Tariq Rashid, Ijaz Hussain, Faridur Rehman, Tahir S Haroon.
Phakomatosis Pigmentovascularis Type IIb.
J Coll Physicians Surg Pak Jan ;10(7):265-6.

We report a case of pigmentovascularis type IIb in a 30-year-old female who had extensive port wine stain involving left side of face, head and neck, trunk and upper and lower limbs, nevus of Ota on right side of face. Other findings included varicose veins involving left lower limb, hemihypertrophy of left side of face, macrochelia of left side, pigmentation of both irides and prominent intracerebral vascular markings on left side and atrophy of left cerebral hemisphere.

CASE REPORT: A 30-year old female was referred to the Laser Unit of Dermatology Department of King Edward Medical College/Mayo Hospital, Lahore for the treatment of port-wine stain (PWS).She was born to non-consanguineous parents after a full-term gestation by normal delivery. Since her infancy she lead extensive port wine stain involving her left side of face, head and neck, trunk and upper and lower limbs. The lesion gradually grew larger proportionately with the advancement of age. At the age of two, she developed varicose veins in left lower limb and recurrent leg ulcers which were refractory to healing. There was also history of recurrent infections involving the left lower limb. By the age of fifteen she started to develop slate-grey coloured macules over right side of face and right conjunctiva. There had been no history of seizures, any motor or sensory deficit or ocular complaints. However, the occasionally had had episodes of epistaxis from left aide of nose.Physical examination revealed nevus of Ota on right side of face and partially blanchable red purple patches over left side of face, head and neck, trunk, left upper limb involving the palm, and left lower limb. Few patches were also present over right pectoral and deltoid region. The patches over face had a few nodular lesions, as well. There was hemihypertrophy of left side of face and macrochelia involving the left half of upper lip. Examination of oral cavity showed similar erythematous patches over left buccal mucosa and left half of hard palate. Left lower limb had varicose veins affecting both short saphenous and long saphenous systems. An ulcer of 3x3 cm, with bluish, irregular margins and granulation tissue base but without any area of induration was present over left medial malleolus. Lipodermatosclerosis and scars of atrophie blanche were seen. However, there was no difference in the size of two halves of trunk, both upper or lower limbs. Slit-lamp examination of eyes showed episcleral and conjunctival pigmentation on both sides; corneal diameter of 12.5 and 12.7mm on right and left sides, respectively; heavy blue-grey pigmentation of iris on right side; normal angle on both sides; intraocular pressure of 10 and llmm in right and left eye respectively. Choroidal vessels on left side were congested but there was no hemangioma or nevus. Discs and extraocular movements were normal on both sides. Visual acuity, was 6/6 and 6/12 in the right and left eye respectively. ENT and neurological examination was normal-on both sides. Investigations revealed a normal X-ray skull. CT-scan of brain, MRI of brain and spinal cord showed prominent, intracerebral vascular malformation on left side and atrophy of left cerebral` hemisphere. Doppler flow studies of limbs revealed increased blood flow in the left upper and lower limbs. Abdominal ultrasound showed hypoplasia of left kidney.

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