Are arteriovenous malformations a causative factor for hypertrophic and nodular port-wine stains?
Authors: Tianyou Wang, Xiaoxi Lin, Yunbo Jin, Xi Yang
Journal: Journal of the American Academy of Dermatology (2014)

To the Editor: Arteriovenous malformation (AVM), a vascular hamartomatous malformation, is rarely found on biopsy of hypertrophic and nodular port-wine stains (PWS).1Sanchez-Carpintero I. Mihm M.C. Mizeracki A. Waner M. North P.E. Epithelial and mesenchymal hamartomatous changes in a mature port-wine stain: morphologic evidence for a multiple germ layer field defect.J Am Acad Dermatol. 2004; 50: 608-612Abstract Full Text Full Text PDF PubMed Scopus (47) Google Scholar, 2Chen D. Hu X.J. Lin X.X. Ma G. Jin Y.B. Chen H. et al.Nodules arising within port-wine stains: a clinicopathologic study of 31 cases.Am J Dermatopathol. 2011; 33: 144-151Crossref PubMed Scopus (26) Google Scholar Digital subtraction angiography (DSA) is the gold standard method for diagnosis of AVM.3Liu A.S. Mulliken J.B. Zurakowski D. Fishman S.J. Greene A.K. Extracranial arteriovenous malformations: natural progression and recurrence after treatment.Plast Reconstr Surg. 2010; 125: 1185-1194Crossref PubMed Scopus (236) Google Scholar Lesions that show an early visualized draining vein during DSA is one of the main characteristics. We herein provide DSA evidence of limited AVM in mature PWS. A 22-year-old man visited our plastic and reconstructive surgery unit for treatment of his PWS birthmark (Fig 1). A PWS hyperplasia nodule in his temporal region had enlarged over the past 5 years. He had received no other treatment, except cryotherapy in his cervical region at age 1, and he had no previous trauma to the lesion or family history of vascular malformations. Magnetic resonance imaging and enhanced computed tomography showed abundant blood flow supplying the lesion and a well-defined soft tissue mass in the temporal region. DSA showed a tortuous feeding artery and an early visualized draining vein in arterial phase (Fig 2), suggesting the existence of AVM in the mass. A mass biopsy specimen was composed of large numbers of capillaries, and possible arterioles or postcapillary venules. Another patient, a 34-year-old man with a PWS birthmark on his left mid-facial region, was examined on our unit. A PWS hyperplasia nodule on his upper eyelid had enlarged slowly over the past 10 years. He had no previous treatment. His case history and the manifestation profile of his PWS lesion were similar to that of the previous patient. DSA showed that the feeding artery of the hyperplasia lesion was the ophthalmic artery, and the draining vein was visualized early, in 1.9 seconds. Draining veins of normal organs are usually seen about 3 seconds after the arterial phase. AVM is a progressive vascular malformation, and the expansion of an AVM lesion progresses inevitably according to its natural course. DSA in these 2 cases proved the existence of limited abnormal arteriovenous communications. Micro-AVM may be the cause of the enlargement in these two hypertrophic PWS nodules. Moreover, we suspect the existence of unknown micro-AVM or AVM in some cases of hypertrophic and nodular PWS. The diagnoses in these two cases are not analogous to “capillary malformation−arteriovenous malformation” (CM-AVM). Differentiating characteristics of CM-AVM include4Eerola I. Boon L. Mulliken J. Burrows P. Dompmartin A. Watanabe S. et al.Capillary malformation-arteriovenous malformation, a new clinical and genetic disorder caused by RASA1 mutations.Am J Hum Genet. 2003; 73: 1240-1249Abstract Full Text Full Text PDF PubMed Scopus (550) Google Scholar: (1) Autosomal dominance (RASA1 mutation); (2) manifestation as Sturge-Weber syndrome, Klippel-Trenaunay syndrome, or Parkes Weber syndrome; (3) pale pink CM lesions accompanied by high skin temperature; (4) diffuse AVM lesions under CM lesions.