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Chapter 92 – Benign Eyelid Lesions

Chapter 92 – Benign Eyelid Lesions

ANN G. NEFF KEITH D. CARTER

INTRODUCTION The eyelids may be affected by a wide spectrum of benign and malignant lesions. In a study that analyzed all eyelid lesions submitted for histopathological examination over a 38-year period, benign lesions were 3 times more frequent than malignant neoplasms.[1] Many lesions which affect the eyelids may occur on any skin surface, but some occur exclusively or more frequently on the eyelids. The more common benign eyelid lesions are presented here, classified by origin, with each discussion highlighting the important clinical features, differential diagnosis, pertinent systemic associations, histopathology, and treatment. EPITHELIAL TUMORS Epithelial cells of the epidermis are arranged in four layers, from deep to superficial: • The stratum germinativum, known as the basal layer • The stratum spinosum, referred to as the squamous or prickle cell layer • The stratum granulosum, called the granular layer • The stratum corneum, also known as the horny or keratin layer A variety of histopathological changes that affect these layers of the epidermis may be observed within lesions affecting the eyelids. Such changes may be helpful to distinguish the vast variety of lesions that may be found on the eyelids. Hyperkeratosis, or thickening of the keratin layer, is seen clinically as an adherent scale. Parakeratosis is a form of hyperkeratosis characterized by incomplete keratinization, with retention of nuclei within the keratin layer. Dyskeratosis is abnormal keratinization of cells within the squamous layer. Acanthosis, or thickening of the squamous layer, is seen commonly in proliferative epithelial lesions. Acantholysis refers to separation of epithelial cells. Each type of epithelial tumor may exhibit some variability in its clinical picture and morphological features. In addition, different types of tumors may share similar clinical and morphological features, which results in clinical diagnostic confusion. A definitive diagnosis of these various lesions depends upon histopathological examination. Squamous Papilloma The most common benign lesion of the eyelid is the squamous papilloma, also known as a fibroepithelial polyp, acrochordon, or skin tag. These lesions may be single or multiple and commonly involve the eyelid margin. Squamous papillomas characteristically are flesh colored and may be sessile or pedunculated ( Fig. 92-1 ). Diagnosis is made by the typical clinical appearance and histological characteristics. The differential diagnosis includes seborrheic keratosis, verruca vulgaris, and intradermal nevus. Microscopically, the lesion has finger-like projections with a fibrovascular core, and the overlying epidermis demonstrates acanthosis and hyperkeratosis. Treatment is simple excision at the base of the lesion. Figure 92-1 Squamous papilloma. A, Typical flesh-colored, pedunculated skin tag involving the left upper eyelid. B, Fibroepithelial papilloma consists of a narrow-based (to the right) papilloma with fibrovascular core and finger-like projections covered by acanthotic, hyperkeratotic epithelium. (B, From Yanoff M, Fine BS. Ocular pathology, ed 5. St. Louis: Mosby; 2002.) Cutaneous Horn A cutaneous horn is a projection of packed keratin ( Fig. 92-2 ). This is a clinically descriptive term, not a diagnostic one. Cutaneous horn is not a distinct pathological entity but may develop from a variety of underlying lesions, including seborrheic keratosis, actinic keratosis, inverted follicular keratosis, verruca vulgaris, basal cell carcinoma (BCC), squamous cell carcinoma (SCC), and other epidermal tumors. Because definitive therapy is dependent on the underlying cause, biopsy of the cutaneous horn (including the underlying epidermis) is required to obtain a histological diagnosis.[2] Seborrheic Keratosis Seborrheic keratosis, also known as senile verruca, is a common benign epithelial neoplasm which may occur on the face, trunk, and extremities. These lesions usually affect middle-aged and older adults, occurring as single or multiple, greasy, stuck-on plaques ( Fig. 92-3 ). Eyelid lesions are often pedunculated. Color 699 Figure 92-2 Cutaneous horn. Note the projection of packed keratin that arises from the skin in the region of the left lateral canthus. Figure 92-3 Seborrheic keratosis. Brown, stuck-on plaque, typical of seborrheic keratosis. varies from tan to brown, and the surface is frequently papillomatous. The differential diagnosis includes skin tag, nevus, verruca vulgaris, actinic keratosis, and pigmented BCC. Seborrheic keratoses are not considered premalignant lesions. A systemic association, however, known as the sign of Leser–Trélat, denotes a rapid increase in the size and number of seborrheic keratoses which may occur in patients with occult malignancy.[3] A variant of seborrheic keratosis, which shares a similar histopathological appearance, is dermatosis papulosa nigra, which occurs primarily in dark-skinned individuals. These lesions usually appear on the cheeks and periorbital region as multiple pigmented papules ( Fig. 92-4 ). Although different histopathological types of seborrheic keratoses exist, all lesions share features of hyperkeratosis, acanthosis, and papillomatosis. [4] Most lesions contain horn cysts, which are keratin-filled inclusions within the acanthotic epidermis, and pseudohorn cysts, which represent invaginations of surface keratin. [5] Simple excision may be performed for biopsy or cosmesis, or to prevent irritation. Inverted Follicular Keratosis Inverted follicular keratosis, also known as basosquamous cell acanthoma, usually appears as a small, solitary, papillomatous lesion Figure 92-4 Dermatosis papulosa nigra. Multiple pigmented papules involving the malar region. on the face. It is a well-demarcated, keratotic mass, which may appear as a cutaneous horn. The lesion may resemble verruca vulgaris and seborrheic keratosis—many consider it an irritated seborrheic keratosis.[6] Histopathology reveals hyperkeratosis and lobular acanthosis. Proliferation of basaloid cells occurs with areas of acantholysis and zones of squamous cells, often arranged in whorls called squamous eddies. Treatment is complete excision, because recurrence is common after incomplete removal. Pseudoepitheliomatous Hyperplasia Pseudoepitheliomatous, or pseudocarcinomatous, hyperplasia is a benign epithelial proliferation which often develops rapidly over several weeks. It appears elevated, with an irregular surface which may be hyperkeratotic or ulcerated. This lesion represents an epidermal response to an underlying disorder, often inflammatory in nature. Associated conditions include mycotic infections, burns, ulcers, surgical wounds, radiation therapy, and insect bites. It also may occur adjacent to malignant neoplasms such as basal and squamous cell carcinomas. Histopathology reveals acanthotic squamous epithelium irregularly invading the dermis, with an inflammatory reaction, often involving the overlying epidermis. Although the squamous cells usually lack atypical changes, differentiation from low-grade SCC may be difficult. Biopsy is necessary for diagnosis, with treatment directed at the underlying condition. Keratoacanthoma Keratoacanthoma most commonly appears as a solitary, rapidly growing nodule on sun-exposed areas of middle-aged and older individuals. The nodule is usually umbilicated, with a distinctive central crater filled with a keratin plug ( Fig. 92-5 ). The lesion develops over weeks and typically undergoes spontaneous involution within 6 months to leave an atrophic scar. Lesions which occur on the eyelids may produce mechanical abnormalities, such as ectropion or ptosis, and occasionally may cause destructive changes.[7] The differential diagnosis includes SCC, BCC, verruca vulgaris, and molluscum contagiosum (MC). Patients with Muir–Torre syndrome may develop, in association with internal malignancy, multiple keratoacanthomas and sebaceous neoplasms. Microscopically, there is cup-shaped elevation of acanthotic squamous epithelium which surrounds a central mass of keratin. Microabscesses which contain necrotic keratinocytes and neutrophils may be found within the proliferative epithelium. The base is usually noninfiltrating and often demarcated from 700 Figure 92-5 Keratoacanthoma. A, Lesion shows typical clinical appearance; history was also typical. B, The lesion that can be seen above the surface epithelium has a cup-shaped configuration, and a central keratin core. The base of the acanthotic epithelium is blunted (rather than invasive) at the junction of the dermis. the underlying dermis by an inflammatory reaction. Cellular atypia may be present, making differentiation from SCC difficult. Many pathologists consider keratoacanthoma a type of low-grade SCC.[8] Treatment may speed recovery, limit scarring, and confirm the diagnosis. Complete excision is recommended because an invasive variant exists, with the potential for perineural and intramuscular spread.[8] Both radiotherapy and intralesional fluorouracil have been advocated.[9] [10] Actinic Keratosis Actinic keratosis, also known as solar or senile keratosis, is not truly a benign condition, being the most common premalignant skin lesion. The lesions develop on sun-exposed areas and commonly affect the face, hands, and scalp and, less commonly, the eyelid. They usually appear as multiple, flat-topped papules with an adherent white scale. The development of SCC in untreated lesions reportedly ranges as high as 20% (see Chapter 93 ).[11] Microscopically, actinic keratoses display hyperkeratosis, parakeratosis, and dyskeratosis. Epidermal atrophy, or thinning, often is present. Atypical keratinocytes in the deep epidermal layers often form buds which extend into the papillary dermis. The underlying dermis usually contains chronic inflammation. Management is surgical excision or cryotherapy (following biopsy). Epidermal Inclusion Cyst Epidermal inclusion cysts appear as slow-growing, round, firm lesions of the dermis or subcutaneous tissue. Eyelid lesions are usually solitary, mobile, and less than 1?cm in diameter. These Figure 92-6 Epidermal inclusion cyst. This lesion appeared as a slow-growing, cystic lesion in a region of previous penetrating trauma. cysts may be congenital in origin or may arise from traumatic implantation of surface epidermis ( Fig. 92-6 ). Cysts may become infected or may rupture, producing a surrounding foreign body granulomatous reaction. Diagnosis is based on the clinical appearance and histopathology. Differential diagnosis includes dermoid cyst, pilar cyst, and neurofibroma. Microscopically, the cyst is filled with keratin and is lined by a keratinizing, stratified squamous epithelium. Adnexal structures are not present in the cyst wall.[12] Treatment is complete excision, preferably of the entire cyst wall, to prevent recurrence. Pilar Cyst Pilar cysts, formerly known as sebaceous cysts, are smooth, round, movable dermal or subcutaneous masses, clinically identical to epidermal inclusion cysts. The differentiation within these cysts is thought to be toward hair keratin.[13] These cysts tend to occur in areas with large numbers of hair follicles and are found most commonly on the scalp. They may occur occasionally in the periocular region, particularly in the brow or along the eyelid margin. Histopathology reveals an epithelium-lined cyst, with palisading of the basal layer. The lining lacks a granular layer, unlike that of epidermal cysts. Eosinophilic material within the cyst comprises desquamated cells and keratin, and commonly calcifies. Cyst rupture may occur and incite a foreign body granulomatous response. Treatment is complete surgical excision— incomplete excision may result in recurrence. Epidermoid and Dermoid Cysts Although generally considered in discussions of orbital lesions (see Chapter 94 ), epidermoid and dermoid cysts are included here because they may appear as an eyelid mass. These cysts can occur as superficial, subcutaneous, or deep orbital lesions. Both are choristomas that are firm, slowly enlarging, nontender masses, most commonly in the lateral upper eyelid and brow region ( Fig. 92-7 ). Superficial lesions usually are recognized during early childhood.[14] These cysts presumably occur secondary to entrapment of skin along embryonic closure lines. Attachment to underlying bony sutures often is present, most commonly the frontozygomatic suture (see Chapter 82 ). Lesions may extend posteriorly into the orbit. Diagnosis is suspected clinically, confirmed after excision with histopathological examination. Microscopically, both dermoid and epidermoid cysts are lined by a stratified squamous keratinizing epithelium. Dermoid cysts also contain adnexal elements in the cyst wall, including hair follicles 701 Figure 92-7 Dermoid cyst. Cystic, subcutaneous lesion in the right upper lid and brow region, attached to the underlying frontozygomatic suture. and sebaceous and eccrine glands; these elements are lacking in epidermoid cysts. Treatment is complete surgical excision. Preoperative orbital imaging is indicated if the entire cyst cannot be palpated or if orbital extension is suspected. Complete excision eliminates the potential for cyst rupture, which can produce secondary foreign body granulomatous inflammation. Nevus Verruca Nevus verruca, or linear epidermal nevus, is a flesh-colored lesion composed of a linear series of hyperkeratotic papules. This localized form usually appears at birth or early in life. A generalized, widespread form of nevus verruca also exists, which may be associated with skeletal and central nervous system abnormalities. [15] Diagnosis is based on the typical clinical appearance and biopsy findings. Histopathology reveals papillomatosis, hyperkeratosis, and acanthosis of the epidermis, with no dermal involvement. Treatment modalities include surgical excision, cryotherapy, electrodesiccation, and dermabrasion. Nevus Sebaceus of Jadassohn The nevus sebaceus of Jadassohn may be confused clinically with linear epidermal nevus. The nevus sebaceus typically is found on the face during early childhood and appears as a well-defined, yellow, papillomatous lesion, which typically enlarges during adolescence. Initially it is characterized by epithelial hyperplasia, but subsequent hyperplasia of the adnexal structures occurs. Secondary tumor formation often develops during adulthood. Syringocystadenoma papilliferum, a benign tumor of apocrine origin, may appear.[16] BCC and SCC have been associated rarely with nevus sebaceus. ADNEXAL TUMORS Lesions of adnexal origin arise from the epidermal appendages, which include the sebaceous glands of Zeis, meibomian glands, pilosebaceous units (consisting of hair follicles and associated sebaceous glands), eccrine sweat glands, and apocrine sweat glands of Moll. Benign Lesions of Sebaceous Origin Sebaceous lesions of the eyelid may arise from several sources: the glands of Zeis, found in association with the eyelashes; the meibomian glands, located within the fibrous tarsal plates; and sebaceous glands, associated with hair follicles of the eyebrows and on the cutaneous surfaces of the eyelids. The sebaceous glands create their secretions by a holocrine mechanism, in which the central cells undergo disintegration and subsequent extrusion into a common excretory duct. Figure 92-8 Milia. Multiple, small, white lesions that affect the upper and lower eyelids. MILIA. Milia form as multiple, firm, white lesions which range from 1–4?mm in diameter. They usually appear on the face and commonly affect the eyelids, nose, and malar region ( Fig. 92-8 ). Lesions may occur spontaneously or secondarily due to trauma, radiotherapy, skin infection, or bullous diseases. Occlusion of pilosebaceous units with retention of keratin is thought to be the causative mechanism. Histopathology reveals a dilated, keratin-filled hair follicle, with compression and atrophy of the adjacent sebaceous glands.[17] Treatment includes simple incision, electrodesiccation of the surface, or puncture and expression of the contents. ACQUIRED SEBACEOUS GLAND HYPERPLASIA. This lesion occurs predominantly in elderly patients, usually on the skin of the face and scalp. Skin lesions appear as multiple, small, yellow, slightly umbilicated papules. Individual lesions may resemble BCC. The meibomian glands may also be affected, to produce nodular thickening of the lids. Sebaceous carcinoma must be considered (see Chapter 93 ). Microscopically, numerous sebaceous gland lobules are grouped around a central dilated duct. Multiple management options exist, including excision, cryotherapy, and carbon dioxide laser. SEBACEOUS ADENOMA. This uncommon lesion usually appears in the elderly as a solitary, yellow papule, with a predilection for the eyelid and brow. The importance of this and other benign sebaceous neoplasms is the association with internal malignancy, known as the Muir–Torre syndrome. Even a single cutaneous sebaceous neoplasm may be significant, so patients should be evaluated accordingly.[18] Patients with this syndrome also may develop multiple keratoacanthomas. Microscopically, the sebaceous adenoma is a well-circumscribed lesion, with lobules containing an outer layer of basal germinal cells, which become lipidized centrally. Treatment is complete surgical excision, because incompletely excised lesions commonly recur. Benign Lesions of Eccrine Origin The eccrine sweat glands are found throughout the cutaneous surface of the eyelids. They are composed of three segments, including an intradermal secretory coil, an intradermal duct, and an intraepidermal duct. ECCRINE HIDROCYSTOMA. Eccrine hidrocystomas, also known as sudoriferous or sweat gland cysts, appear as solitary or multiple, small nodules on the eyelids. The overlying skin is shiny and smooth, and the cyst usually is translucent and fluid filled ( Fig. 92-9 ). Eccrine hidrocystomas are thought to be ductal retention cysts, which tend to increase in size in hot, humid weather. The differential diagnosis includes apocrine hidrocystoma and epidermal inclusion cyst. Histopathology reveals a dermal cyst lined by a double-layered cuboidal epithelium without papillary infoldings. Treatment is complete excision. 702 Figure 92-9 Eccrine hidrocystoma. Cystic lesion involving the left lower eyelid margin. The lesion was filled with translucent fluid. SYRINGOMA. The syringoma is a common adnexal tumor arising from adenomatous proliferation of the intraepidermal duct of eccrine glands. They occur primarily in young females, occurring as multiple, small (1–3?mm diameter), skin-color to yellowish papules distributed symmetrically on the lower eyelids and cheeks. Microscopically, syringomas contain ducts lined by double-layered cuboidal epithelium, embedded in a dense fibrous stroma. The ducts may taper to a solid core of cells, to produce a comma-shaped or “tadpole” configuration.[19] Treatment modalities include surgical excision, electrodesiccation, and carbon dioxide laser.[20] CHONDROID SYRINGOMA. Chondroid syringoma, also known as a pleomorphic adenoma or mixed tumor of the skin, most commonly occurs in the head and neck region and, rarely, may involve the eyelid. [21] It appears as a 0.5–3?cm in diameter, asymptomatic, dermal nodule. The lesions are thought to arise from eccrine sweat glands and owe their name to the mixture of sweat gland and cartilaginous elements. Differential diagnosis includes epidermal inclusion cyst, pilar cyst, neurofibroma, and pilomatrixoma. Microscopically, it is identical to pleomorphic adenoma of the lacrimal gland. Ducts lined with an inner secretory layer and an outer myoepithelial layer are embedded in a stroma with areas of chondroid metaplasia. Treatment is surgical excision. Malignant variants have been reported. ACROSPIROMA. Acrospiroma, also known as clear-cell hidradenoma, appears as a solid or cystic subcutaneous nodule, with flesh-colored or erythematous overlying skin which may ulcerate.[22] Acrospiroma occurs in young adults and may appear at any cutaneous site, occasionally on the eyelid. Lesions may resemble keratoacanthoma. Pain may be elicited in approximately 20% of cases on applying pressure to the lesion. Histopathology reveals a well-demarcated dermal mass with ductal and secretory elements. Large, glycogen-rich clear cells and polyhedral cells with eosinophilic cytoplasm are present in the solid portions of the tumor.[23] Treatment is surgical excision. Malignant variants are reported to exist. Benign Lesions of Apocrine Origin The apocrine glands of Moll are found along the eyelid margin in association with the eyelash follicles. They are modified sweat glands which contain a secretory coil, an intradermal duct, and an intraepithelial duct. Their secretions are produced by decapitation of the secretory cells. APOCRINE HIDROCYSTOMA. Apocrine hidrocystoma, also known as cystadenoma, usually appears as a solitary, translucent cyst on the face, sometimes at the eyelid margin. The cyst is usually small (less than 1?cm in diameter) and filled with clear or milky fluid, with shiny, smooth overlying skin ( Fig. 92-10 ). Lesions may display a bluish coloration, attributed to the Tyndall effect. Unlike the eccrine variety, these lesions are thought to be proliferative in origin and do not increase in size in hot weather. Figure 92-10 Apocrine hidrocystoma. Cystic lesion, filled with milky fluid, involving the right lower eyelid margin. Figure 92-11 Cylindroma. Multiple, pinkish-red dermal nodules involving the eyelids, forehead, nose, and malar region. The differential diagnosis includes eccrine hidrocystoma and cystic BCC. An association has been reported, thought to represent an ectodermal dysplasia, in which patients display multiple apocrine hidrocystomas, hypodontia, palmar–plantar hyperkeratosis, and onychodystrophy.[24] Histopathology reveals a dermal cyst with papillary infoldings, lined by an inner secretary layer with eosinophilic columnar cells and an outer myoepithelial layer. Treatment is complete excision. CYLINDROMA. Cylindroma, presumably of apocrine origin, may occur on the eyelid or brow. It usually appears as a dome-shaped, skin-colored, or pinkish-red, dermal nodule ( Fig. 92-11 ). Solitary lesions usually occur in adulthood in the head and neck region and may appear similar to a pilar or epidermal inclusion cyst. Multiple lesions are inherited in an autosomal dominant fashion and usually appear on the scalp, where extensive involvement is referred to as a turban tumor. Multiple lesions have been associated with trichoepitheliomas. Microscopically, the cylindroma consists of islands with large, pale-staining cells centrally and small, cuboidal cells peripherally, surrounded by an eosinophilic basement membrane. Treatment is surgical excision. Benign Lesions of Hair Follicle Origin Benign lesions of hair follicle origin are rather rare tumors, often confused clinically with BCC, the most common malignant eyelid lesion. Confirmation of diagnosis by incisional biopsy is helpful for suspicious-looking lesions, which allows less extensive resection of lesions confirmed as benign.[25] 703 Figure 92-12 Pilomatrixoma. Reddish nodule arising from the left lower eyelid. TRICHOEPITHELIOMA. Trichoepithelioma is a tumor of hair follicle origin with a predilection for the face, including the eyelids and forehead. The solitary lesion tends to occur in older individuals as an asymptomatic, flesh-colored to yellowish, firm papule that rarely ulcerates. Clinical differentiation from BCC and other adnexal tumors may be difficult to make. Multiple lesions, also known as multiple benign cystic epithelioma or Brooke’s tumor, are inherited in an autosomal dominant pattern with variable penetrance. Lesions appear during adolescence as multiple firm nodules involving the face, and also the scalp, neck, and trunk. They may increase in size and number but rarely ulcerate. Diagnosis is made by the clinical appearance, family history, and histopathology. Differential diagnosis includes basal cell nevus syndrome, in which lesions tend to ulcerate more frequently (see Chapter 93 ). Histopathology reveals multiple, keratin-filled horn cysts surrounded by islands of basaloid cells which display peripheral palisading. The abundant fibrous stroma is well demarcated from the surrounding dermis. Lesions may histologically resemble BCC, and rare reports of transformation to BCC exist.[26] Treatment includes surgical excision of solitary lesions and cryosurgery or laser for multiple lesions. TRICHOFOLLICULOMA. Trichofolliculoma is a fairly well-differentiated hamartomatous lesion, usually appearing as an asymptomatic, solitary, flesh-colored nodule during adulthood on the face or scalp. A central umbilication usually is present, which is the opening of a keratin-filled follicle. Small white hairs may protrude from the central pore and are suggestive of the diagnosis. The lesion may be confused clinically with a pilar cyst, nevus, or BCC.[17] Histopathology reveals a dilated follicle, filled with keratin and hair shafts, and lined by stratified squamous epithelium continuous with the epidermis. Surgical excision is curative. TRICHILEMMOMA. Trichilemmoma is a tumor which arises from the outer hair sheath. A solitary lesion generally appears during adulthood as an asymptomatic, flesh-colored, nodular, or papillomatous lesion. The nose is the most common site of occurrence, followed by the eyelid and the brow.[27] The lesion may appear as a cutaneous horn or may resemble verruca vulgaris or BCC. Multiple trichilemmomas are a marker for Cowden’s disease, or multiple hamartoma syndrome, a rare genodermatosis inherited in an autosomal dominant fashion. In addition to the facial trichilemmomas, patients may develop acral keratoses and oral papillomas. Patients are at increased risk of developing breast and thyroid carcinoma, as well as multiple hamartomas. The mucocutaneous lesions usually precede the onset of malignancy.[28] Microscopically, glycogen-rich cells with clear cytoplasm proliferate in lobules, with peripheral palisading and a distinct basement membrane. Hair follicles may be present. Treatment is surgical excision, cryosurgery, or laser. PILOMATRIXOMA. The pilomatrixoma, also known as the calcifying epithelioma of Malherbe, is a benign tumor of hair matrix origin.[29] The lesion tends to occur in children and young adults on the head and upper extremities. Lesions may occur in the periorbital region, particularly the upper eyelid and brow. [30] Usually a solitary lesion, it appears as a solid or cystic, mobile, subcutaneous nodule with normal overlying skin. It is firm, irregular, often reddish blue, and may contain chalky white nodules ( Fig. 92-12 ). Histopathology reveals islands of basophilic epithelial cells, which transform into shadow cells located more centrally. Most tumors contain masses of calcified shadow cells, which may incite a giant cell granulomatous response. Rare cases of malignant transformation have been reported. Treatment is surgical excision. VASCULAR TUMORS Capillary Hemangioma The capillary hemangioma, also known as a benign hemangioendothelioma, is a common vascular lesion of childhood, which occurs in 1–2% of infants and is the most common orbital tumor found in children. Girls are more commonly affected than boys, with a 3:2 ratio. A periorbital hemangioma may appear as a superficial cutaneous lesion, subcutaneous lesion, deep orbital tumor, or combination of these types. Approximately one third of lesions are visible at birth, with the remainder manifest by 6 months of age. There is typically an initial rapid growth phase within 6 months of diagnosis, followed by a period of stabilization and subsequent involution over several years. It is estimated that approximately 75% regress to some extent by the time the child reaches 7 years of age. The classic superficial lesion, the strawberry nevus, appears as a red, raised, nodular mass which blanches with pressure ( Fig. 92-13 ). It may first be seen as a flat lesion with telangiectatic surface vessels. A subcutaneous lesion appears as a bluish-purple, spongy mass. Deep orbital lesions may cause proptosis and globe displacement, with no associated cutaneous findings. The most common ocular complication is amblyopia, which may result from occlusion of the visual axis due to eyelid involvement, or from anisometropia due to induced astigmatism. Strabismus may occur secondary to the amblyopia or be caused by orbital involvement with restriction of ocular motility.[31] Lesions that involve the eyelid and anterior orbit usually can be diagnosed by clinical findings. Cutaneous lesions may be confused with nevus flammeus, which is usually flatter, darker, and does not blanch with pressure. The differential diagnosis of orbital lesions includes rhabdomyosarcoma, neuroblastoma, encephalocele, lymphangioma, and inflammatory masses (see Chapter 95 ). Ultrasonography, computed tomography, and magnetic resonance imaging may aid in diagnosis and in determining the extent of involvement (see Chapter 84 ). Biopsy may be needed for patients who have a rapidly expanding orbital mass or for those in whom noninvasive methods are not diagnostic. Systemic complications are rare in isolated periorbital lesions. However, patients who have extensive visceral hemangiomas may develop thrombocytopenia related to entrapment of platelets within the lesion, with resulting hemorrhagic diathesis, known as the Kasabach–Merritt syndrome. Patients with orbital hemangiomas have a predilection for noncontiguous hemangiomas in the head and neck region. Microscopically, the early proliferative phase of the lesion contains lobules of plump endothelial cells separated by fibrous septa, with frequent mitotic figures and small, irregular vascular lumina. Mature lesions contain more prominent vascular structures and flatter endothelial cells diminished in number. As regression takes place, progressive fibrosis occurs, with thickening of the fibrous septa and replacement of endothelial lobules by adipose tissue. Atrophy of the vascular component of the lesion eventuates. Because most capillary hemangiomas undergo spontaneous regression to some extent, treatment generally is reserved for patients who have specific ocular, dermatologic, or systemic indications 704 Figure 92-13 Capillary hemangioma. A, Superficial, raised, red mass involving the right upper eyelid and medial canthal region. B, High magnification of endothelial cells. (B, From Yanoff M, Fine BS. Ocular pathology, ed 5. St. Louis: Mosby; 2002.) for intervention. Various management modalities have been advocated, each with potential significant risks which are beyond the scope of this discussion. Ocular indications include amblyopia, compressive optic neuropathy, and proptosis with globe exposure. Treatment modalities include intralesional corticosteroid injection,[32] [33] systemic corticosteroids, radiotherapy, laser therapy, systemic interferon, and surgery. Intralesional corticosteroid injection is the favored management, especially for cutaneous hemangiomas. Surgery should be considered for localized, noninfiltrative lesions, or for those which fail to respond to corticosteroids.[34] Amblyopia should be treated with appropriate patching and spectacle correction, as indicated. Cavernous Hemangioma Cavernous hemangioma is the most common benign orbital tumor of adults (only occasionally occurring as a primary eyelid lesion); the lesions usually appear during adulthood and normally do not undergo spontaneous regression. Superficial skin lesions are dark blue, compressible and, unlike the orbital variety, not encapsulated. The differential diagnosis includes lymphangioma, with associated hemorrhage, and varices, which are formed by dilatation of preexisting vascular channels. A rare syndrome exists, termed the blue rubber bleb nevus syndrome, which is characterized by multiple cutaneous lesions consistent with cavernous hemangiomas, associated with gastrointestinal hemangiomas which often bleed.[35] Histologically, cavernous hemangiomas contain dilated, endothelium-lined vascular spaces, often with thrombosis and phlebolith formation. Treatment is surgical excision. Figure 92-14 Nevus flammeus. Flat, purple, vascular lesion involving the skin of the face. Lymphangioma Lymphangiomas may involve the eyelid, conjunctiva, or orbit.[36] Lesions often appear at birth or early in childhood, and only occasionally in adulthood. They often are poorly circumscribed, with an infiltrative growth pattern. Eyelid involvement may occur as a superficial lesion with multiple cyst-like excrescences, or as a complex of channels that cause lid thickening and distortion. Hemorrhage into the lesion may occur, to produce a hematoma when the eyelid is involved or proptosis when orbital lesions are present (see Chapter 95 ). Biopsy may be needed for definitive diagnosis. Microscopically, dilated, thin-walled vascular spaces lined by endothelial cells are present. Lymphoid aggregates may be present which, in the context of orbital lesions, may undergo proliferation associated with upper respiratory infections and cause worsening proptosis. Surgical excision is indicated for optic nerve compromise in orbital lesions, cosmesis, or eyelid malposition. Large lesions may be difficult to manage due to extensive infiltration. Carbon dioxide laser is a useful modality when excision is required. Nevus Flammeus Nevus flammeus, also known as a port-wine stain, presents as a flat, purple, vascular lesion, usually unilateral and in the distribution of a branch of the trigeminal nerve ( Fig. 92-14 ). It is congenital and does not undergo spontaneous regression. If associated with ocular and leptomeningeal vascular hamartomas, it represents the Sturge–Weber syndrome. Ocular manifestations of this syndrome include diffuse choroidal hemangioma, ipsilateral glaucoma, and serous retinal detachment. Histopathology of the skin lesion reveals dilated, telangiectatic capillaries within the dermis. Management is primarily with cosmetics. Tunable dye laser therapy also may be used to improve the appearance of the lesion.[37] Pyogenic Granuloma Pyogenic granuloma is the most common acquired vascular lesion to involve the eyelids.[17] It usually occurs after trauma or surgery as a fast-growing, fleshy, red-to-pink mass, which readily bleeds with minor contact ( Fig. 92-15 ). Lesions also may develop in association with inflammatory processes, including chalazia. The differential diagnosis includes Kaposi’s sarcoma and intravascular papillary endothelial hyperplasia, a rare endothelial proliferation. Microscopically, there is granulation tissue consisting of fibroblasts and blood vessels, with acute and 705 Figure 92-15 Pyogenic granuloma. A, Red mass arising from the palpebral conjunctiva and protruding over the eyelid margin. This lesion developed in association with a chalazion. B, Vascularized tissue (granulation tissue) that consists of inflammatory cells (polymorphonuclear lymphocytes and fibroblasts) and the endothelial cells of budding capillaries. chronic nongranulomatous inflammatory cells. Notably, the lesion is neither pyogenic nor granulomatous. Treatment is by surgical excision at the base of the lesion. TUMORS OF NEURAL ORIGIN Neurofibroma Neurofibromas most commonly are considered in the context of neurofibromatosis, in which patients often develop multiple cutaneous lesions in association with other stigmata of the disease, usually apparent by adolescence.[38] The neurofibromas may occur on any cutaneous surface, including the eyelid, and typically enlarge slowly over many years. They appear as soft, fleshy, often pedunculated masses ( Fig. 92-16 ). Isolated cutaneous neurofibromas, often resembling intradermal nevi, also may occur in individuals with no other associated abnormality. The plexiform neurofibroma, characteristic of type 1 neurofibromatosis, often occurs as a diffuse infiltration of the eyelid and orbit. The upper eyelid is usually ptotic, with an S-shaped curvature ( Fig. 92-17 ). On palpation, the lesion feels like a “bag of worms.” Histopathology reveals units of proliferating axons, Schwann cells, and fibroblasts, with each unit surrounded by a perineural sheath. Management depends on the site and extent of disease. Isolated cutaneous lesions, unrelated to neurofibromatosis, may Figure 92-16 Neurofibroma. Note the fleshy mass on the eyelid of this patient with disseminated cutaneous neurofibromas. Figure 92-17 Plexiform neurofibroma. Note the ptosis and typical S-shaped curvature of the upper lid. be excised surgically. Surgical debulking may be performed for plexiform neurofibromas which produce mechanical ptosis or cosmetic deformity. However, due to the infiltrative nature of these lesions, complete excision is usually impossible and recurrence is common. XANTHOMATOUS LESIONS Xanthomatous lesions are characterized by the presence of histiocytes which have accumulated lipid, resulting in a foamy appearance of the cytoplasm histologically. Xanthelasma Xanthelasma palpebrarum is the most common cutaneous xanthoma, typically occurring in middle-aged and older adults as soft, yellow plaques on the medial aspect of the eyelids ( Fig. 92-18 ). The diagnosis often can be made clinically. Hyperlipidemia is reported to occur in approximately 50% of patients with xanthelasma,[39] [40] therefore screening is recommended.[41] Type IIa is the most commonly associated hyperlipidemia. The differential diagnosis of atypical lesions includes Erdheim–Chester disease, a systemic xanthogranulomatous disorder which has lesions that typically appear more indurated. Microscopically, xanthelasmas are composed of foamy, lipid-laden histiocytes (xanthoma cells) clustered around blood vessels and adnexal structures within the superficial dermis. Surrounding fibrosis and inflammation may be observed. Treatment modalities include surgical excision, carbon dioxide laser ablation, and topical trichloroacetic acid. Recurrence is common. 706 Figure 92-18 Xanthelasma. Multiple, soft, yellow plaques involving the lower eyelid. Lipid-laden foam cells seen in dermis and tend to cluster around blood vessels. Fibrous Histiocytoma Fibrous histiocytomas, also known as fibrous xanthomas or dermatofibromas, have a predilection to occur in the orbit but occasionally may be found on the eyelid.[42] The eyelid lesion appears as an asymptomatic, subcutaneous, mobile mass.[43] Histopathology reveals a poorly demarcated lesion, with a mixture of fibroblastic spindle cells forming a characteristic storiform pattern and large foamy histiocytes. Multinucleated giant cells, lymphocytes, and other inflammatory cells are found throughout the lesion, also. Complete surgical excision is recommended. Juvenile Xanthogranuloma Juvenile xanthogranuloma (JXG), also known as nevoxanthoendothelioma, is a benign histiocytic proliferation which most commonly affects the skin. It occurs mainly in children less than 2 years of age and usually appears within the first year of life. The skin lesions most commonly appear in the head and neck region as elevated orange, red, or brown nodules, which are generally self-limited. They typically increase in size and number initially but subsequently regress spontaneously into an atrophic scar over months to years. Lesions which appear in adulthood are more likely to persist and often require treatment to induce regression. The most common site of extracutaneous involvement is the eye, with a predilection for the iris.[44] The iris may contain localized vascular nodules or diffuse infiltration of tumor. Complications include hyphema, uveitis, and glaucoma, with resulting visual loss and phthisis. Treatment, which includes topical and subconjunctival corticosteroids, is recommended for intraocular lesions,[45] because they rarely regress spontaneously and complications are common. Biopsy of skin lesions helps to confirm the clinical diagnosis in patients who have cutaneous disease alone and in patients who have suspicious eye findings associated with skin lesions. Microscopically, lesions contain an infiltrate of lipid-laden histiocytes, lymphocytes, eosinophils, and Touton giant cells. Fibrosis appears in older lesions. Skin lesions may be treated by excision or, if necessary, corticosteroid injection. PIGMENTED LESIONS OF MELANOCYTIC ORIGIN Skin lesions of melanocytic origin arise from one of three cell types: • Epidermal, or dendritic, melanocytes which lie between the basal cells of the epidermis • Nevus cells, or nevocytes, which usually form nests of cells within the epidermis • Dermal, or fusiform, melanocytes which lie in the subepithelial tissues Figure 92-19 Freckles. Multiple, tan–brown, small macules, involving the skin of sun-exposed areas. Melanocytes are derived from neural crest cells. Epidermal melanocytes produce melanin, which is transferred to surrounding epidermal cells, with tanning and racial pigmentation resulting from this process. Freckles Freckles, also known as ephelides, arise from epidermal melanocytes. They appear as small (1–3?mm diameter), tan-to-brown macules in sun-exposed areas, including the eyelids ( Fig. 92-19 ). Freckles occur more commonly in light-complected individuals and darken with sun exposure. These lesions reflect melanocytic overactivity, not proliferation. Microscopically, hyperpigmentation occurs within the basal layer of the epidermis. No treatment is necessary, but sunscreen may help prevent further darkening of lesions. Lentigo Simplex Lentigo simplex is another epidermal melanocytic lesion which may appear on skin and mucous membranes as small, brown macules. They usually appear during childhood and are unaffected by sun exposure. Lesions may be solitary and have an appearance similar to that of junctional nevi. Multiple lesions may be a manifestation of a systemic syndrome, such as Peutz–Jeghers syndrome. Patients who have this syndrome develop multiple lesions, often periocular and perioral in distribution, in association with gastrointestinal polyps,[46] which may undergo malignant transformation. Multiple lesions may resemble freckles but do not change in pigmentation with sun exposure as freckles often do. Microscopically, lentigo simplex has hyperpigmentation along the basal layer of the epidermis, with an increased number of melanocytes. Elongation of the rete ridges occurs along with mild lymphocytic infiltration of the superficial dermis. Intervention is not required, because these lesions are thought to have no malignant potential. Solar Lentigo Lesions of solar lentigo, also of epidermal melanocytic origin, are tan-to-brown macules found commonly in sun-exposed areas 707 of older individuals. They also are known as senile lentigines but may occur in younger individuals after prolonged sun exposure. These lesions also are found commonly in patients who have xeroderma pigmentosum, often appearing during the first decade of life. Lesions usually have slightly irregular borders but are evenly pigmented. Initially, lesions are a few millimeters in diameter but slowly increase in size. They may resemble junctional nevi and seborrheic keratoses. Lesions should be differentiated from lentigo maligna, a premalignant condition, which usually has variable pigmentation and more prominent border irregularity and notching. Biopsy should be performed on suspicious-looking lesions. Histologically, solar lentigo lesions display hyperpigmentation of the basal layer of the epidermis, with proliferation of melanocytes. More extensive elongation of the rete ridges is found in comparison with lentigo simplex. Treatment is not required, unless desired for cosmetic reasons. Melanocytic Nevi Melanocytic nevi, also known as nevocellular nevi, are derived from nevocytes. They are extremely common lesions, especially in fair-complected individuals.[47] These lesions frequently occur on the eyelid skin and eyelid margin. The clinical appearance often is predictive of the histological type, which may be junctional, compound, or intradermal. Lesions typically occur during childhood as small, flat, tan macules which gradually increase in size radially. Nests of nevus cells are found within the epidermis, at the dermal–epidermal junction, representing a junctional nevus. As the lesion ceases to increase in diameter in older children and young adults, nests of cells “drop off” into the dermis, forming a compound nevus. Clinically, compound nevi are slightly elevated and pigmented. Lesions further evolve as the remaining epidermal nests migrate into the dermis, to produce an intradermal nevus. This lesion, most common in adults, may be dome-shaped, pedunculated, or papillomatous, and usually is less pigmented or amelanotic ( Fig. 92-20 ). Later in life, as the nevus cells induce fibroplasia within the dermis, the cells decrease in number and are replaced by normal dermal tissue. Diagnosis usually is based on the typical clinical appearance. Malignant transformation may occur rarely, generally in the junctional or compound stages. Thus, suspicious-looking lesions that demonstrate irregular growth or appearance should be excised. Otherwise, removal of common nevi is not required, unless desired for cosmesis or relief of mechanical irritation. Congenital Melanocytic Nevus These lesions are derived from nevocytes and occur in approximately 1% of newborns. Lesions may be single or multiple, and usually are deeply pigmented. The border often is irregular and the surface may be covered with hair. Congenital nevi that appear in a symmetrical fashion on adjacent portions of the upper and lower eyelids are referred to as kissing nevi and are formed as a result of melanocytic migration to the lids prior to separation of the embryonic eyelids ( Fig. 92-21 ). The size of congenital nevi is critical in management, because large lesions are associated with a higher risk of malignant transformation. Controversy exists regarding the definition of “large” and “small” congenital nevi. Large lesions in the head and neck region commonly are defined as those greater than or equal to the area of the patient’s palm. The risk of malignant transformation is estimated at 5%.[48] Histologically, congenital nevi display a variety of patterns. Many lesions contain features of compound nevi, with nevus cells in the dermis and the dermal–epidermal junction. Nevus cells often extend into the deep dermis and subcutaneous tissue. Malignant melanoma usually develops within the deep dermis, which makes early diagnosis difficult. Thus, any suspicious-looking Figure 92-20 Intradermal nevus. A, Elevated, papillomatous lesion, amelanotic in color, involving the eyelid margin. B, Nests of nevus cells fill the dermis except for a narrow area just under the epithelium. The nuclei of the nevus cells become smaller, thinner or spindle-shaped, and darker as they go deeper into the dermis (i.e., they show normal polarity). (B, From Yanoff M, Fine BS. Ocular pathology, ed 5. St. Louis: Mosby; 2002.) Figure 92-21 Kissing nevus. Congenital melanocytic nevus in a symmetrical fashion on adjacent portions of the upper and lower eyelids. lesion should be sampled for biopsy. Large lesions should be excised, but complete excision is impossible in some patients due to the size and extent of the lesion. The management of small lesions is controversial—some advocate excision of all congenital nevi.[49] Spindle–Epithelioid Cell Nevus Another variant of nevocellular nevi, the spindle–epithelioid cell nevus, also has been termed benign juvenile melanoma and 708 Spitz nevus. It appears as a pink-to-orange, dome-shaped nodule in children and young adults. Microscopically, this is a compound nevus, with spindle or epithelioid cells throughout the epidermis and dermis. Lesions may contain mitotic figures and nuclear atypia, making histological differentiation from malignant melanoma difficult. Excision provides definitive diagnosis and treatment. Balloon Cell Nevus A third variant of nevocellular nevi is the balloon cell nevus. It appears as a lightly pigmented, elevated lesion, usually less than 5?mm in diameter. No clinically distinguishing features help to differentiate this lesion from other nevocellular nevi.[13] Histologically, the lesion contains balloon cells, which are larger than the nevus cells of typical nevocellular nevi and contain small, pyknotic nuclei and granular or vacuolated cytoplasm. Nevus of Ota Nevus of Ota, or oculodermal melanocytosis, arises from dermal melanocytes. The lesion appears as a blue-to-purple, mottled discoloration of the skin in the distribution of the ophthalmic and maxillary divisions of the trigeminal nerve. It is usually congenital and unilateral and frequently is associated with ipsilateral ocular melanocytosis involving the conjunctiva, sclera, and uveal tract. Diagnosis is based on the typical clinical appearance. Histopathology reveals pigmented, dendritic melanocytes throughout the dermis. Malignant degeneration may occur, particularly in Caucasians, with the choroid the most common site of involvement.[50] Periodic dilated fundus examination, thus, is recommended. Blue Nevus The blue nevus appears as a solitary blue nodule, usually less than 1?cm in diameter. The differential diagnosis includes melanoma, pigmented BCC, and vascular lesions. Microscopically, the lesion is composed of pigmented dendritic melanocytes and melanophages scattered throughout the dermis, often with fibrosis of adjacent tissue. The cellular blue nevus is a lesion which also arises from dermal melanocytes. It is less common and usually larger than the blue nevus, and appears as a solitary blue papule. Histologically, the lesion contains pigmented dendritic melanocytes interspersed with pale spindle cells. This lesion occasionally may metastasize to regional lymph nodes. Excision of these lesions may be performed for definitive diagnosis or cosmesis. INFLAMMATORY LESIONS Chalazion A chalazion is a focal inflammatory lesion of the eyelid which results from the obstruction of a sebaceous gland, either meibomian or Zeis. Extravasated lipid material produces a surrounding chronic lipogranulomatous inflammation. A chalazion may occur acutely with eyelid edema and erythema and evolve into a nodule, which may point anteriorly to the skin surface or, more commonly, through the posterior surface of the lid. The lesion may drain spontaneously or persist as a chronic nodule, usually a few millimeters from the eyelid margin. Lesions also may appear insidiously as firm, painless nodules ( Fig. 92-22 ). Large lesions on the upper lid may even induce astigmatism. Chalazia often occur in patients with blepharitis and rosacea. Diagnosis is based on the typical clinical features. Acute lesions appear similar to hordeola in appearance—differentiation is nearly impossible to make clinically. In recurrent lesions, a sebaceous gland carcinoma needs to be excluded; thus, histopathological examination is important. Histopathology reveals Figure 92-22 Chalazion and external hordeolum. A, The medial lesion of the upper eyelid appeared as a firm, painless nodule, consistent with a chalazion. The lateral lesion caused pain and eyelid erythema, subsequently becoming more localized, with drainage of purulent material through the skin surface. B, A clear circular area surrounded by epithelioid cells and multinucleated giant cells can be seen. In processing the tissue, lipid is dissolved out, leaving a clear space. lipogranulomatous inflammation, with clear spaces corresponding to lipid, surrounded by foreign body giant cells, epithelioid cells, neutrophils, lymphocytes, plasma cells, and eosinophils. A fibrous pseudocapsule may form around a lesion. Treatment varies according to the stage of a lesion. Acute lesions are treated with hot compresses to encourage localization and drainage. Chronic chalazia may be treated using intralesional corticosteroid injection or surgical drainage. Vertical transconjunctival incisions allow adequate exposure of lesions and limit damage to surrounding meibomian glands.[51] Small chalazia, which may resolve spontaneously, can be removed with incision and curettage. Hordeolum A hordeolum is an acute purulent inflammation of the eyelid. An external hordeolum, or stye, results from infection of the follicle of a cilium and the adjacent glands of Zeis or Moll. The lesion typically causes pain, edema, and erythema of the eyelid, which becomes localized and often drains anteriorly through the skin near the lash line (see Fig. 92-22 ). An internal hordeolum occurs due to obstruction and infection of a meibomian gland. Initially, a painful edema and erythema localizes as an inflammatory abscess on the posterior conjunctival surface of the tarsus. In both external and internal lesions, cellulitis of the surrounding soft tissue may develop. Diagnosis is based on the 709 clinical appearance and culture, with Staphylococcus aureus most frequently isolated. Hordeola frequently occur in association with blepharitis. Histopathology reveals an abscess or a focal collection of polymorphonuclear leukocytes and necrotic tissue. Although the inflammatory process usually is self-limited, with drainage and resolution occurring within 5–7 days, hot compresses and topical antibiotics help confine the spread of the lesion. Rarely, incision and drainage are necessary. Systemic antibiotics are used only if significant cellulitis exists. Treatment of accompanying blepharitis is helpful to prevent the formation of new lesions. INFECTIOUS LESIONS Molluscum Contagiosum A common viral skin disease, MC is caused by a large DNA pox virus. Infection usually arises from direct contact or fomites in children and by a sexually transmitted route in adults. The typical lesion appears as a raised, shiny, white-to-pink nodule with a central umbilication filled with cheesy material. Lesions may be single or multiple, but usually fewer than 20 are present. Eyelid margin lesions may produce a follicular conjunctival reaction. Other ocular manifestations include epithelial keratitis, pannus formation, conjunctival scarring, and punctal occlusion. Primary conjunctival or limbal lesions occur rarely. Diagnosis of MC usually is based on the clinical appearance of the lesion. Biopsy rarely is required in an otherwise healthy individual. The differential diagnosis includes keratoacanthoma, verruca vulgaris, squamous papilloma, milia, and SCC or BCC (see Chapter 93 ). Patients who have acquired immunodeficiency syndrome (AIDS) often have an atypical clinical picture of MC. Disseminated disease may be present and lesions often are more confluent. Patients may have 30–40 lesions on each eyelid, or a confluent mass ( Fig. 92-23 ). Secondary keratoconjunctivitis develops less frequently. Histopathology of MC shows invasive acanthosis, with lobules of epithelial hyperplasia invaginating into the dermis. The epithelium at the surface degenerates and sloughs into a central cavity, which opens through a pore to the epidermal surface. Intracytoplasmic inclusions containing virions, referred to as molluscum bodies, are round and eosinophilic in the lower layers of the epidermis. These inclusions increase in size and are more basophilic in the granular and horny layers. Usually, MC spontaneously resolves within 3–12 months but the patient may be treated to prevent corneal complications, reduce transmission, and speed recovery. Various treatment options exist, including simple incision or excision, incision and curettage, cryosurgery, and electrodesiccation. Management is more difficult in patients with AIDS because of extensive involvement and recurrences. Hyperfocal cryotherapy has been effective in these patients.[52] Verruca Vulgaris Verruca vulgaris, or the common cutaneous wart, is caused by epidermal infection with the human papillomavirus, which is spread by direct contact and fomites. Verruca vulgaris is more common in children and young adults and may occur anywhere on the skin, occasionally on the eyelid. Lesions appear elevated with an irregular, hyperkeratotic, papillomatous surface ( Fig. 92-24 ). Lesions along the lid margin may induce a mild papillary conjunctivitis due to shedding of virus particles into the tear film. Patients also may develop a superficial punctate keratitis and may have pannus formation. Primary conjunctival lesions also may occur.[53] Diagnosis is based on the typical appearance and is confirmed by biopsy. Histologically, papillomatosis is present, with hyperkeratosis, acanthosis, and parakeratosis. Large, vacuolated keratinocytes Figure 92-23 Molluscum contagiosum. A, Multiple raised nodules, with areas of confluent lesions, affecting the eyelids of a patient with AIDS. B, Intracytoplasmic, small, eosinophilic molluscum bodies occur in the deep layers of epidermis. The bodies become enormous and basophilic near the surface. The bodies may be shed into the tear film where they cause a secondary, irritative, follicular conjunctivitis. (B, From Yanoff M, Fine BS. Ocular pathology, ed 5. St. Louis: Mosby; 2002.) Figure 92-24 Verruca vulgaris. Skin-colored, irregular lesion with a papillomatous surface appearing on the upper eyelid. are seen, with deeply basophilic nuclei surrounded by a clear halo. Observation is recommended if no ocular complications occur, because most lesions are self-limiting. Treatment, if necessary, is either cryotherapy or complete surgical excision. Incomplete excision may cause multiple recurrences. 710 CONCLUSION The eyelids may be affected by a variety of benign lesions, some indicative of local pathology, others associated with or resulting from systemic pathology. Some lesions may be identified readily by the clinical appearance and behavior. However, many pose a diagnostic challenge, because different lesions may appear similar. Also, individual types of lesions may occur in a variety of forms. Most important is the differentiation of benign from malignant lesions, because management often differs. Biopsy with ocular pathology consultation is, thus, warranted for any suspicious-looking lesion. Epithelial lesions which display painless growth, irregular or pearly borders, ulceration, induration, or telangiectasis should raise concern for malignancy. Signs that herald malignant change in pigmented lesions include irregular borders, asymmetrical shape, color change or presence of multiple colors, recent changes, or diameter greater than 5?mm. In general, biopsy should precede all extensive tumor resections, even if the clinical diagnosis seems apparent. An incisional biopsy should be performed for the diagnosis of large lesions prior to definitive therapy. Small lesions may be excised for both diagnosis and treatment. OUTCOMES Most benign eyelid lesions have an excellent prognosis. The treatment varies according to site, diagnosis, concurrent systemic involvement, and other factors. REFERENCES 1. Aurora AL, Blodi FC. Lesions of the eyelids: a clinicopathological study. Surv Ophthalmol. 1970;15:94–104. 2. Folberg R. Eyelids: terminology of eyelid pathology. In: Folberg, R. Pathology of the eye [CD-ROM]. St Louis: Mosby–Year Book; 1996. 3. Ellis DL, Yates RA. Sign of Leser–Trélat. Clin Dermatol. 1993;11:141–8. 4. Kobalter AS, Roth A. Benign epithelial neoplasms. In: Mannis MJ, Macsai MS, Huntley AC, eds. Eye and skin disease. Philadelphia: Lippincott–Raven; 1996:345–55. 5. Sanderson KV. The structure of seborrheic keratosis. Br J Dermatol. 1968;80: 588–93. 6. Lever WF. Inverted follicular keratosis is an irritated seborrheic keratosis. Am J Dermatopathol. 1983;5:474. 7. Boynton JR, Searl SS, Caldwell EH. Large periocular keratoacanthoma: the case for definitive treatment. Ophthalmic Surg. 1986;17:565–9. 8. Grossniklaus HE, Wojno TH, Yanoff M, Font RL. Invasive keratoacanthoma of the eyelid and ocular adnexa. Ophthalmology. 1996;103:937–41. 9. Farina AT, Leider M, Newall J, Carella R. Radiotherapy for aggressive and destructive keratoacanthomas. J Dermatol Surg Oncol. 1977;3:177–80. 10. Eubanks SW, Gentry RH, Patteson JW, et al. Treatment of multiple keratoacanthomas with intralesional fluorouracil. J Am Acad Dermatol. 1982;7:126–9. 11. Scott KR, Kronish JW. Premalignant lesions and squamous cell carcinoma. In: Albert DM, Jakobiec FA, eds. Principles and practice of ophthalmology: clinical practice, vol 3. Philadelphia: WB Saunders; 1994:1733–44. 12. Folberg R. Eyelids: study of specific conditions. In: Folberg, R. Pathology of the eye [CD-ROM]. St Louis: Mosby–Year Book; 1996. 13. Campbell RJ. Tumors of the eyelids, conjunctiva, and cornea. In: Garner A, Klintworth GK, eds. Pathobiology of ocular disease: a dynamic approach, part B, ed 2. New York: Marcel Dekker; 1994:1367–403. 14. Weiss RA. Orbital disease. In: McCord CD, Tanenbaum M, Nunery WR, eds. Oculoplastic surgery, ed 3. New York: Raven Press; 1995:417–76. 15. Ectodermal tumors of the eyelid. In: Griffith DG, Salasche SJ, Clemons DE, eds. Cutaneous abnormalities of the eyelid and face: an atlas with histopathology. New York: McGraw–Hill; 1987:195–254. 16. Rodgers IR, Jakobiec FA, Hidayat AA. Eyelid tumors of apocrine, eccrine, and pilar origins. In: Albert DM, Jakobiec FA, eds. Principles and practice of ophthalmology: clinical practice, vol. 3. Philadelphia: WB Saunders; 1994:1770–96. 17. Font RL. Eyelids and lacrimal drainage system. In: Spencer WH, ed. Ophthalmic pathology: an atlas and textbook, vol. 4, ed 4. Philadelphia: WB Saunders; 1996:2218–437. 18. Jakobiec FA, Zimmerman LE, La Piana F, et al. Unusual eyelid tumors with sebaceous differentiation in the Muir–Torre syndrome. Ophthalmology. 1988;95:1543–8. 19. Ni C, Dryja TP, Albert DM. Sweat gland tumors in the eyelids: a clinicopathological analysis of 55 cases. Int Ophthalmol Clin. 1982;22:1–22. 20. Nerad JA, Anderson RL. CO2 laser treatment of eyelid syringomas. Ophthalmic Plast Reconstr Surg. 1988;4:91–4. 21. Jordan DR, Nerad JA, Patrinely JR. Chondroid syringoma of the eyelid. Can J Ophthalmol. 1989;24:24–7. 22. Ferry AP, Hadad HM. Eccrine acrospiroma (porosynringoma) of the eyelid. Arch Ophthalmol. 1970;83:591–3. 23. Grossniklaus HE, Knight SH. Eccrine acrospiroma (clear cell hidradenoma) of the eyelid: immunohistochemical and ultrastructural features. Ophthalmology. 1991;98:347–52. 24. Font RL, Stone MS, Schanzer MC, Lewis RA. Apocrine hidrocystomas of the lids, hypodontia, palmar–plantar hyperkeratosis, and onychodystrophy: a new variant of ectodermal dysplasia. Arch Ophthalmol. 1986;104:1811–3. 25. Simpson W, Garner A, Collin JRO. Benign hair-follicle derived tumours in the differential diagnosis of basal-cell carcinoma of the eyelids: a clinicopathological comparison. Br J Ophthalmol. 1989;73:347–53. 26. Sternberg I, Buckman G, Levine MR, Sterin W. Trichoepithelioma. Ophthalmology. 1986;93:531–3. 27. Hidayat AA, Font RL. Trichilemmoma of eyelid and eyebrow: a clinicopathologic study of 31 cases. Arch Ophthalmol. 1980;98:844–7. 28. Bardenstein DS, McLean IW, Nerney J, Boatwright RS. Cowden’s disease. Ophthalmology. 1988;95:1038–41. 29. O’Grady RB, Spoerl G. Pilomatrixoma (benign calcifying epithelioma of Malherbe). Ophthalmology. 1981;88:1196–7. 30. Orlando RG, Rogers GL, Bremer DL. Pilomatrixoma in a pediatric hospital. Arch Ophthalmol. 1983;101:1209–10. 31. Haik BG, Karcioglu ZA, Gordon RA, Pechous BP. Capillary hemangioma (infantile periocular hemangioma). Surv Ophthalmol. 1994;38:399–426. 32. Kushner B. Intralesional corticosteroid injection for infantile adnexal hemangioma. Am J Ophthalmol. 1982;93:496–506. 33. Glatt HJ, Putterman AM, Van Aalst JJ, et al. Adrenal suppression and growth retardation after injection of periocular capillary hemangioma with corticosteroids. Ophthalmic Surg. 1991;22:95–7. 34. Walker RS, Custer PL, Nerad JA. Surgical excision of periorbital capillary hemangiomas. Ophthalmology. 1994;101:1333–40. 35. McCannel CA, Hoenig J, Umlas J, et al. Orbital lesions in the blue rubber bleb nevus syndrome. Ophthalmology. 1996;103:933–6. 36. Pang P, Jakobiec FA, Iwamoto T, Hornblass A. Small lymphangiomas of the eyelids. Ophthalmology. 1984;91:1278–84. 37. Tan OT, Gilchrest BA. Laser therapy for selected cutaneous vascular lesions in the pediatric population: a review. Pediatrics. 1988;82:652–62. 38. Woog JJ, Albert DM, Solt LC, et al. Neurofibromatosis of the eyelid and orbit. Int Ophthalmol Clin. 1982;22:157–87. 39. Bergman R. The pathogenesis and clinical significance of xanthelasma palpebrarum. J Am Acad Dermatol. 1994;30:236–42. 40. Vinger P, Sach B. Ocular manifestations of hyperlipidemia. Am J Ophthalmol. 1970;70:563–73. 41. Ribera M, Pintó X, Argimon JM, et al. Lipid metabolism and apolipoprotein E phenotypes in patients with xanthelasma. Am J Med. 1995;99:485–90. 42. John T, Yanoff M, Scheie HG. Eyelid fibrous histiocytoma. Ophthalmology. 1981;88:1193–5. 43. Jordan DR, Anderson RL. Fibrous histiocytoma. An uncommon eyelid lesion. Arch Ophthalmol. 1989;107:1530–1. 44. Zimmerman LE. Ocular lesions of JXG (nevoxanthoendothelioma). Trans Am Acad Ophthalmol Otolaryngol. 1965;69:412–39. 45. Casteels I, Olver J, Malone M, Taylor D. Early treatment of juvenile xanthogranuloma of the iris with subconjunctival steroids. Br J Ophthalmol. 1993;77:57–60. 46. Traboulsi EI, Maumenee IH. Periocular pigmentation in the Peutz–Jeghers syndrome. Am J Ophthalmol. 1986;102:126–7. 47. Sigg C, Pelloni F. Frequency of acquired melanonevocytic nevi and their relationship to skin complexion in 939 schoolchildren. Dermatologica. 1989;179:123–8. 48. Margo CE, Habal MB. Large congenital melanocytic nevus: light and electron microscopic findings. Ophthalmology. 1987;94:960–5. 49. Solomon LM. The management of congenital melanocytic nevi. Arch Dermatol. 1980;116:1017. 50. Dutton JJ, Anderson RL, Schelper RL, et al. Orbital malignant melanoma and oculodermal melanocytosis: report of two cases and review of the literature. Ophthalmology. 1984;91:497–507. 51. Goldberg RA, Shorr N. ‘Vertical slat’ chalazion excision. Ophthalmic Surg. 1992;23:120–2. 52. Bardenstein DS, Elmets C. Hyperfocal cryotherapy of multiple molluscum contagiosum lesions in patients with the acquired immune deficiency syndrome. Ophthalmology. 1995;102:1031–4. 53. Scharf BH. Viral eyelid infections. In: Krachmer JH, Mannis MJ, Holland EJ, eds. Cornea. Vol II: cornea and external disease: clinical diagnosis and management. St Louis: Mosby–Year Book; 1997:641–51.

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4 comments on “Chapter 92 – Benign Eyelid Lesions

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