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Year : 2015  |  Volume : 60  |  Issue : 1  |  Page : 104
Acquired port-wine stain in an adult male: First reported case from India with review of literature

1 Department of Dermatology, Maulana Azad Medical College and Lok Nayak Hospital, New Delhi, India
2 Department of Pathology, Maulana Azad Medical College and Lok Nayak Hospital, New Delhi, India

Date of Web Publication26-Dec-2014

Correspondence Address:
Shuchi Bansal
1246, Sector 15 (Part II), Gurgaon, Haryana
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0019-5154.147859

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Port-wine stains (PWSs) are congenital vascular lesions caused by progressive ectasia of blood vessels located in the vascular plexus of the dermis. Acquired PWSs develop later in life but are identical in morphology and histology to the congenital PWSs. Less than 75 cases of acquired PWSs have been reported in the published literature, of which there has not been a single report from India so far. Various factors have been proposed for its pathogenesis like trauma, actinic exposure, drugs, tumors, and herpes zoster infection. We report an acquired port-wine stain in a 41-year-old male. The causative factors, treatment, and previous reports of this uncommon entity have also been reviewed.

Keywords: Adult, acquired, port-wine stain

How to cite this article:
Bansal S, Garg VK, Wadhwa B, Khurana N. Acquired port-wine stain in an adult male: First reported case from India with review of literature. Indian J Dermatol 2015;60:104

How to cite this URL:
Bansal S, Garg VK, Wadhwa B, Khurana N. Acquired port-wine stain in an adult male: First reported case from India with review of literature. Indian J Dermatol [serial online] 2015 [cited 2020 Sep 23];60:104. Available from: http://www.e-ijd.org/text.asp?2015/60/1/104/147859

What was known?
Cases of acquired port-wine stains have been infrequently reported in the literature.

   Introduction Top

Port-wine stains (PWSs) are congenital vascular malformations comprised of ectatic dermal capillaries and affecting 0.3-1% of newborn infants. [1] Acquired PWSs are an uncommon entity developing later in life, but morphologically and histologically similar to the congenital lesions. [2] We hereby report a case of 41-year-old male patient who acquired PWS about a year ago. The etiopathogenesis of this rare entity and the previous reports have also been reviewed.

   Case Report Top

A 41-year-old male presented to the outpatient department of dermatology of our tertiary care hospital with complaint of asymptomatic reddish patches involving the lower part of right leg for 10 months. The patch started from near the ankle and gradually spread to involve whole of the medial aspect of right leg over a period of 10 months. We could not elicit any history of antecedent mechanical or thermal trauma, drug intake, topical application or excessive ultraviolet exposure.

Dermatological examination revealed a diffuse partially blanchable erythema extending from the right ankle and involving chiefly the medial aspect of right leg up to the knee [Figure 1]. There were also a few discrete irregularly shaped partially blanchable reddish-purple macules present on the anterior aspect of right leg [Figure 2].
Figure 1: A diffuse partially blanchable erythema extending from the right ankle and involving the medial aspect of right leg up to the knee

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Figure 2: Discrete irregularly shaped partially blanchable reddish-purple macules present on the anterior aspect of right leg

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On the basis of the clinical examination, a provisional diagnosis of acquired PWS was made and the patient investigated. The histopathological examination revealed multiple dilated, variably sized capillaries lined by a single layer of endothelial cells throughout the dermis showing lobular arrangement at places [Figure 3]. Color Doppler of the limb did not reveal any abnormality of the underlying arteries and veins.
Figure 3: (a) Multiple dilated variably sized capillaries in the dermis (H and E, ×40, arrows). (b) High power showing groups of capillaries lined by a single layer of endothelial cells (H and E, ×250, arrow)

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Laser therapy was discussed with the patient, but he declined treatment for the lesion.

   Discussion Top

PWSs are congenital vascular malformations usually presenting at birth in the form of pink-red to purple macules, which become darker, raised, and nodular as the person ages. [1] The most accepted hypothesis is a defective embryological maturation of the sympathetic fibers, resulting in a loss of normal sympathetic control of the cutaneous vessels which leads to ectasia. [3]

Acquired PWSs are infrequently reported in the literature [Table 1]. Various factors have been proposed for the occurrence of acquired PWSs. Of these, trauma has been found to be the most important causative factor in majority of the cases. Trauma-induced PWS was first described by Fegeler in 1949 and hence called Fegeler syndrome. [4] In a review of 59 patients with acquired PWS, trauma was found to be a causative factor in 17 (29%) cases. It has been proposed that injury may result in loss of a previously effective sympathetic regulation of the cutaneous blood flow, leading to development of the lesions. [5]
Table 1: Reports of acquired port-wine stain

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Few cases of acquired PWS have also been proposed secondary to oral medications like isotretinoin, oral contraceptive pills, simvastatin, and metformin. [6],[7] Isotretinoin causes skin fragility and frictional trauma, while simvastain and metformin have been shown to promote angiogenesis by upregulation of vascular endothelial growth factor. Isolated cases of acquired PWS following frostbite injury, obstruction of the peritoneovenous shunt, herpes zoster infection, cerebral arteriovenous malformation, spinal root compression, and solid brain tumor have also been reported.

No definite cause was found for the development of lesion in our patient. In such cases, a likely possibility of having a latent vascular anomaly which manifests clinically after unnoticed trauma should be considered.

An unusual finding in our patient was the presence of a lobular arrangement of dilated vessels in the dermis which has not been observed in previous cases of congenital or acquired PWS. Interestingly, there are multiple reports of development of pyogenic granulomas (PG) within PWS which also shows a lobular pattern of dilated vessels. [8] It has been proposed that the PG probably results from an underlying arteriovenous anastamosis frequently associated with the PWS. [8] Though, there was no clinically appreciable pyogenic granuloma like lesion, the distinct histological features seen in our patient might be attributed to a similar pathogenetic mechanism.

As regards therapy, it includes treatment with krypton, argon, argon-pumped, tunable dye, neodymium: Yttrium-aluminum garnet, copper vapor, and pulsed dye lasers. Pulsed dye laser is usually preferred because of minimal risk of scarring. Acquired PWS tends to have a quicker and better response to pulsed dye therapy than congenital PWS. [9]

The case report demonstrates a rare occurrence of a vascular malformation in an adult patient. This is the first report of an acquired PWS from India. Although no definite cause could be appreciated for the development of lesion in our patient, further reports and future insights into the pathogenesis of such cases would broaden our knowledge regarding this infrequently reported phenomenon.[15]

   References Top

Karvnnen SL, Vaajalahti P, Marenk M, Janas M, Kuokkanen K. Birthmarks in 4346 Finnish newboms. Acta Derm Venereol 1992;72:55-7.  Back to cited text no. 1
Salim A, Kurwa H, Turner R. Acquired port-wine stain associated with glaucoma. Clin Exp Dermatol 2003;28:230-1.  Back to cited text no. 2
Smoiler BR, Rosen S. Port-wine stains. A disease of altered neural modulation of blood vessels? Arch Dermatol 1986;122:177-9.  Back to cited text no. 3
Senti G, Trüeb RM. Acquired naevus flammeus (Fegeler syndrome). Vasa 2000;29:225-8.  Back to cited text no. 4
Adams BB, Lucky AW. Acquired port-wine stains and antecedent trauma: Case report and review of the literature. Arch Dermatol 2000;136:897-9.  Back to cited text no. 5
Hoque S, Holden C. Acquired port wine stain following oral isotretinoin. Clin Exp Dermatol 2005;30:587-8.  Back to cited text no. 6
Rose RF, Merchant W, Wilkinson SM. Acquired port-wine stain occurring in association with a congenital lesion. Clin Exp Dermatol 2011;36:621-3.  Back to cited text no. 7
Castanedo-Cazares JP, Lepe V, Moncada B. Pyogenic granuloma within port-wine stains. Eur J Dermatol 2002;12:616.  Back to cited text no. 8
Lanigan SW. Acquired port wine stains: clinical and psychological assessment and response to pulsed dye laser therapy. Br J Dermatol 1997;137:86-90.  Back to cited text no. 9
Johnson SC, Hanke CW. Unilateral acquired nevus flammeus in women. Cutis 2001;67:225-8.  Back to cited text no. 10
Piaserico S, Belloni Fortina Posttraumatic port-wine stain in a 4-year-old girl: Fegeler syndrome. Pediatr Dermatol 2004;21:131-3.  Back to cited text no. 11
Kulac M, Karaca S, Acar M, Albayrak R, Songur A. Acquired port-wine stain related to acoustic neuroma. Clin Exp Dermatol 2006;31:30-2.  Back to cited text no. 12
Afsar FS, Ortac R. Acquired port-wine stain in a pediatric patient. J Cutan Med Surg 2006;10:151-3.  Back to cited text no. 13
Vagefi MR, Florell SR, Lin CC, McCann JD, Anderson RL. Acquired port wine stain of the upper eyelid after cluster headache. Ophthal Plast Reconstr Surg 2008;24:490-2.  Back to cited text no. 14
Kirkland CR, Mutasim DF. Acquired port-wine stain following repetitive trauma. J Am Acad Dermatol 2011;65:462-3.  Back to cited text no. 15

What is new?
This is the first report of an acquired port-wine stain from India and previous reports of such cases have also been reviewed.


  [Figure 1], [Figure 2], [Figure 3]

  [Table 1]


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