Vascular Stains: Proposal for a Clinical Classification to Improve Diagnosis and Management

Pediatr Dermatol. 2016 Nov;33(6):570-584. doi: 10.1111/pde.12939. Epub 2016 Jul 25.


Vascular stains are a common reason for consultation in pediatric dermatology clinics. Although vascular stains include all vascular malformations, the term is most often used to refer to capillary malformations, but capillary malformations include a wide range of vascular stains with different clinical features, prognoses, and associated findings. The discovery of several mutations in various capillary malformations and associated syndromes has reinforced these differences, but clinical recognition of these different types of capillary vascular stains is sometimes difficult, and the multitude of classifications and confusing nomenclature often hamper the correct diagnosis and management. From our own experience and a review of the most relevant literature on this topic, we propose categorizing patients with capillary vascular stains into seven major clinical patterns: nevus simplex, port-wine stain, reticulated capillary malformation, geographic capillary malformation, capillary malformation-arteriovenous malformation (CM-AVM), cutis marmorata telangiectatica congenita, and telangiectasia. We also discuss the differential diagnosis of vascular stains as well as other conditions that can closely resemble capillary malformations and thus may potentially be misdiagnosed.

Publication types

  • Review

MeSH terms

  • Arteriovenous Malformations / pathology
  • Capillaries / abnormalities*
  • Humans
  • Infant, Newborn
  • Livedo Reticularis
  • Nevus / congenital
  • Port-Wine Stain
  • Skin Diseases, Vascular / pathology
  • Telangiectasis / congenital
  • Telangiectasis / pathology
  • Vascular Diseases / congenital
  • Vascular Diseases / diagnosis

Supplementary concepts

  • Cutis marmorata telangiectatica congenita