- Port-wine stain
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Port-wine stain Classification and external resources 
Mikhail Gorbachev has a prominent port-wine stain on his forehead.ICD-10 Q82.5 (ILDS Q82.510) ICD-9 757.32 OMIM 163000 DiseasesDB 10384 MedlinePlus 001475 eMedicine derm/295 MeSH D019339 A port-wine stain or naevus flammeus is a vascular anomaly consisting of superficial and deep dilated capillaries in the skin which produce a reddish to purplish discoloration of the skin. They are so called for their colour, resembling that of port wine. It is part of the family of disorders known as vascular malformations, specifically an arteriovenous malformation.
The two terms are not always equated. Sometimes the term "naevus flammeus" is divided into two categories: port-wine stain and salmon patch.[1][2]
Port-wine stains are present at birth and persist throughout life. The area of skin affected grows in proportion to general growth. Port-wine stains occur most often on the face but can appear anywhere on the body. Early stains are usually flat and pink in appearance. As the child matures, the color may deepen to a dark red or purplish colour. In adulthood, thickening of the lesion or the development of small lumps may occur[3].
PWS may be one of a group of symptoms and signs, in which case it is considered to be part of a syndrome such as Sturge-Weber syndrome or Klippel-Trenaunay-Weber syndrome.
Contents
Types
A hand with a red wine-port stain
Nevus flammeus may be divided as follows:[4]
Genetics
An association with RASA1 has been described.[5]
Diagnosis
A physician can usually diagnose a port wine stain based entirely upon the history and appearance. In unusual cases, a skin biopsy may be needed to confirm the diagnosis. Depending on the location of the birthmark and other associated symptoms, a physician may choose to order a measurement of intraocular pressure or X-ray of the skull.
Treatment
Many treatments have been tried for port-wine stains including freezing, surgery, radiation, and tattooing; port-wine stains can also be covered with cosmetics. Lasers have made the biggest impact on treatment, because they are the sole method of destroying the cutaneous capillaries without significant damage to the overlying skin.
The flashlamp pumped dye laser, a yellow light laser, has been the most successful at destroying stains in infants and young children.[6] The neodymium YAG laser is used to treat the nodules that may develop in some adult port-wine stains.
Treatment of infants with the flashlamp pumped dye laser generally produces marked improvement in appearance.[5]
However, complete disappearance is rare. In approximately 20%[7] of cases there may be no improvement at all. Stains on the face respond better than those on the trunk or limbs. Older stains may be more difficult to treat.
Prognosis
In the absence of successful treatment, hypertrophy (increased tissue mass) of the stains may produce deformity, loss of function (especially near the eye or mouth), bleeding, and increasing disfigurement. These complications are usually seen later in life. If the PWS is on the face or other highly visible part of the body, the presence of PWS can also cause emotional and social problems for the affected person because of their cosmetic appearance.
Epidemiology
The incidence is 3–5 out of 1000 persons.[8][9]
References
- ^ "nevus flammeus" at Dorland's Medical Dictionary
- ^ "port-wine stain" at Dorland's Medical Dictionary
- ^ Minkis K, Hale E, Geronemus R. "Port Wine Stain Progression: A Potential Consequence of Delayed and Inadequate Treatment" Lasers Surg Med. 41:423-426. 2009
- ^ James, William; Berger, Timothy; Elston, Dirk (2005). Andrews' Diseases of the Skin: Clinical Dermatology. (10th ed.). Saunders. Page 582-3. ISBN 0-7216-2921-0.
- ^ a b Eerola I, Boon LM, Mulliken JB, et al. (December 2003). "Capillary malformation-arteriovenous malformation, a new clinical and genetic disorder caused by RASA1 mutations". Am. J. Hum. Genet. 73 (6): 1240–9. doi:10.1086/379793. PMC 1180390. PMID 14639529. http://linkinghub.elsevier.com/retrieve/pii/S0002-9297(07)63977-9.
- ^ Chapas A, Eickhorst, K, Geronemus R. Efficacy of Early Treatment of Facial Port Wine Stains in Newborns: A Review of 49 Cases. Lasers Surg Med. 2007. (39) 7: 563-68 [1]
- ^ Jasim ZF, Handley JM (2007). "Treatment of pulsed dye laser-resistant port wine stain birthmarks". J. Am. Acad. Dermatol. 57 (4): 677–82. doi:10.1016/j.jaad.2007.01.019. PMID 17658196.
- ^ Jacobs AH, Walton RG, The incidence of birthmarks in the neonate. Pediatrics 1976; 58:218-222
- ^ Alper JC, Holmes LB, The incidence and significance of birthmarks in a cohort of 4641 newborns. Pediatr Dermatol 1986; 1:58-68
External links
- Information about Port Wine Stains from Children's Hospital, Seattle.
- Birthmarks.com
- Sturge-Weber Syndrome Community
Congenital malformations and deformations of integument / skin disease (Q80–Q82, 757.0–757.3) Genodermatosis Congenital ichthyosis/
erythrokeratodermiaADARUngroupedIchthyosis bullosa of Siemens · Ichthyosis follicularis · Ichthyosis prematurity syndrome · Ichthyosis–sclerosing cholangitis syndrome · Nonbullous congenital ichthyosiform erythroderma · Ichthyosis linearis circumflexa · Ichthyosis hystrixEB
and relatedJEB (JEB-H, Mitis, Generalized atrophic, JEB-PA)related: Costello syndrome · Kindler syndrome · Laryngoonychocutaneous syndrome · Skin fragility syndrome ·Naegeli syndrome/Dermatopathia pigmentosa reticularis · Hay–Wells syndrome · Hypohidrotic ectodermal dysplasia · Focal dermal hypoplasia · Ellis–van Creveld syndrome · Rapp–Hodgkin syndrome/Hay–Wells syndromeEhlers–Danlos syndrome · Cutis laxa (Gerodermia osteodysplastica) · Popliteal pterygium syndrome · Pseudoxanthoma elasticum · Van Der Woude syndromeHyperkeratosis/
keratinopathydiffuse: Diffuse epidermolytic palmoplantar keratoderma • Diffuse nonepidermolytic palmoplantar keratoderma • Palmoplantar keratoderma of Sybert • Mal de Meleda •syndromic (connexin (Bart–Pumphrey syndrome • Clouston's hidrotic ectodermal dysplasia • Vohwinkel syndrome) • Corneodermatoosseous syndrome • plakoglobin (Naxos syndrome) • Scleroatrophic syndrome of Huriez • Olmsted syndrome • Cathepsin C (Papillon–Lefèvre syndrome • Haim–Munk syndrome) • Camisa diseasefocal: Focal palmoplantar keratoderma with oral mucosal hyperkeratosis • Focal palmoplantar and gingival keratosis • Howel–Evans syndrome • Pachyonychia congenita (Pachyonychia congenita type I • Pachyonychia congenita type II) • Striate palmoplantar keratoderma • Tyrosinemia type II)punctate: Acrokeratoelastoidosis of Costa • Focal acral hyperkeratosis • Keratosis punctata palmaris et plantaris • Keratosis punctata of the palmar creases • Schöpf–Schulz–Passarge syndrome • Porokeratosis plantaris discreta • Spiny keratodermaungrouped: Palmoplantar keratoderma and spastic paraplegia • desmoplakin (Carvajal syndrome) • connexin (Erythrokeratodermia variabilis • HID/KID)OtherMeleda disease · Keratosis pilaris · ATP2A2 (Darier's disease) · Dyskeratosis congenita · Lelis syndromeDyskeratosis congenita · Keratolytic winter erythema · Keratosis follicularis spinulosa decalvans · Keratosis linearis with ichthyosis congenital and sclerosing keratoderma syndrome · Keratosis pilaris atrophicans faciei · Keratosis pilarisOthercadherin (EEM syndrome) · immune system (Hereditary lymphedema, Mastocytosis/Urticaria pigmentosa) · Hailey–Hailey
see also Template:Congenital malformations and deformations of skin appendages, Template:Phakomatoses, Template:Pigmentation disorders, Template:DNA replication and repair-deficiency disorderDevelopmental
anomaliesMidlineOther/ungroupedAplasia cutis congenita · Amniotic band syndrome · Branchial cyst · Cavernous venous malformation
Accessory nail of the fifth toe · Bronchogenic cyst · Congenital cartilaginous rest of the neck · Congenital hypertrophy of the lateral fold of the hallux · Congenital lip pit · Congenital malformations of the dermatoglyphs · Congenital preauricular fistula · Congenital smooth muscle hamartoma · Cystic lymphatic malformation · Median raphe cyst · Melanotic neuroectodermal tumor of infancy · Mongolian spot · Nasolacrimal duct cyst · Omphalomesenteric duct cyst · Poland anomaly · Rapidly involuting congenital hemangioma · Rosenthal–Kloepfer syndrome · Skin dimple · Superficial lymphatic malformation · Thyroglossal duct cyst · Verrucous vascular malformation · BirthmarkCategories:- Dermal and subcutaneous growths
- Laser medicine
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