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Port-wine stain in proteus syndrome — Clinical Guidelines

Overview

Port-wine stains (PWS) are congenital vascular malformations often associated with Proteus syndrome, characterized by persistent reddish or purplish discoloration of the skin due to dilated capillaries. Treatment aims to reduce lesion appearance but often falls short of complete resolution 3.

Diagnosis

Clinical appearance of persistent vascular lesions since birth 3.

No specific diagnostic tests; evaluation typically relies on visual inspection and patient history 3.

Depth and extent of the lesion may be assessed using photoacoustic probes for optimized laser treatment planning 8.

Management

First-line treatment: Laser therapy, particularly pulsed dye laser (PDL), remains the primary approach 3 5 11.

Adjunctive treatments: Pneumatic skin flattening (PSF) can reduce pain and enhance treatment efficacy in resistant cases 5.

Technological enhancements: Use of exogenous chromophores like liposome@Au core-shell nanoparticles to enhance laser efficacy 2.

Assessment tools: Comprehensive questionnaires for evaluating lesion characteristics post-treatment, focusing on color, boundary sharpness, and size 9 10.

Special Populations

Pediatrics: PDL treatment is commonly used in children, with considerations for pain management and long-term outcomes 5 11.

Comorbidities: No specific guidelines provided in abstracts regarding additional comorbidities; treatment focuses primarily on lesion management 3.

Key Recommendations

Primary treatment with pulsed dye laser (PDL) for port-wine stains to achieve significant color reduction 3 5 11 (Evidence: Strong).

Utilize pneumatic skin flattening (PSF) technology to minimize patient discomfort during multiple laser sessions, especially in pediatric patients 5 (Evidence: Moderate).

Employ comprehensive assessment tools post-treatment to evaluate lesion characteristics objectively 9 10 (Evidence: Moderate).

References

1 Evers MS, Ramousse L, Morge C, Sparrow C, Gobert A, Roullier-Gall C et al.. To each their own: Delving into the vitaminic preferences of non-Saccharomyces wine yeasts. Food microbiology 2023. link 2 Li D, Zhang Q, Xing L, Chen B. Theoretical and in vivo experimental investigation of laser hyperthermia for vascular dermatology mediated by liposome@Au core-shell nanoparticles. Lasers in medical science 2022. link 3 Fölster-Holst R, Shukla R, Kassir M, Galadari H, Lotti T, Wollina U et al.. Treatment Update of Port-Wine Stain: A Narrative Review. Journal of drugs in dermatology : JDD 2021. link 4 Feldstein S, Totri CR, Friedlander SF. Can long-term alopecia occur after appropriate pulsed-dye laser therapy in hair-bearing sites? Pediatric dermatologists weigh in. Dermatologic surgery : official publication for American Society for Dermatologic Surgery [et al.] 2015. link 5 Kautz G, Kautz I, Segal J, Zehren S. Treatment of resistant port wine stains (PWS) with pulsed dye laser and non-contact vacuum: a pilot study. Lasers in medical science 2010. link 6 Terrade N, Mira de Orduña R. Determination of the essential nutrient requirements of wine-related bacteria from the genera Oenococcus and Lactobacillus. International journal of food microbiology 2009. link 7 Mahendran R, Sheehan-Dare RA. Survey of the practices of laser users in the UK in the treatment of port wine stains. The Journal of dermatological treatment 2004. link 8 Viator JA, Au G, Paltauf G, Jacques SL, Prahl SA, Ren H et al.. Clinical testing of a photoacoustic probe for port wine stain depth determination. Lasers in surgery and medicine 2002. link 9 Koster PH, Bossuyt PM, van der Horst CM, Gijsbers GH, van Gemert MJ. Assessment of clinical outcome after flashlamp pumped pulsed dye laser treatment of portwine stains: a comprehensive questionnaire. Plastic and reconstructive surgery 1998. link 10 Pérez B, Abraira V, Núñez M, Boixeda P, Perez Corral F, Ledo A. Evaluation of agreement among dermatologists in the assessment of the color of port wine stains and their clearance after treatment with the flashlamp-pumped dye laser. Dermatology (Basel, Switzerland) 1997. link 11 Taïeb A, Touati L, Cony M, Léauté-Labrèze C, Mortureux P, Renaud P et al.. Treatment of port-wine stains with the 585-nm flashlamp-pulsed tunable dye laser: a study of 74 patients. Dermatology (Basel, Switzerland) 1994. link 12 Merlob P, Reisner SH. Familial nevus flammeus of the forehead and Unna's nevus. Clinical genetics 1985. link

Port-wine stain in proteus syndrome