Presence of portwine stains, gingival overgrowth and convulsive disorders

 # Which of the following is characterized by the presence of portwine stains, gingival overgrowth and convulsive disorders?

A. Sturge Weber syndrome

B. Witkop syndrome

C. Hunter syndrome

D. Fanconi syndrome

The correct answer is A. Sturge Weber Syndrome.

Encephalotrigeminal Hemangiomatosis

(Sturge-Weber syndrome)

The facial cutaneous capillary venous angiomas (or port-wine nevi) are usually the first component

of the syndrome to be observed, at birth, and are confined almost exclusively to the skin area supplied by the trigeminal nerve. Neurologic manifestations are among the most characteristic features of the disease and consist of convulsive disorders and spastic hemiplegia with or without mental retardation. Occasionally, angiomatous lesions also involve the gingiva and buccal mucosa.

Reference: Shafer’s Textbook of ORAL PATHOLOGY, 7th Edition, Page No: 149

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