Port-Wine Stains: A Focused Review on Their Management

November 2017 | Volume 16 | Issue 11 | Original Article | 1145 | Copyright © 2017

Katelyn Mariko Updyke BS,a Amor Khachemoune MD FAAD FACMSbc

aUniversity of Central Florida College of Medicine, Orlando FL bState University of New York Downstate, Brooklyn, NY cDermatology Service, Veterans Affairs Medical Center, Brooklyn, FL

Abstract

Port-wine stain (PWS) is the second most common congenital vascular malformation characterized as ectatic capillaries and venules in the dermis that clinically appears as a deep red to purple patch on the skin. Typically, PWS progressively darken and may become hypertrophic or nodular without treatment. There are several treatment options available for PWS from topical antiangiogenic agents to laser therapies. Vascular-specific lasers are the gold standard in treating PWS and classically pulsed dye lasers are usually the treatment of choice. However, some patients with PWS are recalcitrant to PDL and may require a combination of treatment methods. Nonetheless, even with the advancements in laser therapies utilized today, it is can be difficult to achieve complete clearance of the PWS. Thus, new innovations for treating recalcitrant PWS are underway in order to improve overall patient treatment outcomes.

J Drugs Dermatol. 2017;16(11):1145-1151.

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INTRODUCTION

Historical Description and Epidemiology

Nevus flammeus, or port-wine stains (PWS), initially misnamed as “flat hemangioma” and previously called “firemark,” are the second most common congenital vascular malformation seen in infancy. An estimated of 3 children per 1000 live births, or approximately 400,000 per year, will develop PWS birthmarks.1 Although PWS have no sex predilection, they are much less common in Asians and African Americans.2

Pathogenesis and Clinical Presentation

It is proposed that PWS develop due to irregularities in neural development (ie, axonal degeneration) and genetic mutations that may be familial or sporadic.3,4 Studies have shown that biopsies of PWS specimen have a greater vessel-to-nerve ratio with overall decreased nerve density relative to normal skin.3 It has been proposed that irregular blood flow in PWS leads to chronic ischemia and results in further axonal injury and degeneration. Furthermore, the lack of neural modulation of blood flow results in worsening of ectatic vessels.4 Other dermatologic diseases have neuronal degeneration or loss as part of their pathogenesis but have widely different clinical presentations than PWS. For example, xeroderma pigmentosum (XP), which is characterized as a genetic disease that causes progressive neurologic deterioration with 10,000-fold and 2,000-fold increase in developing non-melanoma and melanoma skin cancers in early childhood, respectively.5 Additionally, skin biopsies from patients with vitiligo, an idiopathic disease that causes skin depigmentation due to the destruction of melanocytes, have also shown both axonal degeneration as part of its pathogenesis.6PWS typically occur in regions that are normally innervated by certain nerve branches, such as the trigeminal nerve, and show significantly decreased nerve fiber density compared to unaffected skin.7,8 They present as well-demarcated, solid, pink, red or purple patches and can affect any part of the body; however, they frequently effect the head and neck area, particularly in a V1 and V2 dermatomal distribution.2 Dilated capillaries and post-capillary venules in associated PWS lesions results in increased hemoglobin content in the overlying skin, which causes the deeply red to purple pigmented skin.1Capillary malformations (CM) do not proliferate and thus do not grow, but instead they demonstrate chronic vascular dilation with possible gradual darkening and thickening over many years with or without treatment.9 The deep reddish purple to violaceous color created by the hemoglobin pigment appears like Port-Wine, which is where they inherited their name.1,10It is important to differentiate PWS from other vascular tumors that may present during infancy or early childhood since the diagnosis may change treatment options and overall patient outcome. Some other vascular tumors include infantile hemangiomas, pyogenic granulomas, spider angiomas, venous malformations, and angiokeratomas which are summarized in Table 1.

Associated Syndromes

Most facial PWS usually occur as isolated birthmarks, but up to 10% of PWS may be associated with Sturge-Weber Syndrome (SWS), a noninherited congenital vascular disorder. SWS is associated with facial PWS and underlying leptomeningeal capillary-venous malformation or choroidal malformation of the eye, which may lead to the development of neurologic or ophthalmologic complications in children with SWS. These

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