The case of a boy with nevus of Ota, extensive Mongolian spot, nevus flammeus, nevus anemicus and cutis marmorata telangiectatica congenita: a unique instance of phacomatosis pigmentovascularis

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The case of a boy with nevus of Ota, extensive Mongolian spot, nevus flammeus, nevus anemicus and cutis marmorata telangiectatica congenita: a unique instance of phacomatosis pigmentovascularis*

Phacomatosis pigmentovascularis is a rare, congenital condition characterized by a combination of cutaneous melanocytic lesions and vascular malformation. We discuss an entirely unique case of Phacomatosis pigmentovascularis with nevus of Ota, extensive Mongolian spot, nevus flammeus, nevus anemicus and cutis marmorata telangiectatica congenita, which may represent a heretofore undescribed vari...

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Cutis marmorata telangiectatica congenita and aberrant Mongolian spots: Type V phacomatosis pigmentovascularis or phacomatosis cesiomarmorata

CMTC: Cutis marmorata telangiectatica congenita PPV: Phacomatosis pigmentovascularis INTRODUCTION The coexistence of cutis marmorata telangiectatica congenita (CMTC) with Mongolian spots has been reported as a distinct type of phacomatosis pigmentovascularis (PPV), namely PPV type V or phacomatosis cesiomarmorata. PPV type V is a rare congenital vascular anomaly, with only 7 previous cases ment...

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Bilateral congenital nevus of Ota in association with Mongolian spot

A 24-year-old woman presented with asymptomatic hyperpigmented bilateral patches on her temples, eyelids and forehead since birth. Furthermore, the patient had a congenital grey patch, compatible with Mongolian spot, on her buttock. She had no vascular or other cutaneous lesion. Histopathologic examination revealed bipolar dendritic melanocytes dispersed in a ribbon-like pattern between the col...

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Laser therapy treatment of phacomatosis pigmentovascularis type II: two case reports

INTRODUCTION Phacomatosis pigmentovascularis is a rare congenital condition characterized by vascular malformation associated with extensive pigmented nevi. Even though it forms a large, prominent skin lesion, therapy for phacomatosis pigmentovascularis is rarely discussed. To the best of our knowledge, this is the first report of phacomatosis pigmentovascularis type II treated with combined la...

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Cutis marmorata telangiectatica congenita: a case report.

Cutis marmorata telangiectatica congenita (CMTC) is a rare cutaneous vascular disorder that was first described in 1922 by Van Lohuizen (1). The clinical presentation is one of persistent cutis marmorata, phlebectasia, telangiectasia, and areas of ulcerations. The disorder shows slow clinical improvement over time. Previously, CMTC has also been described as congenital generalized phlebectasia ...

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ژورنال

عنوان ژورنال: Anais Brasileiros de Dermatologia

سال: 2015

ISSN: 0365-0596

DOI: 10.1590/abd1806-4841.20153466