Department of Dermatology, Venereology and Pediatric Dermatology, Medical University of Lublin, Lublin, Poland.
Dermatol Ther. 2013 Mar-Apr;26(2):176-9. doi: 10.1111/j.1529-8019.2013.01544.x.
Diffuse cutaneous mastocytosis is the rarest subtype of mastocytosis among those that affect children. A 7-month-old girl presented at birth dry and thickened skin. On the second day of life, tense blisters appeared on erythrodermic areas and during the next days spread over the face, trunk, and limbs. The skin was bright red, had a "peau d'orange" appearance, and was considerably thickened with numerous translucent and hemorrhagic blisters. A skin biopsy revealed massive infiltration of the dermis with mast cells, leading to the diagnosis of diffuse erythrodermic mastocytosis. Systemic corticosteroids were given along with antihistamines with good results. Despite the progresses in the understanding of pathogenesis, genetics, and diagnostic criteria of mastocytosis, clear and reliable prognostic markers are still lacking, especially in order to predict systemic involvement. not only in diffuse but also in the commoner forms of the disease.
弥漫性皮肤肥大细胞增多症是儿童中最罕见的肥大细胞增多症亚型。一名 7 月龄女婴出生时皮肤干燥且增厚。出生后第 2 天，出现紧张性水疱，随后几天红斑区域水疱逐渐扩散至面部、躯干和四肢。皮肤呈鲜红色，呈“橘皮样”外观，且明显增厚，伴有大量透明和出血性水疱。皮肤活检显示真皮内大量肥大细胞浸润，导致弥漫性红皮性肥大细胞增多症的诊断。给予全身皮质类固醇和抗组胺药治疗，取得良好效果。尽管在肥大细胞增多症的发病机制、遗传学和诊断标准方面取得了进展，但仍缺乏明确和可靠的预后标志物，尤其是在预测系统受累方面。不仅在弥漫性疾病中，而且在更常见的疾病形式中也是如此。
