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Phakomatosis Pigmentovascularis Type II with Bilateral Nevus of Ota – A Rare Entity
*Corresponding author: Kumari Ritu, Department of Dermatology, All India Institute of Medical Sciences, Deoghar, Jharkhand, India. ritu64026@gmail.com
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Received: ,
Accepted: ,
How to cite this article: Ritu K. Phakomatosis Pigmentovascularis Type II with Bilateral Nevus of Ota – A Rare Entity. Indian J Postgrad Dermatol. 2025;3:199-200. doi: 10.25259/IJPGD_1_2025
Phakomatosis pigmentovascularis (PPV) is a mosaic abnormality of vasomotor nerves and melanocytes resulting in its characteristic cutaneous manifestations. A 34-year-old male presented with extensive port wine stain affecting the right half of body since childhood with associated hypertrophy, varicosity and recurrent ulceration of the lower half of affected right lower limb. Bluish scleral discolouration of both eyes with grey blue discolouration of upper eyelids (left > right) was suggestive of Nevus of Ota (NOA) [Figure 1]. Venous Doppler revealed dilated and tortuous veins incompetent BOYD’s and COCKETT’s perforator. The patient was diagnosed as case of PPV II with bilateral NOA.
- (a) Bluish discolouration of sclera of both eyes. (b) Blue grey discolouration of both upper eyelids (left > right) with port wine stain over the right half of face. (c) Hypertrophy of the right lower limb with varicosity and ulceration over the lower half of the right lower limb. (d) Extensive port wine stain affecting the right half of body.
Acknowledgment:
Dr. Ghazal Ahmed: As Head of Department for mentorship and insightful feedback on the manuscript. Dr. Avinash Kumar and Dr. Amit Kumar: For valuable assistance.
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Institutional Review Board approval is not required.
Declaration patient consent:
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Conflicts of interest:
There are no conflicts of interest.
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