Sturge-Weber Syndrome (SWS) is a rare congenital developmental disorder that arises from the abnormal persistence of the embryonic vascular plexus. The syndrome encompasses hamartomatous malformations that can impact the skin, eyes, and nervous system. The broad array of clinical manifestations and potentially life-threatening complications underscores the crucial and imperative need for an accurate diagnosis. An effective treatment strategy for SWS patients involves a multidisciplinary approach. Dental procedures in these individuals pose challenges due to the potential risk for substantial bleeding during both intra- and postoperative phases. This article aims to document a rare case of Sturge Weber Syndrome in a 21-year-old female patient who presented with seizures, unilateral facial port wine stains, gingival hyperplasia, and intracranial calcifications.

OBJECTIVE: This study aims to evaluate the potential impact of port wine stains on the development of permanent teeth in mixed dentition and provide insights for managing tooth abnormalities in patients with port wine stains.
METHODS: A retrospective analysis was conducted on 21 patients with mixed dentition and unilateral maxillary port wine stains. Two researchers concurrently utilized Nolla Analysis to assess the developmental stage of bilateral maxillary and permanent mandibular teeth based on panoramic radiographs. The cumulative developmental values of upper and lower permanent teeth on both sides were calculated, and a comparison was made between the developmental stages of the upper and lower jaws.
RESULTS: Port wine stains can influence the maturity of permanent upper teeth, within the unilateral maxillary port wine stains range, with an early developmental completion observed on the affected side compared to the unaffected side. While the developmental stages of the lower teeth on both sides showed similarities.
CONCLUSIONS: Port wine stains can accelerate the maturity of teeth on the affected side, leading to alterations in the order of tooth eruption and subsequent abnormal occlusion in children with mixed dentition. These findings provide a basis for developing appropriate management strategies for addressing tooth abnormalities in patients with port wine stains.

BACKGROUND: Port wine stains slowly grow thicker over time, nodules appear on the surface, and the color slowly deepens from pink to purple. Even after laser treatment, some port wine stains will recur and slowly grow, and the erythema appears again. The purpose of this study was to investigate the effectiveness of bleomycin in combination with triamcinolone acetonide in the treatment of inhibiting the proliferation and recurrence of port wine stains.
RESULTS: Histopathological change: Before treatment, dense capillaries were distributed within the lesion, and blood fills the lumen. Lack of normal skin structure: After bleomycin and triamcinolone acetonide injection, the number of capillaries was significantly reduced, and fibrosis occurred in tissues. Changes in facial morphology: After treatment, the port wine stains became thinner, the asymmetry of the face was effectively improved, and the appearance have been significantly improved. After 5 years of follow-up, there were no recurrent cases.
CONCLUSIONS: Bleomycin and triamcinolone acetonide injection can effectively inhibit the proliferation of port wine stains and prevent port wine stains from recurring after treatment.

Background: Conventional treatments of port wine stain birthmarks often do not achieve the desired outcome in patients with hypertrophy. Potential reasons include deeper and larger blood vessels, abnormal arrangement of blood vessels, and darker or thicker epidermis. However, these factors may not significantly limit the efficacy of fractional carbon dioxide (CO2) laser. The aim of this case report was to examine the extended application of fractional CO2 laser in treating patients with hypertrophic port wine stain birthmarks. Methods: Two cases with hypertrophic port wine stain birthmarks treated with fractional CO2 laser for 5 years are described in this case report. Results: When compared with conventional treatment, both cases reported better outcomes, including a reduced risk of infection, pigmentation, and scarring, a decrease of clinical erythema, and much less pain. Conclusions: The findings demonstrate that fractional CO2 laser has the potential to be an effective modality for the treatment of patients with hypertrophic port wine stains.

UNASSIGNED: Port wine stains (PWS) often cause cosmetic effects and psychological distress. Pulsed dye lasers (PDL) and photodynamic therapy (PDT) are the most commonly used treatments. PDL is still the \"gold standard\" of therapy to date. However, its shortcomings have become apparent as clinical applications have increased. PDT has been proven as an alternative to PDL. Patients with PWS still lack enough evidence about PDT to make informed treatment decisions.
UNASSIGNED: The purpose of this systematic review and meta-analysis was to assess the safety and effectiveness of PDT for PWS.
UNASSIGNED: The online datasets, comprising PubMed, Embase, Web of Science, and the Cochrane Library, were searched for meta-analysis-relevant publications. Two reviewers separately evaluated the risk of bias in each listed study. Grading of Recommendations Assessment, Development, and Evaluation (GRADE) was used to assess the treatment and safety outcomes.
UNASSIGNED: Our search retrieved 740 hits and only 26 studies were finally included. Among the 26 studies included, 3 were randomized clinical trials, and 23 were prospective or retrospective cohort investigations. Based on a gathered assessment, the percentage of individuals achieving a 60% improvement was estimated to be 51.5% [95% confidence interval (CI): 38.7-64.1; I 2 = 83.8%] and a ≥75% improvement was 20.5% (95% CI: 14.5-26.5; I 2 = 78.2%) after 1-8.2 treatment sessions (GRADE score: very low). Due to the statistical diversity of the meta-analysis, a subgroup assessment was performed to determine the sources of diversity. The collected findings indicated that the impact of PDT on enhancing the medical effectiveness of PWS was significant in different treatment sessions, different types of ages, different locations of PWS, and different types of PWS. Pain and edema occurred in most patients. Hyperpigmentation was present in 7.9-34.1% of the patients in 17 studies. Photosensitive dermatitis, hypopigmentation, blister, and scar were infrequently reported, with 0-5.8% incidences.
UNASSIGNED: Photodynamic therapy is recommended as a safe and effective treatment for PWS based on the current evidence. However, our findings are based on poor-quality evidence. Therefore, comparative investigations of a large scale and high quality are necessary to support this conclusion.

BACKGROUND: Pulsed dye laser (PDL) is the main treatment for port wine stain (PWS), but a considerable number of patients show low clearances. The reason for the poor efficacy is related to PDL-induced angiogenesis. Vascular endothelial growth factor (VEGF) plays an important role in PDL-induced angiogenesis and can activate the tyrosine kinase activity of VEGF receptor (VEGFR) in endothelial cells. It triggers a full range of responses, and then participates in the regulation of angiogenesis. Tivozanib is an inhibitor of VEGFR tyrosine kinase activity, which can block the pro-angiogenic effect of VEGF and reduce vascular permeability.
METHODS: Different energy densities of PDL were used to irradiate the abdominal skin of rats. According to the general and pathological changes of the irradiated area, the energy density of 8 J/cm2 with smaller scab and stronger vascular effect was selected for follow-up experiments. Divided the rat abdomen skin into four areas, irradiated three of them uniformly with an energy density of 8 J/cm2 , and applied different concentrations of Tivozanib coating agent to the laser irradiation area, and grouped them as follows: (1) vacant group, (2) control group, (3) 0.5% Tivozanib group, (4) 1% Tivozanib group. Camera and dermoscopy were used to observe skin changes. Hematoxylin-eosin staining, immunohistochemical staining, and blood vessels were counted to detect dermal vascular regeneration. Transcriptome sequencing and real-time polymerase chain reaction (PCR) were conducted to elucidate the mechanism and validate the reliability.
RESULTS: The number of blood vessels in the 0.5% Tivozanib group and 1% Tivozanib group was significantly reduced on the 7, 10, and 14 days compared with the control group. The number of blood vessels in the 1% Tivozanib group was significantly reduced compared with the 0.5% Tivozanib group, indicating that Tivozanib successfully inhibited PDL-induced angiogenesis, and the inhibitory effect of 1% Tivozanib was more significant than that of 0.5% Tivozanib. Transcriptome sequencing results showed a total of 588 significantly differentially expressed genes, including 90 upregulated genes and 498 downregulated genes. Gene ontology (GO) and kyoto encyclopedia of genes and genomes (KEGG) enrichment analysis showed that the significantly differentially expressed genes were mainly enriched in the metabolic pathways which were closely related to angiogenesis. Finally, real-time PCR was used to verify the genes with higher expression differences, the top ranking and closely related to angiogenesis, namely, Cxcl1, Cxcl2, Cxcl3, Cxcl6, Ccl3, Csf3, IL1β, iNOS, Mmp9, Mmp13, Plau, Ets1, Spp1, Nr4a1. The results were consistent with the trend of transcriptome sequencing results, which proved the reliability of this study.
CONCLUSIONS: This study explored the inhibitory effect of Tivozanib on PDL-induced angiogenesis, and provided a new idea for the treatment of clinical PWS. Transcriptome sequencing explored the mechanism and provided reliable clues for later in-depth research.

CLOVES syndrome is a novel sporadic mosaic segmental overgrowth syndrome, currently categorized under the canopy of PROS (PIK3CA-related overgrowth spectrum) disorders. All PROS disorders harbor heterozygous postzygotic activating somatic mutations involving the PIK3CA gene. As an upstream regulator of the PI3K/AKT/mTOR signal transduction pathway, activating mutations of PIK3CA gene commence in uncontrolled growth of cutaneous, vascular (capillaries, veins, and lymphatics), adipose, neural, and musculoskeletal tissues. The excessive growth is segmental, patchy, asymmetric, and confined to body parts affected by the mutation. The term \'CLOVES\' is an acronym denoting congenital lipomatous overgrowth, vascular malformations, epidermal nevi and spinal (scoliosis) and/ or skeletal anomalies. The syndrome is characterized by an admixture of overgrown tissues, derived mainly from mesoderm and neuroectoderm. Among PROS disorders, CLOVES syndrome represents the extreme end of the spectrum with massive affection of almost the entire body. The syndrome might judiciously be treated with medications hampering with the PI3K/AKT/mTOR signal transduction pathway. This article aims at reviewing the cutaneous and musculoskeletal manifestations of CLOVES syndrome, as the paradigm for PROS disorders. CLOVES syndrome and other PROS disorders are still misdiagnosed, underdiagnosed, underreported, and undertreated by the dermatology community.

Based on the well-known principle of selective photothermolysis, laser has been a promising way for the treatment of port wine stains (PWSs). The laser wavelengths used for PWS\'s clinical treatment include but are not limited to pulsed dye laser (PDL) in 585-600 nm, long-pulse 755-nm alexandrite, and 1064-nm Nd:YAG lasers. The objective of this study was to investigate the optimal wavelength for PWS\'s laser treatment. A two-scale mathematic model was constructed to simultaneously quantify macroscale laser energy attenuation in two-layered bulk skin and microscale local energy absorption on target blood vessels within Krogh unit. The effects of morphological parameters, including epidermal melanin content, epidermal thickness, dermal blood content, blood vessel depth, and diameter on laser energy deposition within target blood vessels, were investigated from the visible to near-infrared bands (500-1100 nm). The energy deposition ratio of target blood vessel to epidermal surface was proposed to determine the optimal laser wavelength for PWS with different skin morphological parameters. The bioheat transfer modeling and animal experiment are also conducted to prove our wavelength optimization. The optimal wavelengths for lightly pigmented skin with small and shallow target blood vessels are 580-610 nm in the visible band. This wavelength coincides with commercially used PDL. The optimal wavelength shifts to 940 nm as the epidermal pigmentation increases or the size and blood vessel depth increases. The optimal wavelength changes to 1005 nm as the epidermal pigmentation or the size and burying depth of target blood vessel further increases. Nine hundred forty nanometers can be selected as a general wavelength in PWS treatment to meet the need in most widely morphological structure. Lasers with wavelengths in the 580-610, 940, and 1005 nm regions are effective for treating PWS because of their high optical selectivity in blood over the epidermis.

BACKGROUND: Vascular lesions such as port wine stains (PWS) lead to facial and psychological problems, which require careful and precise treatments. The key point of treating PWS is to selectively destroy the abnormal blood vessels. Hence, the in vivo monitoring of targeted vessels is crucial. Optical coherence tomography angiography (OCTA), an emerging label-free imaging tool, facilitates the evaluation of skin structure and vasculature at a high resolution. In this study, we utilised OCTA to capture the structural and vascular morphology in patients with PWS. Moreover, we quantitatively characterised the morphological features of different types of PWS.
METHODS: This observational clinical study was conducted on 3 patients with flat PWS and 3 patients with thickened PWS. The age range was 4-27 years, and all of them had not received any treatment before this study. The OCTA images of the PWS lesions and contralateral skin were compared. Vascular morphology was characterized, and ectatic vessel depth was quantified according to the OCTA images.
RESULTS: The blood vessels of the PWS lesions tend to had larger diameters and higher densities than those in the contralateral normal skin. The vessel diameters of PWS lesions were 73 ± 14 μm, with high heterogeneity ranging from 10 to >150 μm, however, the vessel diameters of normal skin were 28 ± 2 μm, ranging from 10 μm to 60 μm. In terms of different PWS lesions, the thickened type showed a trend of larger vessel diameter and higher density than those of the purplish red type. The ectatic vessels were located at the depth of 216 ± 13 μm in the PWS skin.
CONCLUSIONS: OCTA can facilitate the in vivo three-dimensional visualization of structure and vasculature for PWS lesions. Various quantitative analysis parameters, such as vessel diameter, density, and depth, are typically measured using OCTA. This fact demonstrates the superior capability of OCTA for the precise and comprehensive assessment of PWS lesions.

The 755-nm Alexandrite Laser has a good clinical effect in treating resistant port wine stain, without causing thermal damage of normal tissue and side effects such as purpura. However, little is known about the mechanism of vascular damage induced by 755-nm laser irradiation, which restricts the optimization of laser parameters. In this work, the thermal damage model and the pressure damage model were used to study the damage mechanism of 755-nm laser irradiation on vessels, and the incident energy density and pulse width required for vascular damage were determined according to the damage mode. Under the irradiation of 755-nm laser, the vascular injury pattern was the co-occurrence of vessel rupture and vessel constriction, and the energy density required for the treatment of vessels with a diameter of 200 μm to reach the damage threshold was the lowest.