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.