Wilson Joshua, Walling Hobart W, Stone Mary Seabury
Department of Dermatology, University of Iowa, Iowa City, IA 52242, USA.
Pediatr Dermatol. 2007 Jan-Feb;24(1):18-24. doi: 10.1111/j.1525-1470.2007.00326.x.
Degos disease is a rare disorder, characterized by the development of typical atrophic porcelain-white macules on the trunk and extremities. It most commonly occurs in middle-aged Caucasian adults and eventuates in fatality in the majority of patients. A minority of reported patients have a more benign course and a favorable outcome. Degos disease is extremely rare in children. We report Degos disease in a teenager with an extended survival and the absence of internal involvement. We discuss the concept of benign cutaneous Degos and review the 34 patients (30 adults and four children) identified in the world literature with skin biopsy and at least 1 year of follow-up; the average age was 37.6 years; 26 were female and eight were male. As opposed to the malignant form of Degos disease, benign cutaneous Degos is more commonly reported in women (3:1). No laboratory abnormalities have been predictive of a benign versus malignant course. We also discuss the 24 reported instances of Degos disease (malignant and benign) in the pediatric population, of which 14 (58%) were fatal, with death occurring on average 3.6 years after diagnosis.
迪戈斯病是一种罕见的疾病，其特征是在躯干和四肢出现典型的萎缩性瓷白色斑疹。它最常见于中年白种成年人，大多数患者最终会死亡。少数报告的患者病程较为良性，预后良好。迪戈斯病在儿童中极为罕见。我们报告了一名青少年患迪戈斯病，生存期延长且无内脏受累。我们讨论了良性皮肤迪戈斯病的概念，并回顾了世界文献中经皮肤活检且至少随访1年确诊的34例患者（30名成年人和4名儿童）；平均年龄为37.6岁；26名女性，8名男性。与恶性迪戈斯病不同，良性皮肤迪戈斯病在女性中更为常见（比例为3:1）。没有实验室异常能够预测疾病是良性还是恶性病程。我们还讨论了儿科人群中报告的24例迪戈斯病（恶性和良性）病例，其中14例（58%）死亡，平均在诊断后3.6年死亡。
