Autosomal Dominant Inheritance

Bilateral asymmetrical areas of hypopigmented whorls ("marbling"); central nervous system, eye, tooth, skin, nail, and bone anomalies

Mukiple angiomas of skin and mucous membranes that are dilations of capillaries and venules; bleeding from any site: nose, gastrointestinal, pulmonary, and urinary systems

Frontal patch of white hair, heterochromia iridis, lateral displacement of inner canthus, and at times, cochlear deafness

Clinical findings are similar to type I without the lateral displacement of inner canthus; deafness is more common

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