Chapter V.5 Vascular Lesions Fezal Özdemir V.5

Contents

V.5.1 Introduction ...... 303 V.5.5.2 Subcorneal Hematoma...... 310 V.5.2 ...... 303 V.5.5.2.1 Definition...... 310 V.5.2.1 Cherry ...... 304 V.5.5.2.2 Clinical Features...... 310 V.5.2.1.1 Definition ...... 304 V.5.5.2.3 Dermoscopic Criteria...... 310 V.5.2.1.2 Clinical Features...... 304 V.5.5.2.4 Relevant Clinical Differential V.5.2.1.3 Dermoscopic Criteria...... 304 Diagnoses...... 310 V.5.2.1.4 Relevant Clinical Differential V.5.5.2.5 Histopathology...... 310 Diagnoses...... 304 V.5.5.2.6 Management ...... 310 V.5.2.1.5 Histopathology ...... 304 V.5.6 Case Study ...... 310 V.5.2.1.6 Management ...... 305 References...... 312 V.5.3 ...... 305 V.5.3.1 Definition ...... 305 V.5.3.2 Clinical Features...... 305 V.5.3.3 Dermoscopic Criteria...... 305 V.5.1 Introduction V.5.3.4 Relevant Clinical Differential Diagnosis...... 306 V.5.3.5 Histopathology ...... 306 The biological classification of vascular lesions V.5.3.6 Management ...... 307 into two major categories as tumors and malfor- mations [19] forms a framework for conceiving V.5.4 ...... 307 them in a coherent manner, although evidence V.5.4.1 Solitary ...... 307 for their association has been reported in a small V.5.4.1.1 Definition...... 307 minority [9]. Among them, the lesions that V.5.4.1.2 Clinical Features...... 307 simulate pigmented tumors, especially the V.5.4.1.3 Dermoscopic Criteria...... 307 melanoma, are discussed in this chapter. Some V.5.4.1.4 Relevant Clinical Differential of these fall into the category of tumors (hem- Diagnoses...... 307 , pyogenic granuloma) and some into V.5.4.1.5 Histopathology...... 307 malformations (angiokeratoma). In addition, V.5.4.1.6 Management ...... 308 subcorneal and subungual hemorrhage is in- V.5.5 Hemorrhages ...... 308 cluded here for diagnostic purposes. V.5.5.1 Subungual Hematoma...... 308 V.5.5.1.1 Definition...... 308 V.5.5.1.2 Clinical Features...... 308 V.5.2 Hemangiomas V.5.5.1.3 Dermoscopic Criteria...... 308 V.5.5.1.4 Histopathology...... 308 Hemangiomas are benign vascular tumors V.5.5.1.5 Relevant Clinical Differential due to the proliferation of blood vessels. The Diagnoses...... 308 term includes many entities, namely, infantile V.5.5.1.6 Management ...... 308 hemangiomas (localized, segmental, multifocal, 304 F. Özdemir

­undetermined), congenital hemangiomas (non- involuting, rapidly involuting) [20], cherry hem- angioma, , verrucous hemangio- ma, arteriovenous hemangioma, thrombosed , 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- Fig. V.5.1. a Cherry hemangioma, clinical view. A soft, face measuring several millimeters in diameter polypoid, red-colored papule with a diameter of 4×6 mm (Fig. V.5.1a). The incidence rises sharply in the is shown. b Cherry hemangioma, dermoscopy view. A fourth decade, being almost universal in old typical lacunar pattern with circumscript, round-to-oval red structures, namely, lacunas, is shown 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.5.2.1.4 Relevant Clinical Differential Diagnoses

V.5.2.1.3 Dermoscopic Criteria Thrombosed lesions may resemble angiokera- toma or melanoma, which can be differentiated Cherry hemangiomas reveal a typical lacunar by dermoscopy. Pyogenic granuloma differs pattern with red-blue lacunas (or lagoons) [29, with its sudden unset. 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) V.5.2.1.5 Histopathology [2, 16]. Criteria for melanocytic lesions should be absent, which is valid for all vascular lesions Cherry hemangiomas are composed of dilated [3, 30, 34]. Histopathologically, red lacunas rep- and scant intervening stroma with a V.5 resent dilated blood vessels in the upper dermis, thin epidermal collarette at the periphery. In the and if they are dark-red or black in color, this early stage of development numerous, newly means that they are partially or completely formed capillaries with narrow lumina and thrombosed [16, 29, 33, 34]. prominent endothelial cells, localized in the up- Vascular Lesions Chapter V.5 305 per dermis, become dilated as the lesion ages; face, lips, and oral mucosa, are the sites of predi- thus, in a fully mature lesion, there are numer- lection [7, 22]. Rare multiple lesions may be ous dilated capillaries lined by flattened endo- grouped or eruptive and disseminated in na- thelial cells [4]. These endothelial cells have ture. been shown to have non-replicating nature [31], indicating that cherry hemangioma may not be a true . V.5.3.3 Dermoscopic Criteria Pyogenic granuloma has been reported that V.5.2.1.6 Management pyogenic granulomas cannot be diagnosed with more accuracy by dermoscopy than with naked Cherry hemangiomas are usually left alone un- eye [34]. Specific dermoscopic criteria of it have less cosmetically displeasing or prone to bleed- not yet been published. Recently, based on a ing. If necessary, they may be removed by morphological study of 13 patients, Zaballos et ­surgery (larger lesions), cryotherapy (10-s al. have evaluated the dermoscopic findings of freeze–thaw cycle with a 1-mm margin), elec- pyogenic granuloma. The most frequently ob- trosurgery or laser (potassium titanyl phosphate served features were reddish homogeneous area vascular laser [6], flash-lamp-pumped pulsed- (92%), white collaratte (84%), “white rail” lines dye laser, or continuous-wave krypton laser [1]). that intersect the lesion (30%), and ulceration Laser-treated lesions undergo , (46%). It was concluded that the absence of spe- necrosis, and eventual healing by 4 weeks [1]. cific criteria for other skin tumors and a reddish Laser treatment is paralleled by psychological homogeneous area surrounded by a white col- benefit also [10]. larette were the most frequent dermoscopic pat- tern in pyogenic granuloma. The histopatho- logical counterparts were attributed to the V.5.3 Pyogenic Granuloma proliferating capillaries for reddish homoge- neous areas, to the hyperplastic epithelium em- V.5.3.1 Definition bracing the lesion for “white collarette,” and to the fibrous septa surrounding the lobules for Pyogenic granuloma (lobular capillary heman- “white rail” lines [35]. A preliminary study on gioma, granuloma telangiectaticum, granuloma dermoscopic findings of pyogenic granuloma of pregnancy) is a shiny red, often friable, and was also carried out in our dermoscopy unit. In bleeding nodular lesion that develops rapidly, a period of 3 months, 11 histopathologically often following trauma, on the skin and mucosa confirmed cases of pyogenic granuloma were of children, young adults and pregnant women. included. The most commonly occurring fea- tures were reddish and whitish homogeneous area (82%), white collaratte (55%), “white fence,” V.5.3.2 Clinical Features the rods intersecting the lesion (82%), and ul- ceration (55%). It was concluded that the most Pyogenic granuloma arises as solitary, soft, frequent dermoscopic pattern in pyogenic gran- bright-red papule evolving rapidly in a few uloma was reddish and whitish homogeneous weeks and forming a darker, slightly peduncu- area with white fence (82%) and the other two lated nodule, measuring up to 2–3 cm, with a findings occurred in more than half of the pa- frequently eroded and crusted surface which tients (Fig. V.5.2b). Considering these two stud- may bleed very easily (Fig. V.5.2a) [25]. It is ies, it can be concluded that dermoscopy is a fa- common in one specific clinical setting, the gin- cilitative method in the diagnosis of pyogenic giva in pregnancy [8]. The areas subject to trau- granuloma. ma, the hands (especially the fingers), forearms, 306 F. Özdemir

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

V.5.3.4 Relevant Clinical Differential V.5.3.5 Histopathology Diagnosis The microscopic diagnosis is usually straightfor- Pyogenic granuloma includes malignant skin ward. Pyogenic granuloma is simply composed tumors, namely, hypo-apigmented melanoma, of aggregates of angiomatous lobules. These lob- basal cell carcinoma and squamous cell car­ ular aggregates of proliferating capillaries are cinoma, Kaposi’s , , and within a fibromyxoid or collagenous stroma benign lesions such as multifocal infantile which is infiltrated by inflammatory cells. As the hemangiomas, cherry hemangioma, bacillary lesion grows old, the capillary lumens become , angiolymphoid hyperplasia with more dilated and evident. When the surface is eosinophilia, or milker’s nodule. The most im- ulcerated, the stroma becomes markedly infil- V.5 portant one, melanoma, cannot be excluded trated by neutrophils. In these lesions fibrin, with confidence without a biopsy. hemorrhage, and necrosis may also be present, obscuring the lobular architecture. An epidermal collarette is seen in some exophytic tumors [5]. Vascular Lesions Chapter V.5 307

V.5.3.6 Management V.5.4.1.2 Clinical Features

Many lesions which occur in pregnancy resolve Early lesions appear bright red and soft, later with parturition; thus, waiting is the best strat- becoming blue-black, firm, and hyperkeratotic, egy in these patients. Drug-induced cases on measuring 2–10 mm in diameter. It is usually oral contraceptives, systemic retinoids, or pro- seen between the age of 10 and 40 years. Al- tease inhibitors usually regress upon withdraw- though single, multiple lesions may occur, the al of the causative agent. In other cases pyogenic legs are the site of predilection, but they may oc- granulomas tend to persist. In this setting, re- cur anywhere. A case of intra-oral solitary an- moval of the lesion is necessary to relieve any giokeratoma has been reported [18]. bleeding, discomfort, cosmetic distress, or diag- nostic uncertainty. Treatment options, namely, surgical excision (full-thickness skin excision V.5.4.1.3 Dermoscopic Criteria and linear closure), shave excision, or curettage followed by electrodessication to the base, vari- Solitary angiokeratoma reveals a lacunar or ous types of laser therapies, cryotherapy, injec- multicomponent pattern with large, several to tion of sclerosing agents [21, 22, 24], chemical numerous, sharply demarcated, red-blue lacu- cauterization with silver nitrate, and ligation of nas. The distinctive lacunas, sometimes togeth- the base (only in clinically obvious cases) [14], er with whitish-yellowish keratotic areas, are have all been reported to be effective. Formation diagnostic. At times a blue-whitish veil, due to of multiple satellites after surgical removal of the acanthotic epidermis with orthokeratosis, is pyogenic granuloma, due to a reflection of the observed in solitary angiokeratoma. This should proliferative response, should be kept in mind. not be considered in the diagnostic algorithm, Systemic steroids may be effective in treating since it is not associated with any network or disseminated or recurrent pyogenic granulomas other melanoma-specific criteria. A reddish with satellites [32]. halo around the lesion can also be seen due to a recent trauma in solitary angiokeratoma [16, 30, 34]. V.5.4 Angiokeratomas

The term “angiokeratoma” is applied to a group V.5.4.1.4 Relevant Clinical Differential of vascular lesions characterized by telangiecta- Diagnoses sias and epidermal hyperplasia, which is sec- ondary. The varieties are solitary, circumscript, Solitary angiokeratoma mimics viral warts, seb- Mibelli, Fordyce, and Fabry types. Solitary an- orrheic keratosis, pigmented basal cell carcino- giokeratoma is the one to emphasize for its re- ma, thrombosed hemangioma, melanocytic semblance to various pigmented lesions, chiefly nevi, and most importantly, melanoma, all of melanoma. which can be differentiated by means of der- moscopy in most cases. V.5.4.1 Solitary Angiokeratoma V.5.4.1.5 Histopathology V.5.4.1.1 Definition Histopathological findings consist of numerous Solitary angiokeratoma is an acquired lesion as dilated papillary blood vessels underlying an a response to trauma, seen generally on the low- epidermis that shows acanthosis with elonga- er extremities of young adults as a warty, dark tion of rete ridges and . Marked papule. dilatation of the capillaries, sometimes forming large cavernous channels, expands a group of 308 F. Özdemir

dermal papillae. Rete ridges tend to enclose the V.5.5.1.3 Dermoscopic Criteria underlying capillary spaces [4]. The discolored area typically reveals a homoge- neous pattern with sharply demarcated, red- V.5.4.1.6 Management dish-black or dark-red area (blood spots). Oc- casionally, tiny reddish dots may be seen in the Solitary angiokeratomas are asymptomatic, be- neighboring area [30, 34]. Although blood spots nign lesions and require no treatment. Local ex- are mostly observed in subungual hematomas, cision is often needed for cosmetic purposes or their presence cannot rule out melanoma in because of anxiety about the diagnosis. equivocal lesions [26]. The presence of blood, recognized as an amorphous, diffuse pigmenta- tion, or as dots and/or globules of purple-red V.5.5 Hemorrhages color, may also be a potential warning for mela- noma on dermoscopy. Since blood can also be Subungual or subcorneal hemorrhages may observed in subungual melanomas, a careful mimic pigmented melanocytic lesions, thereby search for melanoma-specific criteria (overall requiring an exact diagnosis. asymmetry of color and structure, irregular dif- fuse pigmentation, black dots of various sizes distributed asymmetrically throughout the le- V.5.5.1 Subungual Hematoma sion) is essential [15] before diagnosing subun- gual hematoma. For better interpretation, der- V.5.5.1.1 Definition moscopic examination of the nail bed and matrix has been proposed recently [13]. 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 V.5.5.1.4 Histopathology injury or repeated microtrauma to the nail bed. Subungual hematoma reveals extravasated erythrocytes reaching the matricial bed. V.5.5.1.2 Clinical Features

Erythema, edema, and a throbbing pain may V.5.5.1.5 Relevant Clinical Differential sometimes precede an extensive hemorrhage Diagnoses which requires prompt treatment (trephining) or else the nail plate may be lost [23]. Smaller Subungual melanoma, subungual melanocytic hematomas usually subside with a temporary nevi, various types of nail apparatus lentigines, disturbance in nail growth. Repeated micro- melanotic macule of the nail matrix, subungual trauma causing subungual hematoma is not no- pigmented basal cell carcinoma, ethnic-type ticed by the patient who seeks medical advice nail pigmentation, drug-induced nail pigmenta- only for discoloration. This type of subungual tion, and nail infections should all be consid- hematoma is characterized by round or oval- ered. shaped sharply circumscribed, reddish-black or jet-black areas of discoloration in various sizes [30, 34]. Subungual hematoma is occasionally V.5.5.1.6 Management observed as a longitudinal melanonychia [12]. V.5 Pseudopods protruding from a purple pigmen- No treatment is required if clinical and dermo- tation of the nail plate, seen under cover glass scopic features are straightforward. In case of and oil immersion, has been reported to be an suspicion for malignancy, a biopsy is mandato- additional clinical feature observed in hemor- ry. rhage of the nail unit [11]. Vascular Lesions Chapter V.5 309

V.5.5.2 Subcorneal Hematoma pattern (40%), globular pattern combined with one of these two (46.7%), and parallel-furrow V.5.5.2.1 Definition pattern and fibrillary pattern, one case each. They concluded that the presence of red-black Subcorneal hematoma (black heel, tennis heel, to grayish color, a homogeneous pattern of pig- talon noir, calcaneal petechiae) is a self-limited, mentation, and red-black globules seen as satel- trauma-induced pigmentation secondary to ex- lites at the periphery were the most striking fea- travasation of erythrocytes seen on the heels or tures for the diagnosis of subcorneal hematoma volar skin of athletic individuals. [36].

V.5.5.2.2 Clinical Features V.5.5.2.4 Relevant Clinical Differential Diagnoses Subcorneal hematoma is characterized by sharply circumscribed, blue-black to reddish- Acral melanoma, acral melanocytic nevi, plant- brown macules in various shapes: round; linear; er warts, angiokeratoma, and pyogenic granu- punctuated; or irregular. Participating in sports loma should be considered. that involve frequent starts and stops, leads to a traumatic rupture in the papillary capillaries of volar skin. A resultant hemorrhage, frequently V.5.5.2.5 Histopathology at the back or side of the heel, is observed. Gen- tly paring of the surface of the lesion with a scal- Extravasated erythrocytes may be observed in pel may be diagnostic. the dermal papillae, however some move into the epidermis by transepidermal elimination; therefore, histopathological changes are often V.5.5.2.3 Dermoscopic Criteria limited to stratum corneum where amorphous, yellow-brown material, in rounded collections, Subcorneal hematoma is defined as a red-bluish derived from hemoglobin, is seen [17]. to reddish-black homogeneous area on dermos- copy [30]. Actually, the most frequent dermo- scopic pattern is the homogeneous one. Dermo- V.5.5.2.6 Management scopic features of subcorneal hematoma were defined first by Saida et al. under the name of To be confident, the diagnosis can be aided by “cutaneous hemorrhagic macule” as a sharply paring down the lesion or following up; howev- demarginated, reddish-brown to reddish-black er, if the diagnosis remains in doubt, a biopsy is homogeneous pigmentation [27]; however, for indicated. the ones localized on the planter region, so- called black heel, reddish-black pebble-like droplets aggregated mainly on the ridges of the V.5.6 Case Study skin markings are defined as a typical finding. In 2002 the same authors published their obser- vations similarly, calling this unique dermo- Patient Comment scopic feature “pebbles on the ridges.” They added that subcorneal hematomas on the acral A colored swelling on her head which was de- skin showed the same pattern as that on non- tected by her coiffeur 1 month previously. glabrous skin, namely, dark red-black homoge- neous areas [28]. The only other study on der- moscopy of subcorneal hematomas was carried Questions Asked By the Physician out by Zalaudek et al. in which they observed homogeneous pattern (53.3%), parallel-ridge Age: 52 years 310 F. Özdemir

Fig. V.5.3. Clinical image. A pinkish to violaceous plaque on the scalp of a 52-year-old woman

Comments Duration: 1 month Morphological change: unknown Pain or other symptoms: none The histopathological diagnosis of angioma Trauma: none was expected due to dermoscopic findings Personal/familial skin tumor: none (Fig. V.5.4); however, this localized and plaque Noteworthy disease: type of hemangioma is defined only in infancy Immune system status: normal [37]. In adults, senile are usual and, although they may develop in groups, they do not form an isolated plaque. Exceptionally, Ilyas Clinical Diagnosis and Differential et al. [38] has recently reported a case of ac- Diagnosis quired acral angioma of agminated type; there- fore, the other benign and malignant tumors, Hemangioma, of the scalp, ma- which used to be seen in older age, especially in lignant blue , atypical fibroxanthoma, the area of head and neck or scalp, with the de- plaque type of Kaposi’s sarcoma, tufted angio- fined clinical features, were included in the ma, angiokeratoma circumscriptum, angiolym- clinical differential diagnosis. Histopathologi- phoid hyperplasia with eosinophilia, Dabska cally, the epidermis was intact, having only a tumor, and (plaque-type thin orthokeratotic keratin layer. In the superfi- pattern) were all considered. The clinical image cial dermis, thin-walled dilated capillary vessels is shown in Fig. V.5.3. of variable sizes, lined by single-layered endo- thelial cells, were observed. Several mature lym- phocytes, together with the pilosebaceous units, Management were seen around these dilated capillaries. These histopathological findings were characteristic Punch biopsy and subsequent histopathological for an angioma of the scalp, and surely excluded V.5 examination revealed a diagnosis of angioma. the other mentioned clinical diagnoses. To the Consequently, the patient has only been fol- best of our knowledge, this is the first case of lowed up and the lesion has remained stable “acquired plaque-type angioma” of the scalp re- since presentation. ported in the literature. Vascular Lesions Chapter V.5 311

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V.5