Giant congenital melanocytic nevus with neurocutaneous melanosis

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Giant Congenital Melanocytic Nevi and Neurocutaneous Melanosis

Introduction. The major medical concern with giant congenital melanocytic nevi CMN is high risk of developing cutaneous melanoma, leptomeningeal melanoma, and neurocutaneous melanocytosis. Case Report. A 30-year-old woman with a giant congenital melanocytic nevus covering nearly the entire right thoracodorsal region and multiple disseminated melanocytic nevi presented with neurological symptoms...

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Giant congenital melanocytic nevus is usually defined as a melanocytic lesion present at birth that will reach a diameter ≥ 20 cm in adulthood. Its incidence is estimated in <1:20,000 newborns. Despite its rarity, this lesion is important because it may associate with severe complications such as malignant melanoma, affect the central nervous system (neurocutaneous melanosis), and have major ps...

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Neurocutaneous melanosis presented with giant congenital melanocytic nevi and primary intracranial melanoma

Neurocutaneous melanosis is a rare neurocutaneous syndrome defi ned by the presence of multiple and /or giant congenital cutaneous nevi and melanocytic deposits in the central nervous system with the infi ltration of leptomeninges. The major medical concern with giant congenital cutaneous nevi is high risk of developing cutaneous melanoma, leptomeningeal melanoma, and neurocutaneous melanosis. ...

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Figure 1. Two-year-old boy with a giant congenital melanocytic nevus of the left buttock. Left, Center, Preoperative views. Right, Immediate view after excision and placement of the Integra bilayer matrix dressing. Figure 2. Left, Wound after removal of the silicone layer of the Integra bilayer matrix dressing. Right, Coverage of the wound with a split skin autograft.

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Neurocutaneous melanosis (NCM) is a rare condition characterized by central nervous system melanocytic tumors associated with congenital melanocytic nevi. Phacomatosis pigmentovascularis (PPV) is an association of vascular nevus with pigmentary nevus. Aberrant maturation of neural crest-derived cells is considered to be related to pathogenesis in both conditions. However, association of NCM and...

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ژورنال

عنوان ژورنال: Our Dermatology Online

سال: 2019

ISSN: 2081-9390

DOI: 10.7241/ourd.20191.12