Thirty years old, white man, presented with congenital reddish slightly thicken facial lesion with scattered nodules on the left side, to dermatology clinic, seeking a cosmetic alleviate. He has no other complains (i.e., no bleeding, no headache, no eye pain or visual impairment). Also, the patient has noticed that the lesion slightly decreased with aging.
Family history (FH) was irrelevant.
Keyword: Nevus flammeus, Port-wine stain/ nevus, Childhood nevus, Birth marks, Salmon patch.
The man presented with a purplish-red lesion since birth on his face, with some nodules inside the lesion, comprising the forehead, eyelid, nose, upper lip and cheek, all of which on the left side. This birthmark can be diagnosed based on the physical examination and appearance, and upon the history, and it is known as a Nevus flammeus [1,2].
The man history shows no seizures, no eye pain, or visual complaints or developmental delay. It appeared he was asymptomatic. However, it seems that he might be stressed and suffered the stigma of the lesion being noticed by people, and thus embarrassment in his community from the facial lesion, and that’s why he shown up for cosmetic counsel.
A port wine stain or nevus flammeus is a vascular lesion in the skin, it can be superficial or deep, consists mostly from dilated capillaries, which gave the distinctive colouring of the lesion. Nevus flammus can sometimes be categorized into two, namely, port-wine stain and salmon patch. Salmon patches or angel's kisses are given when the lesion located on the forehead or eyelids, and a stork bites when located on the neck.
Numerous diverse labelling have been proposed, usually by applying descriptive terms or anatomic labels. The cosmetic appearance is an imperative consideration and treatment should be initiated therefore.
The port-wine stain can appear anywhere on the body, however most often it can be found on the face and limbs, on just one side of the body (unilateral). At birth it is found usually as a flat pink or a slightly elevated mark and enlarge as the child grows, into thicken lesion with small lumps or nodules, as the image of my case can display.
This patient nevus has involved apparently the first two branches of the trigeminus distribution as the image showed. This distribution would further require an investigation with thorough evaluation for port-wine stain. It has been stated as well that Port-wine stains are not only congenital but it can be acquired as well .
Physical inspection had shown a generalized vascular nevus covering his left side of the face, with some nodules as the image can exhibit.
Sporadic familial segregation of such anomalies advocates that there is genetic vulnerability .
It has been acknowledged in the literature that nevus flammeus and glaucoma are concurrent together, however it is a rare condition, and as a result patients should be assessed vigilantly to rule out such association, by appropriate referring to the ophthalmologist, when essential to measure the intraocular pressure (IOP), and request a CAT scan, or magnetic resonance imaging (MRI) of the brain for any linked leptomeningeal angiomas when in suspicion.
My patient conversely did not complain of sight problem though. Also left eye physically looked normal; no sclera discolouration, no conjunctiva congestion, nasal and oral cavity was devoid of any lesions. However, his chief worry alternatively was solely cosmetic matter, due to the emotional and social problems incurred. His early on childhood life was totally free, as he has been told by his parents.
The actual cause is yet not well-known, however as a recent research has revealed that port-wine stains are linked with a faulty nerve supply to petite blood vessels. The proposed theory that such nerves control the diameter of the blood vessels, and thus when they are faulty, the blood vessels persist to enlarge and thus expand, where blood will be collected under the skin's surface yielding vascular skin abnormality. Vascular malformations signify a permanent developmental fault. They are macular, sharply circumscribed, pink to purple, and diverse in size. Generally are found on the head and neck, and are unilateral. Port-wine stains, also called nevus flammeus, appear at birth and are localized to the trigeminal area, specifically around the eyelids.
Conventional management can be planned for uncomplicated cases during infancy stage; however the proliferative evolution of these lesions mandates an adequate treatment, whilst vascular malformations, which frequently persist throughout life, constantly necessitate therapeutic interference if they start to grounds clinical symptoms. Moreover, taking into consideration the individual parameters, like the thickness of the lesion, its locality and the growth rate, different treatment options, are available and can be tailored accordingly, including cryotherapy, corticosteroids injections, laser ablation, sclerotherapy, embolization and lastly surgery [3,4]. Presently, nevertheless, none of these treatments represents the solitary treatment preference.
1. Adams B, Lucky W. Acquired Port-wine Stains and Antecedent Trauma, ARCH DERMATOL. 2000 July.VOL 136, pg 897-899
2. Desiree L. Infant Facial Discoloration. Medscape Family Medicine. 2004
3. Redondo P, Aguado L, Martínez-Cuesta A. Diagnosis and management of extensive vascular malformations of the lower limb: part II. Systemic repercussions [corrected] diagnosis, and treatment. J Am Acad Dermatol. 2011 Nov; 65(5):909-23
4. Werner A, Dünne, A, Lippert M, Folz J. Optimal Treatment of Vascular Birthmarks. American Journal of Clinical Dermatology. 2003 - Volume 4 – (11) - pp 745-756
5. Eerola I, Boon LM, Mulliken JB et al. "Capillary malformation-arteriovenous malformation, a new clinical and genetic disorder caused by RASA1 mutations". Am. J. Hum. Genet. 2003, 73 (6): 1240–9. doi:10.1086/379793. PMC 1180390. PMID 14639529. http://linkinghub.elsevier.com/retrieve/pii/S0002-9297(07)63977-9.
6. Suprabha BS, Baliga M,, Total oral rehabilitation in a patient with portwine stains, case report, 2005, vol 23-(2)- pg 99-102.
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