Phakomatosis pigmentovascularis type IIb

Phakomatosis pigmentovascularis (PPV) is a rare congenital disorder characterized by extensive cutaneous, vascular and melanocytic lesions [1]. We present a unique case of type IIb PPV in association with Klippel-Trenaunay syndrome, scleral melanosis, and rare extensive dermal melanocytosis, which manifested as bilateral nevus of Ota, bilateral nevus of Ito and ectopic Mongolian spots. A 66-year-old female Chinese patient was referred to our dermatology department for a chronic ulcer on the right lower limb that had been present for five years. In addition, several diffuse, brown and bluish-gray pigmented lesions on her face, shoulder, and back as well as diffuse, red-purple aggregated patches on her trunk and limbs had been present since birth. Her parents were both Chinese and nonconsanguineous. There was no familial history of PPV or other inherited diseases. Physical examination revealed brown, bluish-gray, and bluish-black spots as well as patches on her forehead, cheeks, and skin around both eyes. The sclera, auricle, nasal mucosa, and palatal mucosa had bluish-gray pigmented lesions bilaterally. These pigmented lesions were diagnosed clinically as bilateral nevus of Ota (Figure 1a–e). Light brown and bluish-gray pigmented lesions on her shoulders were diagnosed clinically as bilateral nevus of Ito (Figure 1f). Bluish-gray pigmented lesions scattered on her back were diagnosed clinically as ectopic Mongolian spots (Figure 1f). Diffuse, red-purple aggregated patches were observed mainly on her right trunk and limbs (Figure 1f–i). Moreover, ipsilateral hemihypertrophy was seen clearly on her right upper and lower limbs. Interestingly, subcutaneous varicosity was also noted on her right leg and abdominal wall (Figure 1f–i). A 6 cm x 4 cm chronic Clinical Letter