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Синдром NEVADA

ORPHA:370059· ICD-10 Q84.8· NEVADA syndrome

Визначення(English summary)

A rare, life-threatening, cutaneous disease characterized by a keratinocytic epidermal nevus presenting thick, hystrix-like, white or brownish hyperkeratosis associated with multiple extracutaneous vascular malformations, including angiodysplasia that involves large-vessel arteriovenous shunts that may be fatal during the neonatal period.