Abstract
IntroductionPhacomatosis pigmentovascularis is a rare congenital condition characterized by vascular malformation associated with extensive pigmented nevi. Even though it forms a large, prominent skin lesion, therapy for phacomatosis pigmentovascularis is rarely discussed. To the best of our knowledge, this is the first report of phacomatosis pigmentovascularis type II treated with combined laser therapy using Q-switched alexandrite and long-pulsed dye lasers.Case presentationsIn the first of two cases reported here, a 2-week-old Japanese baby girl was given a diagnosis of phacomatosis pigmentovascularis type II and Klippel–Trénaunay syndrome because of port-wine stains, cutis marmorata telangiectatica congenita, and aberrant Mongolian spots over her trunk and limbs. After five laser therapy sessions under general anesthesia, her aberrant Mongolian spots and port-wine stains have improved. But interestingly, the cutis marmorata telangiectatica congenita on the patient's back has improved without laser therapy.In the second case, a 4-month-old Japanese baby boy was referred to us because of port-wine stains, cutis marmorata telangiectatica congenita, and aberrant Mongolian spots over his face, trunk and limbs. Phacomatosis pigmentovascularis type II was diagnosed and laser therapy was started. After three laser therapy sessions under general anesthesia, the aberrant Mongolian spots and port-wine stains have improved. The cutis marmorata telangiectatica congenita on the baby's back, buttocks, and arms has faded somewhat without laser therapy.ConclusionsCombined laser therapy improved the phacomatosis pigmentovascularis skin lesions, but was not effective for the cutis marmorata telangiectatica congenita with hemiatrophy. Cutis marmorata telangiectatica congenita without atrophy can be expected to improve on its own. Our results will assist physicians considering how best to treat patients with phacomatosis pigmentovascularis.
Highlights
Phacomatosis pigmentovascularis is a rare congenital condition characterized by vascular malformation associated with extensive pigmented nevi
We here present two patients with Phacomatosis pigmentovascularis (PPV) type II who were treated with combined Q-switched alexandrite laser (QAL) and long-pulsed dye laser (LPDL) therapy
Case 1 was classified as PPV IIb because of the presence of nevus flammeus, aberrant Mongolian spots, and Klippel–Trénaunay syndrome
Summary
We have reported two cases of PPV type II treated with combined laser therapy. The combined laser therapy improved the PPV skin lesions, the PWS, and aberrant Mongolian spots, but was not effective for CMTC with hemiatrophy. To the best of our knowledge, this is the first report of PPV type II treated with combined QAL and LPDL laser therapy. Our results will be of some help to physicians in deciding how best to treat patients with PPV. Consent Informed written consent was obtained from the parents of both patients to publish this case report and the accompanying images; copies of the written consents are available for review by the Editor-in-Chief of this journal. Authors’ contributions KA performed the diagnosis and laser therapy, reviewed the literature, and wrote the manuscript. All authors read and approved the final manuscript
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