Diagnosis and treatment of port-wine stains-associated syndromes
- VernacularTitle:鲜红斑痣相关综合征的诊疗进展
- Author:
Qingfeng LIU
1
;
Lian LIU
;
Ping DIAO
;
Xiaoxue LI
;
Ting ZHANG
;
Haotian CHEN
;
Xu LIU
;
Xian JIANG
Author Information
- Keywords: Port-wine stain; Syndrome; Sturge-Weber syndrome; Proteus syndrome; Neurocutaneous syndromes; Vascular malformations; Lymphatic abnormalities; Diagnosis
- From: Chinese Journal of Dermatology 2024;57(7):656-660
- CountryChina
- Language:Chinese
- Abstract: Port-wine stains (PWS) are one of the common congenital vascular malformations in dermatology, clinically manifesting as pink or red irregular patches occurring on the skin or mucosa at birth or shortly thereafter, which are often not elevated above the skin surface. In a minority of patients, vascular malformations not only affect the skin, but also involve the eyes, brain, limbs and viscera. These patients are at risk for glaucoma, epilepsy, limb pain, and other clinical conditions. In general, these conditions are referred to as PWS-associated syndromes. These syndromes are rare diseases, can affect multiple systems and exhibit a variety of clinical manifestations, which pose challenges in their diagnosis and treatment. This review focuses on the clinical manifestations, diagnoses, pathogenesis and treatment of PWS-associated syndromes.
