Treatment Update of Port-Wine Stain: A Narrative Review

May 2021 | Volume 20 | Issue 5 | Original Article | 515 | Copyright © May 2021


Published online April 29, 2021

Regina Fölster-Holst MDa, Ratnakar Shukla MDb, Martin Kassir MDc, Hassan Galadari MDd, Torello Lotti MDe, Uwe Wollina f, Stephan Grabbe MDg, Mohamad Goldust MDg

aDermatology, Venerology and Allergology, Universitätsklinikum Schleswig-Holstein, Keil, Germany
bDepartment of Dermatology, All India Institute of Medical Sciences), Patna, India
cWorldwide laser institute, Dallas, TX
dCollege of Medicine and Health Sciences, United Arab Emirates University, Al Ain, United Arab Emirates
eUniversity of Studies Guglielmo Marconi, Rome, Italy
fDepartment of Dermatology and Allergology, Städtisches Klinikum Dresden, Academic Teaching Hospital of the Technical University of Dresden, Dresden, Germany
gDepartment of Dermatology, University Medical Center of the Johannes Gutenberg University, Mainz, Germany

Abstract
Background: Port-wine stain (PWS) is a congenital vascular malformation affecting 0.3–0.5% of normal population. These characteristic lesions arise due to the interplay of vascular, neural, and genetic factors. Treatment options include lasers, cosmetic tattooing, electrotherapy, cryosurgery, derma-abrasion, and skin grafting; however, none of these treatment alternatives appears to be satisfactory and is unable to provide consistent, satisfactory responses or even complete cures. Currently, laser is the treatment of choice, as it is comparatively safe and more effective than other procedures. The most commonly used modality is pulsed dye laser (PDL). The literature research includes peer-reviewed articles (clinical trials or scientific reviews). Studies were identified by searching electronic databases (MEDLINE and PubMed) to January 2020 and reference lists of respective articles. Only articles published in English language were included.

J Drugs Dermatol. 20(5):515-518. doi:10.36849/JDD.5005

INTRODUCTION

Port-wine stain (PWS), so named due to its characteristic appearance of red wine spilled or splashed on the skin, is also referred to as nevus flammeus. It is the most prevalent type of congenital vascular malformations and increases in size proportionally with age, does not involute and persists for life. It is present in approximately 0.3%–0.5% of infants as a result of the interplay of various etiological factors, most commonly from abnormal morphogenesis characterized by dilatation of dermal capillaries and post capillary venules.1 Vascular endothelial growth factor (VEGF)-A and its receptor VEGF-R2 expressed in capillary malformation, so antiangiogenic treatment blocking VEGF may prove to be useful and be a future treatment option.2 Tyrosine kinase receptor expression along with its ligand angiopoietin-1 expression is also increased in lesional skin.3 Augmented hemoglobin content in the skin produces a color change.4 Histologically, PWS shows dilated vessels in the dermis without endothelial proliferation.5 The diameter of the vessels varies from 2 to 500 μm.6 Only 17% of PWS vessels show vasa nervorum in contrast to 75% in normal skin.7 There may also be a neural role, usually a deficiency of nerve innervation, that may be a cause of these abnormal hypervascular skin lesions.8

The genetic role is evident by a somatic activating mutation in the GNAQ gene (c. 548GA, pR183Q), which encodes a guanine nucleotide-binding protein G-alpha-q subunit. This seems to generate activation of extracellular signal-regulated kinase (ERK), c-Jun N-terminal kinase and P70 ribosomal S6 kinase.9 In addition, mutations of RASA1 have been demonstrated that are responsible for capillary and arteriovenous malformations and limb enlargement.10 In hypertrophic PWS, hamartomatous changes suggest a multilineage development field effects.11 PWS most commonly presents on the face and neck; however, it can occur anywhere on the body. The condition is associated with psychological stress and impaired quality of life. Importantly, PWS adjacent to eyelids is associated with early-onset glaucoma. Ophthalmic distribution (V1 dermatome) may be an indicator for Sturge-Weber syndrome, particularly if bilateral.12

PWS should be treated at early infancy.4 Previously, treatment options were cosmetic, radiation, skin grafting, dermabrasion, cryosurgery, tattooing, and electrotherapy. However, the benefit of these modalities is limited and none of these provided reliable cosmetic results as laser.