Phacomatosis cesioflammea with Klippel Trenaunay syndrome: A rare association
A 30-year-old male presented with extensive vascular changes on the trunk and limbs and pigmentary changes on the face. Cutaneous examination showed extensive nevus flammeus affecting almost the entire back, crossing the midline and extending to the buttocks and left lower limb [Figure and ]. Similar discrete lesions were present over the flanks and lateral aspect of the chest []. There was bilateral bluish-grey pigmentation over the face along the ophthalmic and maxillary division of the trigeminal nerve consistent with Nevus of Ota []. The sclera, nasal mucosa and palate also showed patchy bluish-grey pigmentation bilaterally [Figure and ]. Soft tissue hypertrophy of the left lower limb was seen in association with nevus flammeus and varicose veins over the left leg. A difference of 3 cm was noted in the circumference of both lower legs at the mid-calf region. The patient also had right-sided scoliosis. Neurological and ophthalmic examinations did not reveal any abnormality. Routine hematological investigations were normal. Radiological examination of the left lower limb showed soft tissue swelling without evidence of bony changes. Color Doppler of the affected limb showed varicosities of short saphenous vein with incompetence of the perforators. There was no history of seizures. Also, there was no history of similar pigmentary or vascular lesions in any of his family members.