A seemingly insignificant lesion.

A 60-year-old man presented to the clinic with a large nodular and vascular disfiguring skin lesion on the face [Figure 1]. He recalled the lesion as irregularly shaped violaceous patches on his face since early childhood. The lesion was present since infancy, but he has never sought medical advice. With increasing age, papules and nodules developed leading to significant disfigurement and creating nuisance for everyday life. There was no history of ulceration or bleeding as he recalled. He had no history of weight loss, prolonged fever, or seizures. Papules and nodules were never painful and no similar lesion developed elsewhere in his body. Family history was also negative.

Figure 1

Port wine stain (nevus flammeus) on face

On physical examination, a very large skin lesion was noticeable primarily on the right side of his face extending beyond the midline to the left in perioral zone. The lesion was extended from right temporoparietal area on scalp to the lateral margin of lower lip on the left with some parts covering right side of the neck and going down to the chest. There was disfigurement of the face including right ear, right eye, right eyebrow, right cheek, right side of the nose, and the entire lower lip [Figure 2]. Very large firm and irregularly shaped nodules were apparent without tenderness mainly on his face between right ear and left side of his chin. A few smaller nodules (maximum 7 mm in diameter) were present on the affected skin parts in neck and chest. Skin of the affected area was mainly purple tinted without any discoloration. No sensory impairment was seen. Visual acuity and hearing were intact and oral cavity examination revealed no abnormality. There was no other cutaneous, neurologic, or skeletal abnormality.

Figure 2

Port wine stain (nevus flammeus) extensive distribution

A diagnosis of port wine stain was made on clinical grounds. Magnetic resonance imaging (MRI) of the brain and ophthalmologic examination revealed no vascular malformations and glaucoma, respectively, ruling out Sturge–Weber syndrome.

A port wine stain (PWS) or nevus flammeus is a low flow capillary malformation that can occur anywhere on the body. The lesions are typically unilateral, pink macular patches, which are present at birth. Unlike common capillary malformations, PWSs usually become nodular and red colored with age and persist for lifetime and sometimes causing cosmetic concerns. Altered neural modulation in papillary plexus is thought responsible for this lesion.[1] They occur in 0.3% of neonates often following the distribution of the fifth cranial nerve.[23]

PWSs are basically sporadic, nevertheless they may be associated with other vascular anomalies or congenital malformations.[3] Lesions appearing in trigeminal nerve distribution are characteristically related to anomalies of the face and leptomeninges, that is, Sturge–Weber syndrome and nearly 10% of these patients can have glaucoma.[2]

Diagnosis of capillary malformations is mostly clinical regarding appearance, time of onset, and chronological progress of the lesion. In children with facial PWS covering more than one segment of the fifth cranial nerve or previous neurologic symptoms, MRI of the brain should be requested considering Sturge–Weber syndrome.[4] Nevus simplex, hemangiomas, arteriovenous malformations, and linear scleroderma are the main differential diagnoses. Pulsed dye laser therapy is now recommended as the treatment of choice for PWS.[5]

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.