Port-wine stain in Adult
See also in: External and Internal EyeAlerts and Notices
Synopsis
A port-wine stain may be isolated or may occur as part of a syndrome. The most common syndromes associated with such capillary malformations are as follows:
- Sturge-Weber syndrome (encephalotrigeminal angiomatosis) – There are cerebral and meningeal lesions in addition to a cutaneous lesion on the forehead. Patients may have seizures, glaucoma, intellectual disability, hemiplegia, or subdural hemorrhages.
- Klippel-Trenaunay syndrome (angio-osteohypertrophy) consists of a triad of vascular stain, hemihypertrophy, and venous varicosities. In the past, the vascular stain has been thought to be a pure capillary malformation; however, it is now recognized that it is more commonly a combined capillary, lymphatic, and/or venous malformation. Some specialists prefer to characterize this syndrome with a description of the specific malformations found in each patient.
- Phakomatosis pigmentovascularis is characterized by the coexistence of a capillary malformation and a cutaneous pigmented lesion. Both are typically present at birth.
Codes
ICD10CM:Q82.5 – Congenital non-neoplastic nevus
SNOMEDCT:
416377005 – Port-wine stain of skin
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Diagnostic Pearls
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Differential Diagnosis & Pitfalls
- Salmon patches – These are small capillary dilatations commonly occurring in the midline on the head (stork bites, angel kisses). They decrease in color with age (except for those on the nape of the neck, which tend to persist) and usually do not require treatment.
- Early infantile hemangiomas in children
- Sturge-Weber syndrome
- Klippel-Trenaunay syndrome
- Cobb syndrome
- Proteus syndrome
- Rubinstein-Taybi syndrome
- Megalencephaly-capillary malformation-polymicrogyria syndrome (MCAP)
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Therapy
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References
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Last Reviewed:11/16/2017
Last Updated:09/30/2018
Last Updated:09/30/2018
Port-wine stain in Adult
See also in: External and Internal Eye