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皮肤萎缩性肥大细胞增多症:对补骨脂素紫外线A光疗法的反应。

Anetodermic mastocytosis: response to PUVA therapy.

作者信息

Del Pozo Jesús, Pimentel María Teresa Yebra, Paradela Sabela, Almagro Manuel, Martínez Walter, Fonseca Eduardo

机构信息

Department of Dermatology, Hospital Juan Canalejo, La Coruña, Spain.

出版信息

J Dermatolog Treat. 2007;18(3):184-7. doi: 10.1080/09546630701258093.

DOI:10.1080/09546630701258093
PMID:17538809
Abstract

Mastocytosis is a group of disorders characterized by the accumulation of mast cells in different tissues and organs. The skin is the most frequently involved organ (90% of cases) where mastocytosis may show a heterogenic clinical expression. Anetodermic lesions are an unusual clinical presentation of mastocytosis. We report a case of anetodermic mastocytosis in a 26-year-old man, with sparse lesions and a benign course. PUVA therapy obtained excellent results in this case. Darier's sign should be investigated in patients with anetodermic lesions of unclear origin in order to exclude cutaneous mastocytosis.

摘要

肥大细胞增多症是一组以肥大细胞在不同组织和器官中积聚为特征的疾病。皮肤是最常受累的器官(90%的病例),肥大细胞增多症在皮肤可能表现出异质性的临床症状。皮肤无弹性症皮损是肥大细胞增多症的一种不寻常临床表现。我们报告一例26岁男性的皮肤无弹性症性肥大细胞增多症,皮损稀疏,病程呈良性。补骨脂素紫外线A光疗法(PUVA疗法)在该病例中取得了极佳效果。对于病因不明的皮肤无弹性症皮损患者,应检查其 Darier 征,以排除皮肤肥大细胞增多症。

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Adult mastocytosis: a review of the Santo António Hospital 's experience and an evaluation of World Health Organization criteria for the diagnosis of systemic disease.
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