Phacomatosis pigmentovascularis is a rare dermal disorder attributed to the presence of various nevi. These lesions exist since birth, so the patient remains well aware of them. Various systemic involvements may be associated with the nevus, but the association of Raynaud's phenomenon is seldom reported. Our patient came with similar features and, on workup, no neurovascular compression was present, such as cervical rib or thoracic outlet syndrome. Therefore, he was managed conservatively and experienced improvement following the treatment. The objective of reporting this case is to highlight the association of Raynaud's phenomenon with such nervous lesions.
Keywords: capillary nevus; developmental malformation; limb hypertrophy; phacomatosis pigmentovascularis; raynaud's phenomena.
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