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Neurocutaneous Disorders

Tuberous Sclerosis
»	Tuberous sclerosis is also known as epiloia. What does this term mean?
»	What is the inheritance of tuberous sclerosis?
»	Where are the genetic defects for tuberous sclerosis?
»	What is the earliest skin sign of tuberous sclerosis?
»	Adenoma sebaceum is a misnomer. What is the correct term for the facial lesions seen in tuberous sclerosis?
»	What are Koenen’s tumors?
»	What is a shagreen patch?
»	What are tubers, and where do they occur?
»	What signs of tuberous sclerosis may be revealed on funduscopic examination?
»	Which cardiac abnormality is characteristic of tuberous sclerosis?
»	Can renal involvement occur in tuberous sclerosis?
»	What is the prognosis for patients with tuberous sclerosis?

What are Koenen’s tumors?

Fig. 5.6 Koenen’s tumor (periungual fibroma).

Subungual and periungual fibromas (Fig. 5-6). These develop at or after puberty and present as firm, flesh-colored growths of 5 to 10 mm in length, projecting from the nail folds and beneath the nail plate.