This is a group of genetic disorders, also
known as the phakomatoses,which
produces a variety of developmental
abnormalities that frequently involve
skin, along with an increased risk of
nervous system tumors. These disorders
are inherited as autosomal dominant
Ash leaf spots.
Subungual and gingival fibromas.
Fibrous plaques on the forehead.
ADENOMA SEBACEUM:MULTIPLE ANGIFIBROMAS
OF THE FACE .ERYTHEMATOUS PAPULES ON THE
LOWER HALF OF FACE-CHEEKS,NASOLABIAL
FOLDS,SIDES OF THE NOSE AND CHIN.
ASH LEAF SPOTS. Hypopigmented macules. The average
size is 1 to 3 cm, and the common shapes are oval,
polygonal, and lance-ovate, whereas the less common
shapes are dermatomal and confettilike.
Ash leaf spots. "confetti" macules. Multiple,
discrete, small, confetti-like, hypopigmented
macules of variable size on the leg.
Detection of ash leaf spots may require Wood's lamp
examination(UVA), especially in fair-skinned
individuals. An ash leaf spot on the scalp will result in
poliosis, which is a circumscribed patch of gray-white