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This autosomal dominant disorder affects skin [multiple BCCs and palmoplantar pits] and has a variable expression of abnormalities in a number of systems, including skeletal malformations (mandibular "keratocysts"), soft tissue, eyes, CNS, and endocrine organs.


Causes:

Nervous system involvement may include hydrocephalus (an enlargement of the head caused by fluid accumulation on the brain), seizures, mental retardation, deafness, and brain tumors (medulloblastoma). Defects in the iris or lens of the eye, and blindness are manifestations that can affect the eyes.

Defects in the bones include cysts in the upper jaw (maxilla) and lower jaw (mandible) that may cause abnormal tooth development or spontaneous jaw fractures. Other bony defects may be associated with this condition, such as scoliosis (curvature of the back), kyphosis (severe curvature of the back), and rib abnormalities.


Symptoms:

* Characteristic facial appearance including wide-set eyes, broad nasal bridge, heavy ridge over eyes, and protruding jaw
* Defects in iris of the eye
* Opaque lens of eye (cataracts)
* Blindness
* Abnormal teeth with pits in the enamel
* Skeletal abnormalities

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