Diagnosis and Classification
Scars may be normotrophic, atrophic, hypertrophic, or keloidal. Both hypertrophic and keloidal scars are abnormal responses to tissue injury. Hypertrophic scars mature and flatten over time, usually after 6 months. The keloid appears as a shiny, smooth, raised proliferation of scar tissue with typical crablike extensions beyond the site of the original injury [see Figure 21]. Keloids differ from hypertrophic scars in that their development is delayed, sometimes occurring months after tissue injury. Keloids do not regress, and they frequently cause pain, itching, and burning. Keloids are more common in African Americans, Hispanics, and persons with a personal or family history of keloids. Other factors associated with the development of keloids include wound tension, especially in skin sites such as the chest, shoulders, and back; ear piercing; healing by second intention; pregnancy; young age; and deep laceration.30
In atrophic scars, there is thinning of the skin and loss of normal architecture. Striae distensae, a so-called stretch mark, is a common dermal atrophic scar that tends to appear during periods of rapid weight gain and in the presence of excess glucocor-ticoid, as well as late in gestation.
Treatment
Treatment with intralesional steroids, 10 to 40 mg/ml once a month for up to 6 months, can effectively flatten keloid and hy-pertrophic scars. Cryotherapy (a 30-second application once a month for 3 months) has been found to be safe and effective.31 Topical silicone gel sheeting, which was first used for burn scars, has been used in the treatment of keloids and hyper-trophic scars.32 There is no release of silicone into the skin, and there are no adverse side effects from this treatment. The mechanism of action is unknown. Potential side effects of intralesion-al corticosteroid treatment include atrophy, depigmentation, telangiectasia, and ulceration and dose-related systemic effects.
Vascular Birthmarks
Vascular proliferations are broadly classified as hyperplasias that show a tendency to regress or as benign vascular tumors that persist.33,34 Vascular hyperplasias include pyogenic granulo-ma and pseudo-Kaposi sarcoma. Vascular hemangiomas can be further subdivided according to their histologic cell of origin (endothelial cell, pericyte, glomus cell), depth of tissue involvement (superficial or deep), and size of involved vessels (capillaries, venules, arterioles, veins, or arteries). Vascular birthmarks such as nevus flammeus and salmon patch may resemble an-giomas but are nonproliferative malformations that usually do not involute.
Epidemiology
Hemangiomas (see below) occur in a female-to-male ratio of 5:1, whereas vascular malformations occur with equal frequency in males and females. A rare familial occurrence of heman-giomas, vascular malformations, or both has been reported in six kindreds, suggesting autosomal dominant inheritance in these cases.
Figure 21 The proliferation of scar tissue in a keloid may extend beyond the original site of injury.
Vascular malformations are congenital developmental defects that are generally of unknown etiology. Port-wine stains may result from progressive ectasia of the superficial vascular plexus in the skin as a result of abnormal neural regulation of blood flow.36 In the Klippel-Trenaunay-Weber syndrome, a mesodermal abnormality affecting differentiation of the limb bud may occur during the third to sixth week of gestation.37
Pathogenesis
The etiopathogenesis of hemangiomas and vascular malformations is not well understood. Hemangiomas arise in response to an angiogenic stimulus that may begin in utero. Through use of immunohistochemical techniques, infantile he-mangiomas and placental microvessels were found to coex-press the vascular antigens GLUT-1 and Lewis Y antigen (LeY).38 These antigens are not present in other vascular tumors, such as pyogenic granulomas, or in vascular malformations. A pathogenic link involving aberrant differentiation of vascular precursor cells or embolization of placental cells to fetal tissue has been hypothesized.38 These antigens are also absent in congenital nonprogressive hemangioma, a distinctive hemangioma consisting of lesions that are fully formed at birth and that either remain static or rapidly involute.39
Overview of management
Evaluation and management of hemangiomas and malformations require a multidisciplinary approach. Specific diagnosis may be aided by imaging techniques such as CT and MRI to assess depth of involvement and extension to adjacent structures and to evaluate associated abnormalities. Laboratory evaluation for associated systemic disease may be required in addition to ophthalmologic, neurologic, and cardiologic assessment for complications of vascular tumors and dysmorphic syndromes.
Hemangiomas
Hemangiomas are proliferating vascular tumors that are not necessarily present at birth. The vascular lesion may appear in neonates as a faint pink patch that subsequently undergoes rapid proliferation over a period of months to years before the lesion stabilizes and regresses.
Figure 22 The strawberry, or capillary, hemangioma appears between the second and fifth weeks of life and undergoes spontaneous involution over a period of several years.
Figure 23 A nevus flammeus is present at birth as a reddish or violaceous macular discoloration, often in a unilateral and segmental distribution; it shows little tendency to involute later in life.
The biologic classification of hemangiomas is very different from that of vascular malformations. Vascular tumors can be classified according to their cell or origin, the size of the involved vessels, and the depth of involvement. Such classifications have led to refinement in terminology.40 The terms strawberry hemangioma and cavernous hemangioma are descriptive clinical terms that do not specify the type of vessels that are involved.
Diagnosis and Classification
Capillary hemangioma, also known as strawberry heman-gioma, appears as a single vascular lesion or multiple lesions during the second to the fifth week of life. Infantile heman-giomas are bright-red, soft, lobulated tumors that increase in size for a period of months [see Figure 22]. Lesions spontaneously involute, sometimes with fibrosis, over a period of several years.33 Histologically, the capillary hemangioma shows a proliferation of endothelial cells that form many new small vessels.
Treatment
It is important to realize that most hemangiomas are uncomplicated and regress without treatment early in life with minimal residual scarring. Follow-up studies have shown that in 90% of patients, hemangiomas regress by 9 years of age.41 Parents may require considerable reassurance that the best course is to refrain from treatment. Care must be taken to prevent trauma and infection, which may lead to scarring.
There is considerable controversy as to when to intervene in the treatment of complicated hemangiomas because of potential side effects, such as scarring. The ideal time to treat would be at the beginning of the period of rapid growth, but this is difficult to predict. Indications for treatment include involvement of a vital orifice, infection, ulceration, ocular involvement, and severe cosmetic deformity. Medical options include intralesional or systemic steroids, the latter at a dose of 1 to 3 mg/kg/day. Antimetabolites have been used for their antiproliferative effect. Interferon alfa has been used for severe hemangiomatosis, but its use is associated with systemic side effects and the potential risk of spastic diplegia. Laser surgery with 585 nm pulsed dye laser may be used to treat the superficial proliferative compo-nent.41 Radiation therapy may lead to scarring and is discouraged in children because of long-term radiation effects, including risk of malignancy. Interventional techniques involving embolization of vessels may be required in cases involving airway obstruction or other life-threatening complications. A multidis-ciplinary team approach involving the dermatologist, pediatrician, radiologist, surgeon, and other specialists is needed for optimal management of complicated cases.42
Vascular malformations
Vascular malformations are usually present at birth. They are permanent or progress in the form of ectasias but do not proliferate. Vascular malformations may be subdivided into the following groups: venous, lymphatic, combined arteriovenous, and capillary (such as port-wine stain) .43 Dysmorphic syndromes such as Sturge-Weber and Klippel-Trenaunay-Weber syndromes are more commonly associated with vascular malformations than with hemangiomas.
Diagnosis and Classification
Salmon patch The salmon patch, one of the most common vascular birthmarks, is a dull-pink macule that appears on the nape of the neck, central forehead, or eyelids. Although the salmon patch is sometimes classified as a nevus flammeus, it is distinguished from the latter by its tendency to fade in early life. The salmon patch is caused by the persistence of fetal capillary ectasia in the dermis.33
Port-wine stain Port-wine stain, also called nevus flam-meus, appears at birth as a reddish or violaceous macular discoloration, usually in a unilateral, segmental distribution [see Figure 23]. Mature dilated capillaries are present in the dermis. After puberty, nevus flammeus lesions may become thickened and nodular or papular. There is little tendency toward involution. Nevus flammeus lesions may be associated with abnormalities of the larger vessels and with neurologic manifestations.
Sturge-Weber syndrome A facial port-wine stain that involves the skin innervated by the first branch of the trigeminal nerve is a feature of the Sturge-Weber syndrome (also known as encephalotrigeminal angiomatosis). Other features of the Sturge-Weber syndrome include ipsilateral congenital glaucoma and contralateral seizures caused by leptomeningeal angiomatosis.
Figure 24 A spider angioma, which has a central arteriole from which fine vessels radiate, blanches with pressure.
Ophthalmologic and neurologic evaluation may be warranted in patients with the Sturge-Weber syndrome.
Klippel-Trenaunay-Weber syndrome The triad of findings seen in Klippel-Trenaunay-Weber syndrome includes a port-wine stain, usually in a patchy distribution on the involved extremity; varicose veins; and soft tissue or bony hypertrophy. The most common site of involvement is the lower leg; the next most common sites of involvement are the arms and trunk.37
Venous malformation Formerly referred to as cavernous hemangiomas, vascular malformation consists of a collection of abnormal veins and venous pouches that commonly occur around the head and neck but can occur anywhere on the body. They are frequently multiple or have satellite lesions. Superficially, they appear as a subcutaneous swelling with a bluish hue on the skin surface or mucous membrane. Deeper components may be invisible on clinical examination. Lesions enlarge for several months, become stationary for an indefinite period, and spontaneously resolve.
Treatment
Because vascular malformations do not proliferate, treatment may be cosmetic and can be postponed to later in life. However, a multidisciplinary approach is needed to treat potential complications of vascular malformations associated with dysmor-phic syndromes. Salmon patch tends to fade in early life and usually requires no treatment.
Treatment of port-wine stains by excision, tattooing, ionizing radiation, cryosurgery, or dermabrasion is largely unsatisfactory. Use of the argon laser has resulted in lightening of vascular lesions; however, there is wide variability in response. The effectiveness of this treatment results from the selective absorption of the monochromatic 585 nm laser light by red hemoglobin pigment, which produces thermal energy with resultant photocoagulation of tissue.44 Thinner lesions are more responsive than thicker lesions that have undergone progressive vascular ectasia. In a study of 100 patients of different age groups who had port-wine stains of the head and neck and who were treated with a flashlamp pulsed dye laser, treatment was no more effective when given in early childhood than when given at a later date.45
Acquired Vascular Disorders
Diagnosis and classification
Spider Angioma
Spider angioma, also called spider nevus or arterial spider, appears as a central red punctum from which fine vessels radiate; the appearance of the lesion is suggestive of a red spider [see Figure 24]. The central arteriole may be pulsatile. These telangiectasias (dilated capillaries) are commonly seen on the face, neck, trunk, and upper extremities and occur most commonly in middle-aged or elderly persons. They may arise spontaneously or in association with pregnancy or hepatic dysfunction. Spider angiomas may be treated with laser therapy for cosmetic reasons.
Unilateral Telangiectasia
Acquired unilateral telangiectatic nevi are uncommon, but those that have been reported resulted from mechanical or physical trauma, including sun damage.
Cherry Angioma
Cherry angioma, also called senile angioma, appears as multiple bright-red, soft, dome-shaped papules on the trunk of middle-aged or older persons. Trauma produces slight bleeding. Electrodesiccation may be performed for cosmetic purposes.
Pyogenic Granuloma and Other Vascular Tumors
The pyogenic granuloma is a soft red lesion that is solitary, raised, and nonpulsatile; it often appears after minor skin trauma, such as a puncture wound. Other predisposing factors include hormonal effects, infection, viral oncogenes, microscopic arteriovenous anastomoses, and growth factors.47 Epulis gravi-darum is a variant of a pyogenic granuloma. The lesion was formerly believed to be caused by a pyogenic infection of a small wound; histologically, however, an early lesion resembles a capillary hemangioma. The thin, sometimes verrucous epidermis is friable and apt to become eroded or ulcerated. Lesions rapidly reach a size of 1 to 2 cm and then remain static. Common sites of involvement are the fingers, feet, and face [see Figure 25]. Biopsy is performed to rule out malignant tumors, such as Kaposi sarcoma and amelanotic melanoma.
Other benign tumors with a vascular component include angiofibroma, angioleiomyoma, and angiolipoma. Some of these can be painful. Differential diagnosis of painful skin tumors includes glomus tumor, angiolipoma, angioleiomyoma, neuromas, and eccrine spiradenoma.34 Lesions are usually easily removed by electrodesiccation and curettage. If they recur or if satellite lesions appear after such treatment, excisional biopsy is recommended.
Kimura Disease
Kimura disease and angiolymphoid hyperplasia with eosino-philia are rare tumors of unknown cause that occur mainly on the head and neck in young adults and may resemble pyogenic granuloma.48 Kimura disease, which was first reported in Korea, is most common in Asians. It appears as a granulomatous proliferation of lymphoid tissue that may be accompanied by peripheral eosinophilia and contiguous lymphadenopathy. Lesions may occasionally be seen on the trunk, extremities, and genitalia in addition to the head and neck. Angiolymphoid hy-perplasia with eosinophilia, which may or may not represent a different disease, appears as localized single or multiple nodules.
Figure 25 The pyogenic granuloma, which may show a smooth, verrucous, eroded, or friable surface, may be confused with a malignant tumor.
Figure 26 Leiomyomas are sometimes painful papules that arise from smooth muscle of blood vessels or the arrector pili.
Infectious, allergic, hormonal, and traumatic mechanisms have been postulated. Immunodermatopathologic studies suggest an unusual distribution of adhesion molecules, IgE, and CD23 in these angioproliferating tumors.49
Lipoma
The lipoma, which is a soft, rounded to lobulated subcutaneous tumor of mature fat cells, is commonly seen on the trunk, neck, or forearms. Lesions are rubbery in consistency and freely movable under the overlying skin, which appears normal. There may be a single lesion or multiple lesions, and they are usually asymptomatic unless they impinge on a nerve. Lipomas are of variable size and grow slowly. Histologically, the tumors are usually encapsulated and show fat cells that are indistinguishable from normal adipose tissue. Admixture of other tissue components may result in fibrolipomas (fibrous tissue), an-giolipomas (blood vessels), and myolipomas (smooth muscle). Excision may be performed for cosmetic reasons. If a lesion grows rapidly, biopsy should be performed, though lipomas rarely become malignant.
Leiomyoma
The leiomyoma is an uncommon tumor of smooth muscle that appears as a single brownish-red papule or as multiple papules or small nodules, which are sometimes painful [see Figure 26]. Leiomyomas may arise from the arrector pili (the smooth muscle attached to the hair follicle sheath) or from the smooth muscle surrounding cutaneous blood vessels (angio-leiomyoma). Painful lesions can be excised.
Lymphangioma Circumscriptum
Lymphangioma circumscriptum is characterized by groups of persistent localized or diffuse translucent vesicles. Indications for treatment include severe cosmetic problems, persistent leakage of lymphatic fluid or blood, and recurrent infection. The vesicles frequently recur after surgery, radiotherapy, electro-cautery, or cryosurgery because of the persistence of deep lymphatic cisterns. Carbon dioxide laser in a vaporization mode has been used to ablate superficial cutaneous lesions in patients with lymphangioma circumscriptum.50 The major advantage of this technique is that it may reduce the frequency of recurrences because it seals the communicating channels to the deeper cisterns by vaporizing the superficial lymphatics.
