academy of Allergology and Clinical Immunology) met several times between 2010 and 2014 to discuss the classification and criteria for diagnosis of cutaneous manifestations in patients with mastocytosis. This article provides the major outcomes of these meetings and a proposal for a revised definition and criteria. In particular, we recommend that the typical maculopapular cutaneous lesions (urticaria pigmentosa) should be subdivided into 2 variants, namely a monomorphic variant with small maculopapular lesions, which is typically seen in adult patients, and a polymorphic variant with larger lesions of variable size and shape, which is typically seen in pediatric patients. Clinical observations suggest that the monomorphic variant, if it develops in children, often persists into adulthood, whereas the polymorphic variant may resolve around puberty. This delineation might have important prognostic implications, and its implementation in diagnostic algorithms and future mastocytosis classifications is recommended. Refinements are also suggested for the diagnostic criteria of CM, removal of telangiectasia macularis eruptiva perstans from the current classification of CM, and removal of the adjunct solitary from the term solitary mastocytoma.
皮肤肥大细胞增多症患者的皮肤损害具有高度异质性,包括局限性和播散性形式。虽然已经提出了皮肤肥大细胞增多症(CM)的分类和标准,但仍需要更好地定义肥大细胞增多症患者的皮肤表现亚型。为了满足这一未满足的需求,一个由来自不同组织的专家组成的国际工作组(包括欧洲肥大细胞增多症网络;美国过敏、哮喘和免疫学学院;以及欧洲过敏和临床免疫学学院)在 2010 年至 2014 年期间多次开会,讨论皮肤肥大细胞增多症患者皮肤表现的分类和诊断标准。本文提供了这些会议的主要结果以及修订定义和标准的建议。特别是,我们建议将典型的斑丘疹性皮肤损害(色素性荨麻疹)分为 2 种变体,即形态单一的小斑丘疹性损害,通常见于成年患者,以及形态多样的大损害,其大小和形状多变,通常见于儿科患者。临床观察表明,如果形态单一的变体发生在儿童身上,通常会持续到成年,而形态多样的变体可能在青春期左右消退。这种划分可能具有重要的预后意义,建议在诊断算法和未来的肥大细胞增多症分类中实施。还建议对 CM 的诊断标准进行细化,从 CM 的当前分类中删除持久性毛细血管扩张性斑丘疹,以及从术语孤立性肥大细胞瘤中删除孤立性。