Raynaud's Phenomenon and Limb Hypertrophy Presenting Phacomatosis Pigmentovascularis: A Rare Association

Abstract

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.

Figure 1. Extensive nevus located over the pectoral region, upper abdominal skin, almost covering the whole upper limb along with hypertrophy of biceps and rest of the left limb (A) and left side of the back extending to the upper trunk (B)

Figure 2. Red and pinkish lesions resembling the Port-wine stain on the left side of the posterior chest wall denoted by a black rectangle (A), and thick coarse skin and white pale nails denoted by red and green arrows, respectively (B)

Figure 3. Blanching after applying pressure which is taking a long duration to turn pink; denoting Raynaud’s phenomenon