Copyright 2000 American Medical Association. All Rights Reserved. Applicable FARS/DFARS Restrictions Apply to Government Use.2000
While cases of congenital port-wine stains (PWSs) are relatively common, cases of acquired PWSs are quite rare. Many of the reported cases of the acquired type have been reported to be related to previous trauma.
We encountered a case of acquired PWSs in a 3-year-old girl. Her parents noted that the lesions appeared in areas of antecedent trauma. This prompted us to review all reported cases of acquired PWSs and to assess the relationship to trauma. Among the 59 cases reported, there was a slight female predominance (female-male ratio, 35:24). Seventeen (29%) of the cases were related to trauma. One report evaluated the effectiveness of lasers in the treatment of acquired PWSs and found that 54% of patients treated with pulsed dye lasers had an excellent response or complete clearance.
Port-wine stains are not only congenital but can be acquired as well. Trauma can be one of the causes of acquired PWSs. To explain this phenomenon, various theories, including abnormal vascular repair and altered vascular innervation, have been proposed. Lasers are the treatment of choice in all cases of PWSs and may be more effective in those that are acquired.
VASCULAR LESIONS in neonates are relatively common, occurring in 44% of the newborn population.1 Approximately 1.4% of the lesions are congenital port-wine stains (PWSs).1 Port-wine stains are vascular malformations with normal endothelial cell cycles, in contrast to hemangiomas, which represent vascular tumors with endothelial cell hyperproliferation.2
Multiple articles report cases of an acquired form of PWSs. These lesions are morphologically indistinguishable from their congenital counterparts. Cases of acquired PWSs are rare. They have been reported to occur after trauma to the skin3- 10 as well as without antecedent trauma.4,6,9- 17 We describe a 3-year-old girl who presented with multiple acquired PWSs following minor trauma to the skin. Her case prompted us to review the literature concerning the pathogenesis and therapy of acquired PWSs.
A 3-year-old white girl presented at the age of 2 years with a history of numerous red to violaceous macules and patches erupting on her face. Her parents noted that the lesions seemed to be related to multiple, discrete events of mild trauma, including minor falls and other typical pediatric injuries, that caused ecchymoses and modest edema. When the patient was 3 years old, her parents noted no change in the facial lesions but did report that additional, similar lesions had developed on her right lower extremity. They again suggested that these new lesions were in areas of previous trauma.
On examination, she had numerous, well-defined, 4-mm to 5-cm, erythematous to violaceous, blanching macules and patches with a few telangiectasias located on both legs and on the right eyebrow, right preauricular area, right knee, and left arm. She had no systemic complaints and was otherwise healthy. Her parents had multiple photographs that showed that the lesions were not present at birth.
Based on the historical information and the typical clinical presentation, no biopsy was performed, and a diagnosis of trauma-induced acquired PWSs was made. Laser therapy was discussed with the parents of the child, but they did not wish any treatment for the lesions at that time.
At birth, PWSs are well-defined, pink, purple, or red macules and patches. These vascular malformations tend to grow with the child, darken, and become more irregularly surfaced in adult life. Unlike hemangiomas, PWSs persist throughout life.18 They are generally found unilaterally on the face and neck, but can occur anywhere on the body.18 Some authors report that nearly 50% of all facial PWSs are located in the distribution of the trigeminal nerve.19 These lesions are often isolated findings but also may be associated with other congenital abnormalities, such as Sturge-Weber syndrome, in which patients also have seizures, glaucoma, abnormal cerebral vasculature, and mental retardation. The differential diagnoses of PWSs include arteriovenous malformations and cutis marmorata telangiectatica congenita. Arteriovenous malformations may first present in childhood after trauma; however, they are generally localized.
Histopathologically, PWSs are characterized by capillaries that have multiple ectasias and dilatations. With time, both dilatations of vessels and ectasias increase. These vessels are generally located in the middle to superficial dermis, which is composed of loosely arranged collagen.20 The histopathological features of acquired and congenital PWSs are identical.
While the exact mechanism of the formation of these lesions remains unknown, some studies have implicated alterations in the surrounding supportive dermal structure, without vessel wall abnormalities.21 Some authors suggest that alterations in capillary neural tone play a role in the vessel changes. Smoller and Rosen22 compared nerve densities in normal skin and PWS lesional skin and found significantly fewer nerves in the skin with a PWS: only 17% of the vessels in a PWS lesion were associated with nerves, whereas 75% of all vessels within normal skin had associated neural connections. They suggested that this discrepancy in density leads to altered neural modulation of vascular tone, thereby ultimately affecting the orientation and vasoactive properties of the vessels.
Rosen and Smoller23 later theorized that both acquired and congenital PWSs were the result of malformed sympathetic innervation. In the case of congenital PWS, there was a maturational defect in the local sympathetic nervous system, whereas loss of sympathetic innervation, possibly through trauma, could lead to acquired PWSs. It has also been shown that the vessels within a PWS do not react normally to vasoactive stimuli, further supporting the role of altered autonomic innervation.24 In an attempt to explain the phenomenon of acquired PWSs, it was proposed that trauma causes perivascular atrophy, which ultimately leads to vessel dilatation.8 Alternatively, the reparative process in vessels, after trauma, may proceed abnormally, resulting in dilated vessel walls.8
Multiple reports suggest that trauma,3- 10 including sports-related injuries,7- 9 plays a role in the pathogenesis of acquired PWSs. Table 1 lists previously reported cases of acquired PWSs and their relationship to trauma. Many patients described with acquired PWSs were adults. In fact, Dinehart et al9 reported that onset occurred at 16 years of age or younger in only 4 of 10 patients. The remaining 6 patients were older than 23 years (mean age, 28 years) when they first noticed their vascular lesions. In a review of the 59 reported cases of acquired PWS, the mean±SD age was 24±16 years, with a slight female predominance (female-male ratio, 35:24). Seventeen (29%) of these cases were related to trauma. Dinehart et al9 found that 1 of their patients had a PWS that was caused by sports-related trauma to the eyelid. In another sports-related injury, a 46-year-old man sustained a hard cricket ball strike to his face that resulted in significant soft tissue damage.7 Tsuji and Sawabe8 also described 3 patients with acquired PWSs caused secondarily by trauma. Two patients had previous trauma to fingers, while 1 had a left leg injury. In 1 of the cases occurring on the finger, there was a gradual enlargement of a violaceous patch over that same area during a 10-year period. Subsequent biopsy findings confirmed that the lesion was a PWS.
One might propose that some cases of acquired PWSs, especially in children, simply represent imperceptible lesions that slowly enlarge, resulting in the PWS. The antecedent trauma may then either draw one's attention to the area or in fact play a role in increasing its rate of growth, thereby alerting the patient to its presence.
There have, however, also been multiple reported cases in which no antecedent trauma was identifiable4,6,9- 17 (Table 1). Cobb and Goldman14 reported a case of acquired PWSs that had developed 8 years earlier, without antecedent trauma, in a 41-year-old man. Dinehart et al9 found that of their 10 patients with acquired PWSs, only 1 had a history of trauma to the affected area. Similarly, Pasyk16 reported acquired PWSs in 3 adult patients with no documented trauma preceding the development of the vascular lesions. Traub11 also was unable to discover any preceding event that could have led to the development of the PWS. Others, who have not reported trauma, have suggested that the use of oral contraceptive pills was related to the development of acquired PWSs.13 It has even been suggested that chronic actinic exposure may play a role in the development of PWSs.16
In the case reports of acquired PWSs, only a few of the authors discussed treatment.9,10,16 Lasers were the most frequently used treatment. Only 2 reports investigated the efficacy of laser therapy in acquired PWSs.9,10 Relatively recent advances in laser technology have allowed successful treatment of PWSs with fewer adverse effects, such as scarring. Both copper vapor and pulsed dye lasers have been used. Dinehart and colleagues treated 4 of their 10 patients with acquired PWSs with the copper vapor laser, with "poor" results in 2 patients and an "excellent" response in the other 2 patients. One of the patients with a poor result also was treated with a pulsed dye laser. The authors treated 3 patients with pulsed dye laser alone and achieved excellent responses. One patient did not receive treatment because of the "color of skin." Another patient had a pulsed dye laser test site but failed to return for follow-up.9 A larger study, involving 13 patients with acquired PWSs, used between 2 and 11 treatments with the pulsed dye laser. Complete clearance was achieved in 6 (46%) of the 13 patients treated. Excellent results were noted in 2 other patients, and the remaining 5 patients had good, fair, and poor results. One of these patients developed hypopigmentation and another did not tolerate the therapy. Comparing the response to treatment in patients with congenital and acquired PWSs, one author noted that fewer treatments were required in patients with acquired PWSs.10
Acquired PWSs can occur in sites of trauma. Further work is required to determine if dilated, ectatic vessels result from dermal collagen atrophy or from a primary alteration in the superficial cutaneous vasculature. Lasers continue to be the most important modality in the treatment of PWSs.
Accepted for publication January 31, 2000.
We gratefully acknowledge Kirsten Lyn Hamacher, MD, who graciously translated all relevant information found in the non–English language literature reviewed for this article.
Reprints: Anne W. Lucky, MD, 7691 Five Mile Rd, Cincinnati, OH 45230.
Adams BB, Lucky AW. Acquired Port-wine Stains and Antecedent TraumaCase Report and Review of the Literature. Arch Dermatol. 2000;136(7):897-899. doi:10.1001/archderm.136.7.897