Phakomatosis pigmentovascularis in Child
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Synopsis

- Nevus flammeus plus epidermal nevus
- Nevus flammeus plus dermal melanocytosis with or without nevus anemicus
- Nevus flammeus plus nevus spilus with or without nevus anemicus
- Nevus flammeus plus dermal melanocytosis plus nevus spilus with or without nevus anemicus
- Cutis marmorata telangiectatica congenita plus dermal melanocytosis
The pathophysiology of PPV is poorly understood. One theory suggests abnormalities in neuroectodermal precursor cells that later differentiate into melanocytes and vasomotor nerve cells. Genes responsible for these abnormalities have not yet been identified.
Codes
ICD10CM:Q85.8 – Other phakomatoses, not elsewhere classified
SNOMEDCT:
78572006 – Neurocutaneous syndrome
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Last Reviewed:06/07/2017
Last Updated:07/14/2019
Last Updated:07/14/2019