V.5 Vascular Lesions

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V.5.1 Introduction

The biological classification of vascular lesions into two major categories as tumors and malfor- mations [19] forms a framework for conceiving them in a coherent manner, although evidence for their association has been reported in a small minority [9]. Among them, the lesions that simulate pigmented skin tumors, especially the melanoma, are discussed in this chapter. Some of these fall into the category of tumors (hem- angioma, pyogenic granuloma) and some into malformations (angiokeratoma). In addition, subcorneal and subungual hemorrhage is in- cluded here for diagnostic purposes.

V.5.2 Hemangiomas

Hemangiomas are benign vascular tumors due to the proliferation of blood vessels. The term includes many entities, namely, infantile hemangiomas (localized, segmental, multifocal,

Vascular Lesions

Fezal Özdemir V.5

Contents

V.5.1 Introduction . . . .303

V.5.2 Hemangiomas . . . .303

V.5.2.1 Cherry Hemangioma . . . .304

V.5.2.1.1 Definition . . . .304

V.5.2.1.2 Clinical Features . . . .304

V.5.2.1.3 Dermoscopic Criteria . . . .304

V.5.2.1.4 Relevant Clinical Differential Diagnoses . . . .304

V.5.2.1.5 Histopathology . . . .304

V.5.2.1.6 Management . . . .305

V.5.3 Pyogenic Granuloma . . . .305

V.5.3.1 Definition . . . .305

V.5.3.2 Clinical Features . . . .305

V.5.3.3 Dermoscopic Criteria . . . .305

V.5.3.4 Relevant Clinical Differential Diagnosis . . . .306

V.5.3.5 Histopathology . . . .306

V.5.3.6 Management . . . .307

V.5.4 Angiokeratomas . . . .307

V.5.4.1 Solitary Angiokeratoma . . . .307

V.5.4.1.1 Definition . . . .307

V.5.4.1.6 Management . . . .308

V.5.5 Hemorrhages . . . .308

V.5.5.1 Subungual Hematoma . . . .308

V.5.5.1.1 Definition . . . .308

V.5.5.1.6 Management . . . .308

V.5.5.2 Subcorneal Hematoma . . . 310

V.5.5.2.1 Definition . . . 310

V.5.5.2.2 Clinical Features . . . 310

V.5.5.2.3 Dermoscopic Criteria . . . 310

V.5.5.2.4 Relevant Clinical Differential Diagnoses . . . 310

V.5.5.2.5 Histopathology . . . 310

V.5.5.2.6 Management . . . 310

V.5.6 Case Study . . . 310

References . . . .312

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V.5 undetermined), congenital hemangiomas (non- involuting, rapidly involuting) [20], cherry hem- angioma, tufted angioma, verrucous hemangio- ma, arteriovenous hemangioma, thrombosed capillary aneurysm, and lobular capillary hem- angioma (pyogenic granuloma). Some of these entities are beyond the scope of this chapter, and some are misnomers, but some will be consid- ered for their unique dermoscopic criteria and resemblance to melanoma.

V.5.2.1 Cherry Hemangioma V.5.2.1.1 Definition

Cherry hemangiomas (senile angioma, ruby spot, Campbell de Morgan spot) are the most com- mon vascular lesions that appear as small, bright- red papules in adulthood, usually on the trunk.

V.5.2.1.2 Clinical Features

Cherry hemangiomas vary in size from hardly visible, pinpoint lesions to soft, raised, dome- shaped, bright-red papules with a smooth sur- face measuring several millimeters in diameter (Fig. V.5.1a). The incidence rises sharply in the fourth decade, being almost universal in old age. They develop alone or in groups, several to hundreds in number occurring anywhere on the skin but most commonly on the trunk and proximal extremities [25].

V.5.2.1.3 Dermoscopic Criteria

Cherry hemangiomas reveal a typical lacunar pattern with red-blue lacunas (or lagoons) [29, 34] which are characterized by circumscribed, round-to-oval structures with a color varying from red, red-blue, dark-red to black (Fig. V.5.1b) [2, 16]. Criteria for melanocytic lesions should be absent, which is valid for all vascular lesions [3, 30, 34]. Histopathologically, red lacunas rep- resent dilated blood vessels in the upper dermis, and if they are dark-red or black in color, this means that they are partially or completely thrombosed [16, 29, 33, 34].

V.5.2.1.4 Relevant Clinical Differential Diagnoses

Thrombosed lesions may resemble angiokera- toma or melanoma, which can be differentiated by dermoscopy. Pyogenic granuloma differs with its sudden unset.

V.5.2.1.5 Histopathology

Cherry hemangiomas are composed of dilated capillaries and scant intervening stroma with a thin epidermal collarette at the periphery. In the early stage of development numerous, newly formed capillaries with narrow lumina and prominent endothelial cells, localized in the up-

Fig. V.5.1. a Cherry hemangioma, clinical view. A soft, polypoid, red-colored papule with a diameter of 4×6 mm is shown. b Cherry hemangioma, dermoscopy view. A typical lacunar pattern with circumscript, round-to-oval red structures, namely, lacunas, is shown

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per dermis, become dilated as the lesion ages;

thus, in a fully mature lesion, there are numer- ous dilated capillaries lined by flattened endo- thelial cells [4]. These endothelial cells have been shown to have non-replicating nature [31], indicating that cherry hemangioma may not be a true neoplasm.

V.5.2.1.6 Management

Cherry hemangiomas are usually left alone un- less cosmetically displeasing or prone to bleed- ing. If necessary, they may be removed by surgery (larger lesions), cryotherapy (10-s freeze–thaw cycle with a 1-mm margin), elec- trosurgery or laser (potassium titanyl phosphate vascular laser [6], flash-lamp-pumped pulsed- dye laser, or continuous-wave krypton laser [1]).

Laser-treated lesions undergo inflammation, necrosis, and eventual healing by 4 weeks [1].

Laser treatment is paralleled by psychological benefit also [10].

V.5.3 Pyogenic Granuloma V.5.3.1 Definition

Pyogenic granuloma (lobular capillary heman- gioma, granuloma telangiectaticum, granuloma of pregnancy) is a shiny red, often friable, and bleeding nodular lesion that develops rapidly, often following trauma, on the skin and mucosa of children, young adults and pregnant women.

V.5.3.2 Clinical Features

Pyogenic granuloma arises as solitary, soft, bright-red papule evolving rapidly in a few weeks and forming a darker, slightly peduncu- lated nodule, measuring up to 2–3 cm, with a frequently eroded and crusted surface which may bleed very easily (Fig. V.5.2a) [25]. It is common in one specific clinical setting, the gin- giva in pregnancy [8]. The areas subject to trau- ma, the hands (especially the fingers), forearms,

face, lips, and oral mucosa, are the sites of predi- lection [7, 22]. Rare multiple lesions may be grouped or eruptive and disseminated in na- ture.

V.5.3.3 Dermoscopic Criteria

Pyogenic granuloma has been reported that pyogenic granulomas cannot be diagnosed with more accuracy by dermoscopy than with naked eye [34]. Specific dermoscopic criteria of it have not yet been published. Recently, based on a morphological study of 13 patients, Zaballos et al. have evaluated the dermoscopic findings of pyogenic granuloma. The most frequently ob- served features were reddish homogeneous area (92%), white collaratte (84%), “white rail” lines that intersect the lesion (30%), and ulceration (46%). It was concluded that the absence of spe- cific criteria for other skin tumors and a reddish homogeneous area surrounded by a white col- larette were the most frequent dermoscopic pat- tern in pyogenic granuloma. The histopatho- logical counterparts were attributed to the proliferating capillaries for reddish homoge- neous areas, to the hyperplastic epithelium em- bracing the lesion for “white collarette,” and to the fibrous septa surrounding the lobules for

“white rail” lines [35]. A preliminary study on dermoscopic findings of pyogenic granuloma was also carried out in our dermoscopy unit. In a period of 3 months, 11 histopathologically confirmed cases of pyogenic granuloma were included. The most commonly occurring fea- tures were reddish and whitish homogeneous area (82%), white collaratte (55%), “white fence,”

the rods intersecting the lesion (82%), and ul-

ceration (55%). It was concluded that the most

frequent dermoscopic pattern in pyogenic gran-

uloma was reddish and whitish homogeneous

area with white fence (82%) and the other two

findings occurred in more than half of the pa-

tients (Fig. V.5.2b). Considering these two stud-

ies, it can be concluded that dermoscopy is a fa-

cilitative method in the diagnosis of pyogenic

granuloma.

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V.5 V.5.3.4 Relevant Clinical Differential Diagnosis

Pyogenic granuloma includes malignant skin tumors, namely, hypo-apigmented melanoma, basal cell carcinoma and squamous cell car- cinoma, Kaposi’s sarcoma, glomus tumor, and benign lesions such as multifocal infantile hemangiomas, cherry hemangioma, bacillary angiomatosis, angiolymphoid hyperplasia with eosinophilia, or milker’s nodule. The most im- portant one, melanoma, cannot be excluded with confidence without a biopsy.

V.5.3.5 Histopathology

The microscopic diagnosis is usually straightfor- ward. Pyogenic granuloma is simply composed of aggregates of angiomatous lobules. These lob- ular aggregates of proliferating capillaries are within a fibromyxoid or collagenous stroma which is infiltrated by inflammatory cells. As the lesion grows old, the capillary lumens become more dilated and evident. When the surface is ulcerated, the stroma becomes markedly infil- trated by neutrophils. In these lesions fibrin, hemorrhage, and necrosis may also be present, obscuring the lobular architecture. An epidermal collarette is seen in some exophytic tumors [5].

Fig. V.5.2. a Pyogenic granuloma, clinical view. A soft, dark-red nodule 8×10 mm in diameter with a shiny, partly eroded surface is shown. b Pyogenic granuloma, dermoscopy view (inset shows lower magnification).

A reddish and whitish homogeneous area with a partial white collaratte (white arrow) and rods intersecting the lesion, namely, “white fence” (black arrows) and some ulceration at the periphery are shown

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V.5.3.6 Management

Many lesions which occur in pregnancy resolve with parturition; thus, waiting is the best strat- egy in these patients. Drug-induced cases on oral contraceptives, systemic retinoids, or pro- tease inhibitors usually regress upon withdraw- al of the causative agent. In other cases pyogenic granulomas tend to persist. In this setting, re- moval of the lesion is necessary to relieve any bleeding, discomfort, cosmetic distress, or diag- nostic uncertainty. Treatment options, namely, surgical excision (full-thickness skin excision and linear closure), shave excision, or curettage followed by electrodessication to the base, vari- ous types of laser therapies, cryotherapy, injec- tion of sclerosing agents [21, 22, 24], chemical cauterization with silver nitrate, and ligation of the base (only in clinically obvious cases) [14], have all been reported to be effective. Formation of multiple satellites after surgical removal of pyogenic granuloma, due to a reflection of the proliferative response, should be kept in mind.

Systemic steroids may be effective in treating disseminated or recurrent pyogenic granulomas with satellites [32].

V.5.4 Angiokeratomas

The term “angiokeratoma” is applied to a group of vascular lesions characterized by telangiecta- sias and epidermal hyperplasia, which is sec- ondary. The varieties are solitary, circumscript, Mibelli, Fordyce, and Fabry types. Solitary an- giokeratoma is the one to emphasize for its re- semblance to various pigmented lesions, chiefly melanoma.

V.5.4.1 Solitary Angiokeratoma V.5.4.1.1 Definition

Solitary angiokeratoma is an acquired lesion as a response to trauma, seen generally on the low- er extremities of young adults as a warty, dark papule.

V.5.4.1.2 Clinical Features

Early lesions appear bright red and soft, later becoming blue-black, firm, and hyperkeratotic, measuring 2–10 mm in diameter. It is usually seen between the age of 10 and 40 years. Al- though single, multiple lesions may occur, the legs are the site of predilection, but they may oc- cur anywhere. A case of intra-oral solitary an- giokeratoma has been reported [18].

V.5.4.1.3 Dermoscopic Criteria

Solitary angiokeratoma reveals a lacunar or multicomponent pattern with large, several to numerous, sharply demarcated, red-blue lacu- nas. The distinctive lacunas, sometimes togeth- er with whitish-yellowish keratotic areas, are diagnostic. At times a blue-whitish veil, due to the acanthotic epidermis with orthokeratosis, is observed in solitary angiokeratoma. This should not be considered in the diagnostic algorithm, since it is not associated with any network or other melanoma-specific criteria. A reddish halo around the lesion can also be seen due to a recent trauma in solitary angiokeratoma [16, 30, 34].

V.5.4.1.4 Relevant Clinical Differential Diagnoses

Solitary angiokeratoma mimics viral warts, seb- orrheic keratosis, pigmented basal cell carcino- ma, thrombosed hemangioma, melanocytic nevi, and most importantly, melanoma, all of which can be differentiated by means of der- moscopy in most cases.

V.5.4.1.5 Histopathology

Histopathological findings consist of numerous

dilated papillary blood vessels underlying an

epidermis that shows acanthosis with elonga-

tion of rete ridges and hyperkeratosis. Marked

dilatation of the capillaries, sometimes forming

large cavernous channels, expands a group of

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V.5 dermal papillae. Rete ridges tend to enclose the underlying capillary spaces [4].

V.5.4.1.6 Management

Solitary angiokeratomas are asymptomatic, be- nign lesions and require no treatment. Local ex- cision is often needed for cosmetic purposes or because of anxiety about the diagnosis.

V.5.5 Hemorrhages

Subungual or subcorneal hemorrhages may mimic pigmented melanocytic lesions, thereby requiring an exact diagnosis.

V.5.5.1 Subungual Hematoma V.5.5.1.1 Definition

Subungual hematoma (bleeding under nail, ten- nis toe, jogger’s toe) is a collection of blood be- neath a finger or toe nail, often caused by a crush injury or repeated microtrauma to the nail bed.

V.5.5.1.2 Clinical Features

Erythema, edema, and a throbbing pain may sometimes precede an extensive hemorrhage which requires prompt treatment (trephining) or else the nail plate may be lost [23]. Smaller hematomas usually subside with a temporary disturbance in nail growth. Repeated micro- trauma causing subungual hematoma is not no- ticed by the patient who seeks medical advice only for discoloration. This type of subungual hematoma is characterized by round or oval- shaped sharply circumscribed, reddish-black or jet-black areas of discoloration in various sizes [30, 34]. Subungual hematoma is occasionally observed as a longitudinal melanonychia [12].

Pseudopods protruding from a purple pigmen- tation of the nail plate, seen under cover glass and oil immersion, has been reported to be an additional clinical feature observed in hemor- rhage of the nail unit [11].

V.5.5.1.3 Dermoscopic Criteria

The discolored area typically reveals a homoge- neous pattern with sharply demarcated, red- dish-black or dark-red area (blood spots). Oc- casionally, tiny reddish dots may be seen in the neighboring area [30, 34]. Although blood spots are mostly observed in subungual hematomas, their presence cannot rule out melanoma in equivocal lesions [26]. The presence of blood, recognized as an amorphous, diffuse pigmenta- tion, or as dots and/or globules of purple-red color, may also be a potential warning for mela- noma on dermoscopy. Since blood can also be observed in subungual melanomas, a careful search for melanoma-specific criteria (overall asymmetry of color and structure, irregular dif- fuse pigmentation, black dots of various sizes distributed asymmetrically throughout the le- sion) is essential [15] before diagnosing subun- gual hematoma. For better interpretation, der- moscopic examination of the nail bed and matrix has been proposed recently [13].

V.5.5.1.4 Histopathology

Subungual hematoma reveals extravasated erythrocytes reaching the matricial bed.

V.5.5.1.5 Relevant Clinical Differential Diagnoses

Subungual melanoma, subungual melanocytic nevi, various types of nail apparatus lentigines, melanotic macule of the nail matrix, subungual pigmented basal cell carcinoma, ethnic-type nail pigmentation, drug-induced nail pigmenta- tion, and nail infections should all be consid- ered.

V.5.5.1.6 Management

No treatment is required if clinical and dermo-

scopic features are straightforward. In case of

suspicion for malignancy, a biopsy is mandato-

ry.

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V.5.5.2 Subcorneal Hematoma V.5.5.2.1 Definition

Subcorneal hematoma (black heel, tennis heel, talon noir, calcaneal petechiae) is a self-limited, trauma-induced pigmentation secondary to ex- travasation of erythrocytes seen on the heels or volar skin of athletic individuals.

V.5.5.2.2 Clinical Features

Subcorneal hematoma is characterized by sharply circumscribed, blue-black to reddish- brown macules in various shapes: round; linear;

punctuated; or irregular. Participating in sports that involve frequent starts and stops, leads to a traumatic rupture in the papillary capillaries of volar skin. A resultant hemorrhage, frequently at the back or side of the heel, is observed. Gen- tly paring of the surface of the lesion with a scal- pel may be diagnostic.

V.5.5.2.3 Dermoscopic Criteria

Subcorneal hematoma is defined as a red-bluish to reddish-black homogeneous area on dermos- copy [30]. Actually, the most frequent dermo- scopic pattern is the homogeneous one. Dermo- scopic features of subcorneal hematoma were defined first by Saida et al. under the name of

“cutaneous hemorrhagic macule” as a sharply demarginated, reddish-brown to reddish-black homogeneous pigmentation [27]; however, for the ones localized on the planter region, so- called black heel, reddish-black pebble-like droplets aggregated mainly on the ridges of the skin markings are defined as a typical finding.

In 2002 the same authors published their obser- vations similarly, calling this unique dermo- scopic feature “pebbles on the ridges.” They added that subcorneal hematomas on the acral skin showed the same pattern as that on non- glabrous skin, namely, dark red-black homoge- neous areas [28]. The only other study on der- moscopy of subcorneal hematomas was carried out by Zalaudek et al. in which they observed homogeneous pattern (53.3%), parallel-ridge

pattern (40%), globular pattern combined with one of these two (46.7%), and parallel-furrow pattern and fibrillary pattern, one case each.

They concluded that the presence of red-black to grayish color, a homogeneous pattern of pig- mentation, and red-black globules seen as satel- lites at the periphery were the most striking fea- tures for the diagnosis of subcorneal hematoma [36].

V.5.5.2.4 Relevant Clinical Differential Diagnoses

Acral melanoma, acral melanocytic nevi, plant- er warts, angiokeratoma, and pyogenic granu- loma should be considered.

V.5.5.2.5 Histopathology

Extravasated erythrocytes may be observed in the dermal papillae, however some move into the epidermis by transepidermal elimination;

therefore, histopathological changes are often limited to stratum corneum where amorphous, yellow-brown material, in rounded collections, derived from hemoglobin, is seen [17].

V.5.5.2.6 Management

To be confident, the diagnosis can be aided by paring down the lesion or following up; howev- er, if the diagnosis remains in doubt, a biopsy is indicated.

V.5.6 Case Study

Patient Comment

A colored swelling on her head which was de- tected by her coiffeur 1 month previously.

Questions Asked By the Physician

Age: 52 years

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V.5 Duration: 1 month

Morphological change: unknown Pain or other symptoms: none Trauma: none

Personal/familial skin tumor: none Noteworthy disease: hypertension Immune system status: normal

Clinical Diagnosis and Differential Diagnosis

Hemangioma, angiosarcoma of the scalp, ma- lignant blue nevus, atypical fibroxanthoma, plaque type of Kaposi’s sarcoma, tufted angio- ma, angiokeratoma circumscriptum, angiolym- phoid hyperplasia with eosinophilia, Dabska tumor, and bacillary angiomatosis (plaque-type pattern) were all considered. The clinical image is shown in Fig. V.5.3.

Management

Punch biopsy and subsequent histopathological examination revealed a diagnosis of angioma.

Consequently, the patient has only been fol- lowed up and the lesion has remained stable since presentation.

Comments

The histopathological diagnosis of angioma was expected due to dermoscopic findings (Fig. V.5.4); however, this localized and plaque type of hemangioma is defined only in infancy [37]. In adults, senile angiomas are usual and, although they may develop in groups, they do not form an isolated plaque. Exceptionally, Ilyas et al. [38] has recently reported a case of ac- quired acral angioma of agminated type; there- fore, the other benign and malignant tumors, which used to be seen in older age, especially in the area of head and neck or scalp, with the de- fined clinical features, were included in the clinical differential diagnosis. Histopathologi- cally, the epidermis was intact, having only a thin orthokeratotic keratin layer. In the superfi- cial dermis, thin-walled dilated capillary vessels of variable sizes, lined by single-layered endo- thelial cells, were observed. Several mature lym- phocytes, together with the pilosebaceous units, were seen around these dilated capillaries. These histopathological findings were characteristic for an angioma of the scalp, and surely excluded the other mentioned clinical diagnoses. To the best of our knowledge, this is the first case of

“acquired plaque-type angioma” of the scalp re- ported in the literature.

Fig. V.5.3. Clinical image.

A pinkish to violaceous plaque on the scalp of a 52-year-old woman

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C

Core Messages

■ In vascular lesions always be aware not to miss a melanoma.

■ As a rule, dermoscopy is very helpful for diagnosing vascular lesions.

■ Dermoscopically, cherry hemangiomas reveal a typical lacunar pattern with red-blue lagoons.

■ The most frequently observed features in pyogenic granuloma are reddish homogeneous area, white collaratte,

“white rail” lines that intersect the lesion, and ulceration.

■ Solitary angiokeratomas reveal a lacunar or multicomponent pattern with large red-blue lacunas.

■ Hemorrhages reveal a homogeneous pattern with sharply demarcated dark- red blood spots.

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