# Port-wine stain - Category: chronic - Review status: reviewed (every claim checked against a document named on this page) - Sources: ICPC-3 (WONCA International Classification of Primary Care, 3rd edition) class SD55.00 - condition scope only, no dose · No dose - referral pathway, no medicine given in primary care · Sturge-Weber Syndrome - StatPearls - NCBI Bookshelf - https://www.ncbi.nlm.nih.gov/books/NBK459163/ · Nevus Flammeus - StatPearls - NCBI Bookshelf - disease-level clinical article (port-wine-stain-full.txt) - Verified date: 2026-08 ## Verified against - No dose - referral pathway, no medicine given in primary care - Nevus Flammeus - StatPearls - NCBI Bookshelf - disease-level clinical article (port-wine-stain-full.txt) ## Treatment metadata - No drug therapy in primary care (Referral & Advice) ## Complete treatment card ```text PORT-WINE STAIN Sources: ICPC-3 (WONCA International Classification of Primary Care, 3rd edition) class SD55.00 - condition scope only, no dose · No dose - referral pathway, no medicine given in primary care · Sturge-Weber Syndrome - StatPearls - NCBI Bookshelf - https://www.ncbi.nlm.nih.gov/books/NBK459163/ · Nevus Flammeus - StatPearls - NCBI Bookshelf - disease-level clinical article (port-wine-stain-full.txt) Review status: REVIEWED against 2 sources listed above (2026-08) IS IT THIS? - reference only, to read alongside your own examination SYMPTOMS - what the patient reports (4) - The mark does not hurt, does not bleed by itself, and feels no warmer than surrounding skin - It enlarges in step with the growth of the child - Over the years it deepens in colour, turning purplish - In Sturge-Weber, fits often start by two years old, sometimes with opposite-sided neurological deficit and learning difficulty [learning difficulty · seizures] SIGNS - what you find (12) - Pink or red uniform macules and patches of varying size with map-like edges, present from birth and lifelong - Skin of the head and neck is involved in 70% to 90% of cases; trunk or limbs are possible and mucosa rarely - On the face it is usually segmental, following the trigeminal nerve territory - Facial lesions thicken and hypertrophy in later life, with or without nodules, as dermal vessels progressively dilate - Overgrowth of the fat, muscle and bone beneath is rare and mostly seen with facial lesions - A stain over the midline lumbosacral region may be a skin marker of hidden spinal dysraphism - Forehead involvement, taking in upper eyelid and midline frontonasal skin, is the single strongest predictor of Sturge-Weber - Highest CNS risk comes with widespread bilateral disease, hemifacial forehead involvement, or a midline stain over more than half of one forehead side - Limb overgrowth alongside a combined capillary, lymphatic and venous malformation suggests Klippel-Trenaunay syndrome - Multiple scattered stains with a pale halo, plus arteriovenous malformation, point to the RASA1 capillary malformation-arteriovenous malformation syndrome [pallor] - An acquired form, Fegeler syndrome, follows trauma and also drugs such as isotretinoin, the pill, simvastatin and metformin, plus frostbite and herpes zoster - A choroidal angioma in Sturge-Weber can bring on glaucoma, buphthalmos or retinal detachment TESTS (6) - Diagnosis rests on the clinical appearance; assess a facial lesion at birth - Colour Doppler ultrasound gauges flow resistance and picks out fast-flow arteriovenous malformations that mimic it - CT, MRI and MR angiography map superficial or deep tissue anomalies in combined malformations or a syndrome - Image the spine when a stain sits with another skin marker such as a hair tuft, haemangioma, lipoma, dermoid cyst or tail-like appendage - Brain imaging in Sturge-Weber can show cerebral atrophy with gyriform calcification - Biopsy is not routine, but serves to rule out mimics, prove an acquired lesion, and provide tissue for genetic study IF NOT THIS - what else fits (8) - Salmon patch - Early haemangioma - Arteriovenous malformation - Tufted angioma - Eccrine angiomatous hamartoma - Cutis marmorata telangiectatica congenita - Segmental infantile haemangioma, which calls for a PHACES workup - Nevus simplex Source StatPearls "Nevus Flammeus" - disease-level clinical article Status traced to the source above 1. NO DRUG THERAPY IN PRIMARY CARE (REFERRAL & ADVICE) [1st line] Adult A congenital vascular birthmark; primary care's role is recognition, reassurance, and referral to dermatology/laser clinic for cosmetic treatment, plus screening for associated syndromes if periorbital. - Refer, with advice Peds Children follow the same pathway: recognise and refer. No primary-care medicine is implied. Source No dose - referral pathway, no medicine given in primary care Why A congenital vascular birthmark; primary care's role is recognition, reassurance, and referral to dermatology/laser clinic for cosmetic treatment, plus screening for associated syndromes if periorbital. Caution A port-wine stain in the trigeminal (upper facial) distribution raises concern for Sturge-Weber syndrome - refer for neurological and ophthalmological assessment. Rare in Egyptian primary care. No medicine is prescribed for this in primary care - this entry is for recognition and referral. Anything given is decided by the service it is referred to. RED FLAG - Glaucoma risk specifically named for periocular/eyelid PWS (app says only 'screening for associated syndromes if periorbital', without naming glaucoma) RED FLAG - Regular follow-up is required for high-risk cases (eyelid involvement, combined vascular malformations, syndromic PWS). A port-wine stain involving any part of the forehead, the upper eyelid, or the midline frontonasal area is the strongest indicator of Sturge-Weber syndrome - refer on forehead involvement even when the stain does not follow a classic trigeminal pattern. Children with Sturge-Weber syndrome often develop seizures by the age of 2 years, which may be accompanied by contralateral neurological deficits and learning difficulties - warn parents what to watch for. A port-wine stain over the midline lumbosacral area can be a cutaneous marker of occult spinal dysraphism. Imaging is indicated when a port-wine stain is accompanied by another cutaneous marker such as a tuft of hair, a haemangioma, a lipoma, a dermoid cyst, or a true or pseudo tail. A solitary nodule arising on a port-wine stain in adulthood may be a pyogenic granuloma, which is often ulcerated and bleeds after trivial trauma. Refer for laser treatment early: starting in infancy gives more consistent improvement, better outcomes, and a lower risk of later skin thickening and disfigurement. Where the stain involves mucosa, gingival hyperplasia can develop and predispose to periodontal disease and poor dental hygiene - arrange dental review. Prices are indicative (dataset snapshot 2026-06); verify with the pharmacy. ``` --- Dawaa Reference is a reference for prescribers, not a medical device, and does not replace clinical judgement. [Privacy policy](/privacy)