Case Report

Nevus Flammeus: A Rare Case Report  

B. Kartheeki1 , Abhishek Singh Nayyar1 , A Ravikiran2 , Y Samatha2
1. Department of Oral Medicine and Radiology, Saraswati Dhanwantari Dental College and Hospital and Post-Graduate Research Institute, Parbhani, Maharashtra, India
2. Department of Oral Medicine and Radiology, Sibar Institute of Dental Sciences, Guntur, Andhra Pradesh, India
Author    Correspondence author
International Journal of Clinical Case Reports, 2016, Vol. 6, No. 25   doi: 10.5376/ijccr.2016.06.0025
Received: 08 Aug., 2016    Accepted: 08 Sep., 2016    Published: 10 Oct., 2016
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This is an open access article published under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Preferred citation for this article:

Kartheeki B., Nayyar A. S., Ravikiran A. and Samatha Y., Nevus Flammeus: A Rare Case Report, International Journal of Clinical Case Reports, 6(25): 1-5 (doi: 10.5376/ijccr.2016.06.0025)


Port-wine stain (PWS), also referred to as “nevus flammeus,” is a vascular malformation of skin which appears as red to violaceous patches on the surface of the skin. There is no hereditary predilection and no known risk factors. Rarely, may it, also, be acquired secondary to trauma. It comes under a group of disorders known as vascular malformations and may be seen as a part of syndromes like Sturge-Weber syndrome, a rare neuro-cutaneous disorder, sporadic in occurrence, without any identified underlying genetic abnormality. It is also termed as encephalo-trigeminal angiomatosis, manifesting clinically, as a cutaneous lesion, a nevus flammeus that involves the region innervated by the branches of the trigeminal nerve. Herewith, we are reporting a case with facial port wine stains with enlargement of middle third of face, upper lip and gingival tissues on right side as described.

Port wine stain (PWS); Sturge-Weber Syndrome; Nevus flammeus


Port-wine stain or, Nevus flammeus, is a capillary malformation characterized clinically by localized macular erythema and pathologically by ectasia of the dermal capillaries lined with normal-appearing flat endothelial cells without any evidence of mitotic activity. (Kono et al., 2006) Port-wine stain can occur anywhere on the body but mostly involves the head and neck region, typically following the course of trigeminal nerve on the face. The exact etiology and origin of port-wine stain remain unknown, the most likely being the deficiency of surrounding neurons that regulate blood flow through the venules. Hence, the blood vessels are unable to constrict and remain dilated. It is believed that port-wine stains develop within the first 2 to 8 weeks of gestation. (Schneider et al., 1988) They usually present at birth as sharply demarcated pink or, red macules or, patches. They appear to fade during the first 12 months of life. Later on, the capillaries become more ectatic and the color gradually deepens with age. They become dark-red during adolescence and turn violaceous with advancing age. The lesions are macular initially but the surface becomes irregular and thickened over time. In the past, therapeutic modalities for port-wine stain treatment included excision with skin grafting, cryotherapy, ionizing radiation, dermabrasion, and electrotherapy and/or, tattooing. All of these often left cosmetically unacceptable scarring. The introduction of argon laser was a major advancement in the treatment of port-wine stain. The argon laser produces blue-green light that is absorbed by hemoglobin within the blood vessels. The radiant energy is, then, converted into heat leading to destruction of the blood vessels. (Ortiz and Nelson, 2012)


Case Report

A male patient aged 58 years reported with a chief complaint of swelling in the upper right back tooth region since 6 days. Patient had a unilateral enlargement of middle third of face and upper lip on right side with erythema over the skin of the upper and middle thirds. (Figure 1) Intra-orally, patient had enlarged and edematous gingival tissues in upper right back tooth region. Patient gave a history of repeated swelling in that region since 25 years, from when, he had episodes of exacerbations occasionally. The swelling was characterized by mild, continuous, localized pain, of sudden onset, during the phases of exacerbation. Patient, also, gave history of mobility in relation to the one of the teeth in that region which became mobile and got exfoliated 2 months back. Now, patient had mild continuous localized pain in upper right gingiva since 6 days. Patient used to take medication occasionally for the swelling from past 2 to 3 years. On a careful examination, a single, diffuse, oval swelling, of size around 8x5cms, was present in the middle and lower third of face on right side, extending from the level of supra-orbital region superiorly to inferior border of mandible inferiorly. Medially, the swelling extended from the ala of the nose to 3cms in front of tragus of ear distally. There was enlargement of upper lip on right side from philtrum to commissure. There was obliteration of inferior palpebral depression and nasolabial fold with slight drooping of the corner of the mouth on right side which was evident. Slight edema was present surrounding the orbit. Skin over the face was erythematous on right side extending from the midline of forehead till the middle third of the face. (Figure 2) There was slight rise of temperature over the surface of the skin on the face. Swelling was soft and compressible. Skin over the swelling was pinchable. On intra-oral examination, a single, diffuse, dome-shaped swelling, of size 7x1cms, was present on the buccal aspect of gingiva involving marginal, interdental and attached gingival tissues and alveolar mucosa from the mesial aspect of tooth # 12 to the distal aspect of tooth # 17 mediolaterally. Supero-inferiorly, the swelling extended from the marginal gingiva of teeth # 13, 14, 15 and 16 to the depth of the alveolus. There was vestibular obliteration from teeth # 13 till tooth # 17 mediolaterally. Overlying mucosa was erythematous and smooth. (Figure 3) A single, diffuse, dome-shaped swelling, of size 5x1cms, was present on the palatal aspect, too, involving the marginal, interdental and attached gingival tissues and palatal mucosa from the mesial aspect of tooth # 15 to 1cm distal to the distal aspect of tooth # 18. Posterior to tooth # 18, there was erythema over an area of around 1cm from midline of the palate till the distal aspect of tooth # 18 region. Overlying surface was smooth and erythematous. Two growths of size around 2x2cms were, also, present on the mesial aspect of tooth # 16 palatally extending from the marginal gingival and interdental papilla of teeth # 15 and 16 till the middle thirds of the crowns. Another growth was present on distal aspect of tooth # 16 palatally from the edentulous ridge and filled the edentulous region. Overlying surface was irregular, slightly erythematous and growth was sessile and could be demarcated from the adjacent mucosa by a crease. (Figure 4) There was bulkiness of entire buccal fat pad of around 4x3cms area. Erythema of right half of hard palate and right buccal vestibule was, also, evident. Numerous infoldings of right buccal mucosa suggestive of marble stone appearance was noted. On palpation, the swellings on buccal and palatal aspects were slightly tender, soft and compressible. The growths on palatal aspect of tooth # 16 were firm, non-tender and slightly compressible. An orthopantomograph (OPG) was advised which revealed a generalized horizontal bone loss in the affected region. An ill-defined radiolucency involving enamel, dentin and pulp was seen irt tooth # 27 and there was complete loss of tooth structure irt tooth # 17. (Figure 5) Considering the chief complaint, history of present illness and observed clinical features, a provisional diagnosis of nevus flammeus of the face on right side was arrived-at. The patient was managed for the oro-dental complications.



Figure 1 A single, diffuse, oval swelling present in the middle and lower third of right side of the face.



Figure 2 Enlargement of upper lip on right side from philtrum to commissure region. 



Figure 3 A single, diffuse swelling present on the buccal aspect of gingiva involving marginal, interdental and attached gingival tissues and alveolar mucosa from the mesial aspect of tooth # 12 to the distal aspect of tooth # 17.



Figure 4 A single, diffuse present on the palatal aspect involving the marginal, interdental and attached gingival tissues and palatal mucosa from the mesial aspect of tooth # 15 to 1cm distal to the distal aspect of tooth # 18.



Figure 5 Orthopantomograph (OPG) revealing a generalized horizontal bone loss in the affected region with an ill-defined radiolucency involving enamel, dentin and pulp irt tooth # 27 and complete loss of tooth structure irt tooth # 17.



Port-wine stains or, Nevus flammeus, represent hamartomatous vascular malformations and are named due to the deep reddish hue they impart on the skin or, mucosa in the affected areas. (Fishman and Mulliken, 1993) Sturge-Weber syndrome is one such syndrome characterized by port-wine stains. It was first described by Schirmer and later, more specifically, by Sturge in 1879 and is, also, known as Sturge-Weber disease, Encephalo-trigeminal angiomatosis and meningo-facial angiomatosis. The classic feature of this disease is angioma of leptomeninges. The other common clinical features include epilepsy (80%), dermal angiomas resulting in port-wine stains (76%), and abnormal findings in skull radiographs (63%), mental retardation (54%), ocular involvement (37%) and hemiplegia (37%). Characteristically, angioma of the leptomeninges occurs as a unilateral lesion, overlying the posterior temporal, parietal and occipital areas. (Gorlin and Pindborg , 1964) Intra-orally, angiomatosis can involve lips causing macrochelia, hemihypertophy of buccal mucosa, palate and floor of the mouth. Gingival enlargement can vary from light vascular hyperplasia to extensive overgrowths. Pyogenic granulomas, unilateral hypertrophy of alveolus, ipsilateral, premature or, delayed eruption, malocclusion is other common abnormalities reported. This syndrome is of rare occurrence and management becomes complicated due to risk of hemorrhage. (Ahluwalia et al., 1998) There was no history of epileptic seizures in our case; hence, no further investigations were planned to rule-out the possibility of Sturge-Weber syndrome and/or, other related conditions. Laser therapy of port-wine stains using extended pulse pulsed-dye laser is accepted as the optimal approach because the thermal relaxation time for the vessels in port-wine stains is actually 1-10 msec. (Kono et al., 2006) The clinically used port-wine stain treatment modalities include pulsed dye lasers, alexandrite lasers, neodymium:yttrium-aluminum-garnet lasers and intense pulsed light. There are a number of promising experimental therapies for port-wine stain currently being investigated including photodynamic therapy (PDT), angiogenesis inhibitors, hemodynamic alterations in the vasculature and site-specific pharmaco-laser therapy (SSPLT). (Chen JK, et al, 2012; Lu YG, et al, 2010) In the present case, gingival overgrowth was managed by oral hygiene maintenance. But even despite stringent plaque control methods, in some cases, gingivectomy may be required. In such cases, use of Nd:Yag laser for surgery provides the advantage of immediate hemostasis, minimal damage to the surrounding tissues and no post-operative pain and secondary complications associated with conventional surgical procedures. To prevent re-occurrence, maintenance of good oral hygiene is very important even after gingivectomy (Caiazzo et al., 1998).



Management of patient with port wine stain and Sturge-Weber syndrome may be challenging due to the risk of haemorrhage. Extra care must be taken when performing surgical procedures in the affected areas as achieving homeostasis can be a significant problem.



Ahluwalia TP, Lata JKanwa P. Sturge-Weber Syndrome with intra-oral manifestations: A case report. Indian J. Dent Res 1998; 9:140-144.


CaiazzoA, Mehra PPapageorge MB. The use of pre-operative percutaneous transcatheter vascular occlusive therapy in the management of Sturge-Weber syndrome: Report of a case. J. Oral Maxillofac Surg 1998; 56:775-778.


Chen JK, Ghasri PAguilar Gvan Drooge AMWolkerstorfer A, Kelly KMet al.  An overview of clinical and experimental treatment modalities for port wine stains. J Am Acad Dermatol 2012; 67:289-304. PMid: 22305042 PMCid: PMC4143189


Fishman SJMulliken JB. Hemangiomas and vascular malformations of infancy and childhood. Pediatr Clin North Am 1993; 40:1177-1200.


Gorlin RJ, Pindborg JJ. Syndromes of head and neck. New York: McGraw-Hill: 1964;

PMCid: PMC289601


Kono TGroff WFSakurai HTakeuchi MYamaki TSoejima Ket al. Evaluation of fluence and pulse-duration on purpuric threshold using an extended pulse pulsed-dye laser in the treatment of port wine stains. J Dermatol 2006; 33:473-476. PMid: 16848819


Lu YGWu JJYang YDYang HZHe Y. Photodynamic therapy of port-wine stains. J Dermatolog Treat 2010; 21:240-244. PMid: 19722122


Ortiz AENelson JS. Port-wine stain laser treatments and novel approaches. Facial Plast Surg 2012; 28:611-620. PMid: 23188689


Schneider BVMitsuhashi YSchnyder UW. Ultra-structural observations in port wine stains. Arch Dermatol Res 1988; 280:338-345. PMid: 3190266


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