NEVUS SEBACEOUS, SYSTEMATIZED
By Gary M. White, MD
The systematized nevus sebaceous (SNS) is a very large nevus sebaceous that may be associated with various systemic abnormalities. It often follows Blaschko's lines.
- SNS appears to be stem from a post-zygotic mosaic mutation in RAS genes.
- Rarely, the patient may develop hypophosphatemic Vitamin D-resistant rickets as manifested by bone abnormalities, muscle weakness and bone pain.
- Phacomatosis Pigmentokeratotica is a distinctive new epidermal nevus syndrome characterized by the presence of nevus sebaceous arranged along Blaschko lines, a speckled lentiginous nevus, and skeletal and neurologic abnormalities.
The patient should be evaluated for abnormalities of the CNS, skeletal system, eyes. Appropriate studies beyond a good HP include skeletal survey, head CT or MRI, u.a., liver and renal studies, and calcium and phosphate.
The patient should be referred to a plastic surgeon for consideration of cosmetic repair. For any portions that cannot be excised, long term observation for development of BCCs and rarely other neoplasms is indicated.
JAAD July 2010 Volume 63, Issue 1, Pages 25–30
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