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Indian Journal of Dermatology
  Publication of IADVL, WB
  Official organ of AADV
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CASE REPORT
Year : 2017  |  Volume : 62  |  Issue : 2  |  Page : 207-209

Phacomatosis cesioflammea with cutis marmorata-like lesions and unusual extracutaneous abnormalities: Is it a distinct disorder?


1 Nirvana Skin Clinic, Vadodara, Gujarat, India
2 KG Patel Children Hospital, Vadodara, Gujarat, India
3 Bhailal Amin General Hospital, Vadodara, Gujarat, India
4 Department of Dermatology, University Medical Center Freiburg, Freiburg, Germany

Correspondence Address:
Shyam B Verma
Nirvana Skin Clinic, Makarpura Road, Vadodara - 390 009, Gujarat
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-5154.201760

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A 2-month-old boy was presented with widespread lateralized blue macules (nevus cesius), an extensive nevus flammeus, and large patches of cutis marmorata telangiectatica congenita. Moreover, he had macrocephaly, a coarse facial appearance with depressed nasal bridge, retinal abnormalities, septal defects of the heart, and obliteration of the left brachiocephalic vein and major veins of the left arm with pronounced collateralization. The multisystem disorder of this boy cannot be categorized within the present classification of distinct types of phacomatosis pigmentovascularis. Although some similar complex cases have previously been reported, it seems too early to give them a specific name. Rather, the present case should be included, so far, into the group of unclassifiable types of phacomatosis pigmentovascularis.


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