Phakomatosis pigmentovascularis type II combined with Sturge-Weber syndrome: a case report
Keywords:
phakomatosis pigmentovascularis, Sturge-Weber syndromeAbstract
Phakomatosis pigmentovascularis (PPV) is defined as the coexistence of a vascular (usually capillary) nevus and an extensive pigmentary nevus associated to a variety of other cutaneous nevus and/or extracutaneous alterations. Genetic concept of twin spots is the proposed mechanism of the disease. Due to various clinical manifestations, many classifications were proposed. Combined Sturge-Weber syndrome (SWS) and Klippel-Trénaunay syndrome (KTS) with PPV had also been reported. SWS and KTS may be associated with systemic forms of PPV. There is no specific curative treatment. Recognition of possible underlying systemic, local anomalies and complications including cosmetic and psychosocial concern dictates the management.
We report a case of PPV type II combined with SWS in a 19 year-old Thai man.
References
Ruiz-Maldonado R, Durเn-McKinster C, Orozco-Covarrubias L, et al. Phakomatosis Pigmentovascularia. In: Ruggieri M, Castroviejo I P, Rocco C D, at al. 2008 edition Neurocutaneous Disorders: Phakomatoses & Hamartoneoplastic Syndromes : Springer; 2008:449-454.
Vidaurri H, Tamayo l, Duran C, et al. Phakomatosis Pigmentovascularis II and II B: Clinical findings in 24 patients. J Dermatol. 2003; 30: 381–388.
Moutray T, Napier M, Shafiq A, et al. Monozygotic Twins Discordant for Phacomatosis Pigmentovascularis: Evidence for the Concept of Twin Spotting. Am J Med Genet A. 2010;152A:718-20.
Happle R. Phacomatosis pigmentovascularis revisited and reclassified. Arch Dermatol. 2005;141: 385–388.
Odile Enjolros. Vascular Malformations. In: Bolognia J A, Jorizzo J L, Schaffer J L, editors. 3rd ed. Bolognia’s Dermatology: Saunders, 2012:1711-1728.
Lee A, Driscoll D, Gloviczki P, et al. Evaluation and Management of Pain in Patients with Klippel-Trenaunay Syndrome: A Review. Pediatr Dermatol. 2005;115:744-9.
Uysal G, Güven A, Ozhan B Phakomatosis pigmentovascularis with Sturge-Weber syndrome: a case report. J Dermatol. 2000;27:467-70.
Robaee A A, Banka N, Alfadley A. Phakomatosis pigmentovascularis type IIb associated with Sturge-Weber syndrome. Pediatr Dermatol. 2004;21:642-5.
Saricaog LM, Gu V D, Karakurt A. et al. An unusual case of Sturge-Weber syndrome in association with phakomatosis pigmentovascularis and Klippel-Trenaunay- Weber syndrome. Retina. 2002;22:368–371.
Lo P Y, Tzung T Y. Phakomatosis pigmentovascularis type IIb with a patent umbilical vein and inferior vena cava hypoplasia. Br J Dermatol. 2003;148:836–838.
Lee CW, Choi DY, Oh YG et al. An infantile case of Sturge-Weber syndrome in association with Kilppel-Trenaunay-Weber syndrome and phakomatosis pigmentovascularis. J Korean Med Sci. 2005;20:1082–1084.
Diociaiuti A, Guidi B, Aguilar Sanchez J A et al. Phakomatosis pigmentovascularis type IIIb: a case associated with Sturge-Weber and Klippel-Trenaunay syndromes. J Am Acad Dermatol.2005;53:535–538.
Greve B, Raulin C. Prospective study of port wine stain treatment with dye laser: Comparison of two wavelengths (585 nm vs. 595 nm) and two pulse durations (0.5 milliseconds vs. 20 milliseconds). Lasers in Surgery and Medicine. 2004;34:168–173.
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เนื้อหาและข้อมูลในบทความที่ลงตีพิมพ์ในวารสารโรคผิวหนัง ถือเป็นข้อคิดเห็นและความรับผิดชอบของผู้เขียนบทความโดยตรงซึ่งกองบรรณาธิการวารสาร ไม่จำเป็นต้องเห็นด้วย หรือร่วมรับผิดชอบใดๆ
บทความ ข้อมูล เนื้อหา รูปภาพ ฯลฯ ที่ได้รับการตีพิมพ์ในวารสารโรคผิวหนัง ถือเป็นลิขสิทธิ์ของวารสารฯ หากบุคคลหรือหน่วยงานใดต้องการนำทั้งหมดหรือส่วนหนึ่งส่วนใดไปเผยแพร่ต่อหรือเพื่อกระทำการใดๆ จะต้องได้รับอนุญาตเป็นลายลักอักษรจากบรรณาธิการวารสารโรคผิวหนังก่อนเท่านั้น
