Clinical characteristics and treatment of 52 cases of phakomatosis pigmentovascularis

The Journal of Dermatology
Hyoseung ShinHyunsun Park

Abstract

Phakomatosis pigmentovascularis is a rare syndrome characterized by widespread capillary malformation and pigmented nevus. The objective of this study was to evaluate its characteristics and treatment. Fifty-two patients presenting between 2003 and 2017 were retrospectively reviewed. Type IIa (port-wine stain and dermal melanocytosis with oculocutaneous involvement) was most common. Systemic involvement was observed in 17.3% and it was not significantly correlated to extent of capillary malformation or pigmented nevus. However, systemic involvement was significantly frequent in patients with nevus of Ota and in patients with pigmented nevus located on the head and neck (P = 0.004 and 0.035, respectively). Capillary malformation was almost cleared in 28.6% of patients using pulsed dye laser, whereas pigmented nevus was almost cleared in 23.7% and completely cleared in 42.1% of patients using Q-switched neodymium:yttrium-aluminum-garnet laser. Treatment outcome score showed significant inverse correlation with the extent of port-wine stain or pigmented nevus (P = 0.047, ρ = -0.308 and P = 0.011, ρ = -0.410, respectively). Pigmented nevus demonstrated better treatment response to lasers than did capillary malformation. Smaller les...Continue Reading

References

May 1, 1985·Archives of Dermatology·Y Hasegawa, M Yasuhara
Mar 24, 2000·The British Journal of Dermatology·I Ono, T Tateshita
May 30, 2003·The Journal of Dermatology·Helena Vidaurri-de la CruzRamón Ruiz-Maldonado
Jun 6, 2003·Dermatologic Surgery : Official Publication for American Society for Dermatologic Surgery [et Al.]·Taro KonoMotohiro Nozaki
Jun 10, 2004·The Journal of Dermatology·Thiam Seng Colin, Prasad W Kumarasinghe Sujith
Mar 23, 2005·Archives of Dermatology·Rudolf Happle
Nov 30, 2007·Journal of the American Academy of Dermatology·Montse Fernández-GuarinoPedro Jaén Olasolo
Dec 17, 2008·Nature·Catherine D Van RaamsdonkBoris C Bastian
May 22, 2009·Twin Research and Human Genetics : the Official Journal of the International Society for Twin Studies·Meike BartelsDorret I Boomsma
Feb 19, 2013·Ophthalmic Surgery, Lasers & Imaging Retina·Christopher R HenryAudina M Berrocal
May 10, 2013·The New England Journal of Medicine·Matthew D ShirleyJonathan Pevsner

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