The patient was a 25-year-old Japanese woman with an extensive nevus flammeus which extended primarily over the left side of her face and the right side of the trunk and extremities. She had been blind in the left eye since birth. A bluish macular discoloration was noted over both sides of the face (nevus of Ota) and the right shoulder (nevus of Ito). Muscle strength of the right extremities was remarkably diminished and the right extremities were longer than the left. Results of radiological examination of the skull showed a remarkable hypertrophy of the bones of the ipsilateral side of the nevus flammeus. The combination of Sturge-Weber and Klippel-Trénaunay syndromes indicates a nosological relationship.
Furukawa T, Igata A, Toyokura Y, Ikeda S. Sturge-Weber and Klippel-Trenaunay Syndrome With Nevus of Ota and Ito. Arch Dermatol. 1970;102(6):640-645. doi:10.1001/archderm.1970.04000120058010