eMedicine Specialties > Dermatology > Diseases of the Vessels

Capillary Malformation

Author: Richard J Antaya, MD, Director of Pediatric Dermatology, Associate Professor, Departments of Dermatology and Pediatrics, Yale University
Contributor Information and Disclosures

Updated: Sep 25, 2009

Introduction

Background

Capillary malformation, usually referred to as a port-wine stain or nevus flammeus, is the most common type of vascular malformation. As a congenital malformation of the superficial dermal blood vessels, capillary malformation is present at birth and grows in size commensurate with the child; capillary malformations remain present for life and have no tendency toward involution. A rare form of capillary malformation that is not present at birth is referred to as an acquired capillary malformation; however, this article focuses on the more common congenital lesion.

Past nosology of this lesion has resulted in much confusion, and an excessive number of descriptive terms have been applied to it. Confusion originated from difficulty in differentiating vascular malformations from vascular proliferative lesions, such as hemangiomas, and from the use of wholly clinical descriptions in categorizing these lesions. Vascular malformations are categorized by predominate vessel type, such as arterial, venous, lymphatic, capillary, or complex (a combination of different vessels).

Although some capillary malformations may be associated with other vessel malformations, most occur alone as venulocapillary malformations. In 2008, Happle argued that the term capillary malformation should be used as a more generalized designation for several congenital disorders of dilated capillaries (eg, angiokeratomas, nevus anemicus, cutis marmorata telangiectatica congenita).1 While this notion has merit, the accepted nomenclature is that capillary malformation be reserved for a patch of red-colored skin, historically referred to as a port-wine stain or a nevus flammeus.

Pathophysiology

Capillary malformations and other vascular malformations are the result of abnormal morphogenesis. Capillary malformations are characterized by ectatic papillary dermal capillaries and postcapillary venules in the upper reticular dermis, with some evidence of increased vessel density and no apparent proliferation of vessels. These ectatic vessels are lined by flat, benign-appearing endothelial cells, similar to the vessels of normal skin, with similar staining characteristics for endothelial antigens, including fibronectin, von Willebrand factor, and collagenous basement membrane proteins. The endothelial cells also exhibit cell turnover similar to normal vessels, supported by a paucity of mitoses or an uptake of tritiated thymidine. One study demonstrated a mean vessel depth of 0.46 mm in capillary malformations, suggesting that most of the vessels are superficial.

Evidence supports a neural role in both the development and progression of capillary malformations. Animal studies show that the sympathetic nervous system influences the composition and functional properties of the vessel wall during development.

Immunohistochemical studies of capillary malformations reveal a significantly decreased density of perivascular nervous tissue in lesional skin, suggesting that inadequate innervation may be responsible for decreased vascular tone and progressive vascular dilatation.2 Confocal microscopic studies demonstrate an inverse correlation between nerve density and blood vessel diameter and evidence that capillary malformations with the lowest nerve density exhibit the highest blood vessel density and the poorest response to laser intervention.3 The finding of increased vessel diameter and/or decreased nerve density may be secondary to other factors, such as local cytokine production or abnormal receptors; however, this has not been elucidated.

The potent endothelial cell mitogen vascular endothelial growth factor (VEGF)–A and its most active receptor VEGF-R2 expression are significantly increased in capillary malformation skin tissue compared with control skin.4 This may suggest that VEGF and VEGF-R could contribute to the pathogenesis of capillary malformations by inducing vessel proliferation and/or vasodilatation. If this is indeed a pathogenic factor, antiangiogenic treatments using VEGF blocking agents may prove to be useful for capillary malformations. Conversely, one report describes expansion of a biopsy-proven capillary malformation following partial surgical excision in an adult in whom the newly expanded capillary malformation expressed marked elevations of both tyrosine kinase receptor (Tie2) and its ligand angiopoietin-1 and no increase in VEGF.5 Tie2 and angiopoietin-1 are known regulators of vascular remodeling during angiogenesis, mutations of which have been demonstrated in familial venous malformations.

An inactivating mutation of RASA1 on 5q has been detected in some kindreds with multiple, small, round-to-oval, pink capillary malformations.6 RASA1 encodes a GTPase-activating protein, which negatively regulates Ras activity. These kindreds all had members who also had arteriovenous malformations (AVMs) or arteriovenous fistulae (AVFs). This disease has been coined capillary malformation-AVM syndrome to denote the 2 types of vascular malformations observed in these kindreds. One kindred with a novel RASA1 -inactivating mutation included a member with a large lower extremity capillary malformation with associated ipsilateral limb enlargement7 ; however, another large kindred has been identified that has only capillary malformations and no evidence of AVMs or AVFs, suggesting that RASA1 mutations may be implicated more often than previously believed.8

Use of transcutaneous videomicroscopy and handheld dermoscopy reveals 2 distinct patterns of vascular ectasia in capillary malformations. The type 1 abnormality is composed of superficial, tortuous, dilated end-capillary loops in the superficial papillary dermis. The type 2 abnormality consists of dilated, ectatic vessels in the superficial horizontal vascular plexus. Some patients exhibit a combination of both abnormal patterns. Evidence suggests that the type 1 abnormality has a better response to 585-nm flashlamp-pumped pulsed-dye laser (PDL) therapy than the type 2 abnormality.9 Moreover, one study found a correlation between the depth and pattern of the capillary malformations and the location of the lesions, demonstrating improved responses to laser therapy with locations demonstrating a type 1 pattern (V3 region of the face, neck, and trunk).

Frequency

United States

Capillary malformation occurs in 0.3-0.5% of newborns.

International

Worldwide, capillary malformation occurs in 0.1-2% of newborns.

Mortality/Morbidity

  • Isolated capillary malformations do not appear to cause an increase in mortality; however, psychosocial disability secondary to facial disfigurement can be overwhelming. Several studies demonstrate that patients with facial capillary malformations exhibit greater self-concern, ruminative self-doubt in interpersonal interactions, social inhibition, isolated and passive orientation in interpersonal relationships, stigmatization from society, and limitations of privileges and opportunities otherwise afforded to those without facial disfigurement. One study demonstrated that the psychosocial difficulties not only persisted but actually worsened in adulthood.10
  • Development of lobulated capillary hemangiomas (pyogenic granulomas) overlying capillary malformations often results in bleeding.11 The destruction of these lesions usually results in minor scarring of the skin.
  • Any morbidity involved with capillary malformations is associated with more extensive vascular malformations.

Race

According to at least one survey, capillary malformations are more common in whites than in African Americans.12

Sex

The sex distribution of capillary malformations is equal.

Age

Capillary malformations are present at birth. Some lesions may not be readily observed at birth because of anemia or plethora. In certain lesions, some lightening of the lesions may occur during the first year of life; however, beyond that time, further lightening is generally not observed.

The very rare acquired port-wine stain can occur at any age after birth and is identical to congenital capillary malformations both clinically and histologically. The etiology of these lesions is unknown and most are idiopathic; however, trauma, chronic UV exposure, hormonal influences, infections, solid brain tumor, and various internal vascular disorders have been implicated.13

Clinical

History

Nearly all cases of capillary malformation can be diagnosed by taking a careful history and performing a physical examination.

  • Onset: Capillary malformations are always present at birth, but they may not be apparent early in life because of neonatal anemia or plethora.
  • Location
    • Of capillary malformations, most involve the head and the neck.
    • Of facial capillary malformations, 45% are more or less restricted to 1 of the 3 areas supplied by the divisions of the fifth cranial nerve (CN).
    • Of facial capillary malformations, 55% involve an area innervated by more than 1 division of the fifth CN, crossing the midline or occurring bilaterally.
  • Growth
    • Growth of the capillary malformation is commensurate with that of the child.
    • Capillary malformations remain present for life.
    • Capillary malformations show no tendency toward involution.
  • Evolution
    • Capillary malformations may change from pink in infancy to red in early adulthood to deep purple during middle age in some individuals.
    • The surface may become thickened with a cobblestonelike contour. Approximately 65% of facial capillary malformations develop these changes during adulthood.14
    • Nodular vascular lesions may develop, usually in adulthood.
    • Pyogenic granulomas (lobular capillary hemangiomas) with bleeding may develop in capillary malformations, even in childhood.
  • Associations: Capillary malformation may coexist with other vascular malformations. Geographic (ie, well-circumscribed, sharply bordered) cutaneous lesions carry a much higher probability of associated lymphatic malformations than blotchy stains, especially in patients with Klippel-Trenaunay syndrome.15

Physical

Early in life, the lesions appear as flat (macular), mostly well-circumscribed patches. The color varies from pink to red to purple. The color of the capillary malformation does not correlate with the capillary depth or diameter. Blanching with external pressure is variable. In infancy and childhood, the color darkens with crying, fever, or overheating. Capillary malformations are usually unilateral with fairly sharp midline cutoffs. The face is the most frequently affected site, followed by the upper part of the trunk.

Later in life, as the vasculature dilates, the capillary malformation may evolve into a raised, thickened plaque. The capillary malformation becomes deep-red to purple. Lesions may become studded with vascular papules, imparting a cobblestonelike appearance. Vascular papules often form and may be prone to bleeding. Skin and underlying soft tissue or bony hypertrophy may be present. Lobulated capillary hemangiomas (pyogenic granulomas) may form, especially with intraoral lesions.

Associated findings

Ocular and/or CNS involvement occurs in 9.5% of patients with facial capillary malformations. Involvement of V1 distribution seems to be a requirement. The highest incidence appears to be in patients in whom the capillary malformation involves the entire cutaneous distribution of V1, with 78% developing eye or CNS complications.16

Glaucoma occurs in approximately 10% of patients with facial capillary malformations, and no leptomeningeal involvement is present. Glaucoma affects 27-45% of patients when capillary malformations involve the skin supplied by both the ophthalmic (CN V1) and the maxillary (CN V2) divisions of the fifth CN, the trigeminal nerve. Glaucoma is less frequent when the face is involved in only 1 of these upper divisions of the trigeminal nerve or if it is affected solely below the eye; however, the prevalence is increased with eyelid involvement. The prevalence may not be correlated with increased vascularity of the choroid or the bulbar conjunctiva. Glaucoma may be due to increased episcleral venous pressure with resultant elevated intraocular pressure, and it can occur without leptomeningeal involvement (eg, in the absence of Sturge-Weber syndrome).

Other types of vascular malformations (venous, lymphatic, arterial, or mixed) may be present.

Associated syndromes

Capillary malformation is a cutaneous finding of several syndromes.

Sturge-Weber syndrome

Sturge-Weber syndrome (encephalofacial or encephalotrigeminal angiomatosis) is characterized by the triad of capillary malformations involving the upper facial dermis, the ipsilateral leptomeninges, and the ipsilateral cerebral cortex. Some authorities believe that only 2 features are necessary to make this diagnosis. The facial skin supplied by the ophthalmic branch (CN V1) of the trigeminal nerve must be involved with the capillary malformation for a patient to meet one of the criteria for Sturge-Weber syndrome.

Sturge-Weber syndrome occurs in less than 10% of patients with capillary malformations on the upper eyelid or the forehead. Involvement of areas on the face supplied by only CN V2 or CN V3 does not carry an increased risk for Sturge-Weber syndrome.

No relationship is apparent between the size of the facial capillary malformation and the severity of CNS involvement, and a small percentage of Sturge-Weber syndrome patients lack any cutaneous involvement.17

Typically, capillary malformations associated with Sturge-Weber syndrome are more extensive than isolated capillary malformations, and patients often have bilateral facial involvement. Complications include glaucoma, seizures, hemiplegia, mental retardation, cerebral calcifications, subdural hemorrhage, and an increased prevalence of underlying soft tissue hypertrophy. Large variability exists in the severity of associated symptoms.

Klippel-Trenaunay syndrome

Klippel-Trenaunay syndrome (angio-osteohypertrophy syndrome) manifests as a triad of capillary malformation, congenital varicose veins, and hypertrophy of underlying tissues, particularly skeletal overgrowth. The sex distribution is equal. The lower limbs are involved in 95% of patients, and involvement is unilateral in 85%. Most patients are asymptomatic at birth, but many experience problems later in childhood. Complications include varicose veins with venous thrombosis and pulmonary embolism; bleeding from varices, the rectum, or the bladder; skin ulceration; increased sweating overlying the capillary malformation; leg circumference or length discrepancy with resultant scoliosis; edema; and recurrent infections.18 Ultrasonographic measurement of the thigh arterial blood flow may be helpful in predicting future leg length discrepancies in patients with lower extremity capillary malformations.19

Parkes-Weber syndrome

With Parkes-Weber syndrome, the diagnostic criteria include an AVM in addition to those listed above for Klippel-Trenaunay syndrome. AVFs are usually diffuse and difficult to ablate. Almost all patients present in childhood with an enlarged, warm extremity. The prognosis is worse than that associated with Klippel-Trenaunay syndrome. A positive bradycardic reaction (Nicoladoni-Branham sign) portends a poorer prognosis. This test is performed by occluding the arterial inflow by compression with a blood pressure cuff. In a limb with a hemodynamically significant AVM, this maneuver leads to reflex bradycardia secondary to a sharp rise in blood pressure. Complications include ulceration and severe lymphedema.

Cobb syndrome

In Cobb syndrome (cutaneomeningospinal angiomatosis), a cutaneous vascular lesion in the skin overlying the spine, is associated with vascular malformations (venous or arteriovenous) in the subjacent spinal meninges. Possible complications result from neurologic damage caused by mass effect on the spinal cord or nerves, bone erosion, and subarachnoid hemorrhage.

Wyburn-Mason syndrome

Wyburn-Mason syndrome (unilateral retinocephalic syndrome), also known as Bonnet-Dechaume-Blanc syndrome, manifests as facial capillary malformations associated with unilateral AVM of the retina and the intracranial optic pathway. Physical findings include monocular amblyopia, mild proptosis, and dilatation of conjunctival vessels. Capillary malformations may occur anywhere on the ipsilateral face (not just the eyelids or periorbitally), and they may have associated facial hypertrophy or occasional involvement of the optic chiasm, the hypothalamus, the midbrain, and the basal ganglia, with associated mental retardation or neurologic signs and symptoms.

Macrocephaly-capillary malformation

Macrocephaly-capillary malformation, previously named macrocephaly-cutis marmorata telangiectatica congenita, is a multisystemic disorder characterized by prenatal overgrowth, somatic and cerebral asymmetry, megalencephaly, characteristic facial features, abnormal mentation, lax joints, thickened and doughy-feeling subcutaneous tissue, syndactyly or polydactyly, and capillary malformation.20

Nevus vascularis mixtus

Nevus vascularis mixtus is the name given when a capillary malformation is paired with nevus anemicus, an example of didymosis or twin spotting.

Associated skeletal or neurologic anomalies

Capillary malformation overlying the lumbar spine may be a marker for an underlying primary skeletal or neurologic anomaly, such as spinal dysraphism, tethered spinal cord, lipomeningocele, or diastematomyelia. The prevalence of underlying defects is increased when multiple abnormalities are present in the lumbar skin. Skin markers include acrochordons (skin tags), an abnormal tuft of hair (fawn's tail), lipomas, an irregular (usually deviated) gluteal cleft, or a dermal sinus tract or sacral dimple that is large or superior to the gluteal fold.

Guggisberg et al found that none of 16 patients with an isolated capillary malformation showed occult spinal dysraphism, whereas 7 of 10 patients with capillary malformations in combination with other lumbar congenital anomalies did have an occult spinal dysraphism.21 Conversely, Tubbs et al found that 21 (17.5%) of 120 patients with an isolated capillary malformation harbored an occult spinal dysraphism, and they recommended MRI for all patients who present with an isolated lumbar capillary malformation.22

Associated dermatologic anomalies

Phakomatosis pigmentovascularis

Phakomatosis pigmentovascularis refers to the presence of a capillary malformation with a melanocytic or other type of nevus. This phenotype is another example of twin spotting, with the pathogenesis hypothesized to include developmental abnormalities, perhaps paired mutations of varied neural crest–derived elements, such as vasomotor nerves and melanocytes. The histopathologic findings of a capillary malformation in phakomatosis pigmentovascularis are the same as those for isolated capillary malformations.

Four types of phakomatosis pigmentovascularis are described.

  • Type I is composed of capillary malformation and nevus pigmentosus et verrucosus or epidermal nevus.
  • Type II is a capillary malformation and dermal melanocytosis with or without nevus anemicus; this is the most common type. It also includes nevus of Ota (oculocutaneous melanosis) and can be associated with Sturge-Weber syndrome and Klippel-Trenaunay syndrome.
  • Type III is a capillary malformation and nevus spilus with or without nevus anemicus.
  • Type IV is a capillary malformation, nevus spilus, and dermal melanocytosis with or without nevus anemicus.

Subdivisions of each type include subtype a for cutaneous involvement only and subtype b for cutaneous and systemic involvement. No systemic involvement is reported for type I.

In 2005, another classification scheme for phakomatosis pigmentovascularis has been proposed by Happle. This includes 3 different distinct categories based on the type of associated lesion: phacomatosis cesioflammea (capillary malformation with bluish gray spots as observed with various lesions of dermal melanocytosis), phacomatosis spilorosea (pale pink telangiectatic capillary malformation associated with a nevus spilus), and phacomatosis cesiomarmorata (cutis marmorata telangiectatica congenita with blue spots). A final category includes others that cannot be included in one of the other 3 variants.23

Angiolipomas

Asymptomatic, noninfiltrating angiolipomas may be present underlying a Capillary malformation in a small minority of patients. These are mostly found on the trunk and pelvic girdle skin and may be associated with laser-resistant capillary malformations.24

Causes

The exact mechanism remains unknown. Evidence for genetic influence is lacking. Postzygotic somatic mutations may account for the mosaic and twin-spotting phenotypes.25 Capillary malformations may result from a neural deficiency of sympathetic innervation of the superficial dermal blood vessels.2

More on Capillary Malformation

Overview: Capillary Malformation
Differential Diagnoses & Workup: Capillary Malformation
Treatment & Medication: Capillary Malformation
Follow-up: Capillary Malformation
Multimedia: Capillary Malformation
References
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  54. Waner M, Suen JY. The treatment of vascular malformations. In: Hemangiomas and Vascular Malformations of the Head and Neck. New York, NY: John Wiley & Sons; 1999:353-66.

  55. Waner M, Suen JY. The natural history of vascular malformations. In: Hemangiomas and Vascular Malformations of the Head and Neck. New York, NY: John Wiley & Sons; 1999:52-62.

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Further Reading

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Keywords

capillary malformation, nevus flammeus, naeuvus flammeus, NF, port-wine stain, port-wine mark, portwine stain, port wine stain, strawberry patch, naevus maternus, venular malformation, venulocapillary malformation, vascular malformation (not capillary hemangioma), birthmark, birth mark

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Contributor Information and Disclosures

Author

Richard J Antaya, MD, Director of Pediatric Dermatology, Associate Professor, Departments of Dermatology and Pediatrics, Yale University
Richard J Antaya, MD is a member of the following medical societies: American Academy of Dermatology, American Academy of Pediatrics, and Society for Pediatric Dermatology
Disclosure: Nothing to disclose.

Medical Editor

Mark W Cobb, MD, Consulting Staff, WNC Dermatological Associates
Mark W Cobb, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, and American Society of Dermatopathology
Disclosure: Nothing to disclose.

Pharmacy Editor

Richard P Vinson, MD, Assistant Clinical Professor, Department of Dermatology, Texas Tech University School of Medicine; Consulting Staff, Mountain View Dermatology, PA
Richard P Vinson, MD is a member of the following medical societies: American Academy of Dermatology, Association of Military Dermatologists, Texas Dermatological Society, and Texas Medical Association
Disclosure: Nothing to disclose.

Managing Editor

Van Perry, MD, Assistant Professor, Department of Medicine, Division of Dermatology, University of Texas Health Science Center
Van Perry, MD is a member of the following medical societies: American Academy of Dermatology and American Society for Laser Medicine and Surgery
Disclosure: Nothing to disclose.

CME Editor

Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University
Catherine Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology
Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

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