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Cafe au lait spots, those large pigmented birthmarks (as opposed to liver marks caused by sun and age), are terribly quirky.
Congenital abnormalities include: skeletal anomalies (especially those affecting the hands), cafe au lait spots and hypopigmentation.
Legius syndrome (LS) is an autosomal dominant condition characterized by cafe au lait spots.
It is characterized by dark patches on the skin called cafe au lait spots and tumors known as neurofibromas, which grow under the skin and along nerves and nerve fibers.
Neurofibromatosis can be extremely difficult to diagnose at its early stages, when patients may exhibit symptoms no more severe than the appearance of dark patches on the skin, known as cafe au lait spots.
On the other hand, seven or more cafe au lait spots (brownish splotches on the skin) at birth suggest von Recklinghausen's disease, a k a neurofibromatosis, a k a Elephant Man's disease.