Eloise R Galligan1, Eulalia Baselga2, Ilona J Frieden3, Nicole W Kittler3, Christine T Lauren4, Kimberly D Morel4, Catherine McCuaig5, Elena Pope6, Megha Tollefson7, Kimberly Tantuco6, Orli Wargon8, Maria C Garzon9. 1. Columbia University Vagelos College of Physicians & Surgeons, New York, New York; Department of Internal Medicine, Greenwich Hospital, Greenwich, Connecticut. 2. Department of Dermatology, Hospital de Sant Joan de Deu Barcelona, Barcelona, Spain. 3. Department of Dermatology, University of California, San Francisco, California. 4. Department of Dermatology, Vagelos College of Physicians & Surgeons, Columbia University Medical Center, New York, New York; Department of Pediatrics, Vagelos College of Physicians & Surgeons, Columbia University Medical Center, New York, New York. 5. Department of Dermatology, Centre Hospitalier Universitaire Sainte-Justine, Montréal, Quebec, Canada. 6. Department of Dermatology, Hospital for Sick Children, Toronto, Ontario, Canada. 7. Department of Dermatology, Mayo Clinic, Rochester, Minnesota. 8. Department of Dermatology, Sydney Children's Hospital, Sydney, New South Wales, Australia. 9. Department of Dermatology, Vagelos College of Physicians & Surgeons, Columbia University Medical Center, New York, New York; Department of Pediatrics, Vagelos College of Physicians & Surgeons, Columbia University Medical Center, New York, New York. Electronic address: mcg2@cumc.columbia.edu.
Abstract
BACKGROUND: High-flow vascular stains (HFVS) are lesions that have the appearance of capillary malformations/port wine stains but are associated with increased arterial flow. OBJECTIVE: To identify features of HFVS that differentiate them from typical "slow-flow" port wine stains. METHODS: Retrospective multicenter cohort study of HFVS evaluated across 7 centers was conducted. HFVS were characterized by clinical features (warmth, thrill, rapid capillary refill), radiologic findings (fast flow), or mutations associated with capillary malformation-arteriovenous malformation syndrome. Investigators reviewed photographs. RESULTS: The study reviewed 70 patients with HFVS (47 multifocal and 23 solitary). Most were flat (77%), warm to the touch (60%), and red or pink-red in color (35%), with heterogeneous color saturation (73%) and well-defined borders (71%). Regional soft tissue swelling/overgrowth was common (47%). Head and neck location was most common (38%). Among 34 HFVS with photographic review over time, all demonstrated changes in appearance. LIMITATIONS: Retrospective design, recall bias, lack of standardized time points or visual analog scale, and image variability. CONCLUSION: Heterogeneity of stain color saturation, warmth to touch, peripheral pallor, and overgrowth/soft tissue swelling help distinguish HFVS from port wine stains. Darkening of color and increased border demarcation may develop over time. These findings raise suspicion for HFVS and provide an indication to assess for extracutaneous involvement.
BACKGROUND: High-flow vascular stains (HFVS) are lesions that have the appearance of capillary malformations/port wine stains but are associated with increased arterial flow. OBJECTIVE: To identify features of HFVS that differentiate them from typical "slow-flow" port wine stains. METHODS: Retrospective multicenter cohort study of HFVS evaluated across 7 centers was conducted. HFVS were characterized by clinical features (warmth, thrill, rapid capillary refill), radiologic findings (fast flow), or mutations associated with capillary malformation-arteriovenous malformation syndrome. Investigators reviewed photographs. RESULTS: The study reviewed 70 patients with HFVS (47 multifocal and 23 solitary). Most were flat (77%), warm to the touch (60%), and red or pink-red in color (35%), with heterogeneous color saturation (73%) and well-defined borders (71%). Regional soft tissue swelling/overgrowth was common (47%). Head and neck location was most common (38%). Among 34 HFVS with photographic review over time, all demonstrated changes in appearance. LIMITATIONS: Retrospective design, recall bias, lack of standardized time points or visual analog scale, and image variability. CONCLUSION: Heterogeneity of stain color saturation, warmth to touch, peripheral pallor, and overgrowth/soft tissue swelling help distinguish HFVS from port wine stains. Darkening of color and increased border demarcation may develop over time. These findings raise suspicion for HFVS and provide an indication to assess for extracutaneous involvement.
Authors: Bede N Nriagu; Victoria R Sanders; Lionel Bercovitch; Kristen Snyder; Elizabeth A Cross; James R Treat; Sarah E Sheppard Journal: Pediatr Dermatol Date: 2021-09-28 Impact factor: 1.997