Marcus Gunn Jaw Winking Syndrome

INTRODUCTION The Marcus Gunn Jaw Winking Syndrome is a form of congenital synkinetic ptosis that is typically unilateral and non-hereditary, although bilateral and familial cases have been reported. The cause remains unknown, but appears to result from a misdirection of either the efferent motor innervation or the afferent proprioceptive fibers of the third and fifth cranial nerves. This results in inappropriate contraction of muscle fibers of the eye or eyelid during mastication. Marcus Gunn...

Oculodermal Melanocytosis

INTRODUCTION Also known as nevus of Ota or nevus fuscocaeruleus ophthalmomaxillaris, this lesion arises from dermal melanocytes. It typically affects tissues along the distribution of the trigeminal nerve and can affect superficial and deep tissues. This lesion is usually congenital, but later onset in puberty or during pregnancy has been reported. Women, especially Asian or black, are most commonly affected. Malignant degeneration may occur, particularly in whites, with the intraocular...

Kaposis Sarcoma

INTRODUCTION Kaposi's sarcoma is a vascular tumor that has been reported to affect up to 25 of patients with AIDS. Up to 20 of AIDS related Kaposi's sarcoma involves the conjunctiva or eyelid. In addition to HIV, Kaposi's sarcoma has been observed in three other clinical settings the classic or European type more prevalent among elderly men of Mediterranean or Eastern European Jewish origin the lymphadenopathic or visceral form, more prevalent in individuals of African origin and the form...

Neurofibroma

INTRODUCTION Neurofibromas are neural tumors composed of a proliferation of axons, Schwann cells, and endoneural fibroblasts. They are most commonly considered in the context of neurofibromatosis where patients develop multiple skin lesions in association with other stigmata of the disease, usually apparent by adolescence. Here they are typically multiple, often occurring in large numbers. The neurofibromas may present on any cutaneous surface, and are common on the face. They typically slowly...

Pemphigus Vulgaris

INTRODUCTION Pemphigus vulgaris is an acquired autoimmune disease in which IgG antibodies are targeted against desmosomal proteins. This results in intraepithelial mucocutaneous blistering. It occurs at all ages but with a peak frequency in the third to sixth decades. Lesions almost always begin on mucous membranes and then spread to involve the skin. Any part of the body can be involved with the eye and eyelids being rather rare. CLINICAL PRESENTATION Lesions present with mucocutaneous...

Hemangiopericytoma

INTRODUCTION This vascular neoplasm arises from the primitive pericytes, which are cells that normally reside in the outer capillary wall. They may occur at any age, and are rare in the eyelids and orbit. Hemangiopericytomas have a great propensity for local spread, especially when recurrent. Although more common in the orbit, localized eyelid hemangiopericytomas have been reported. About 20 of these lesions are malignant, and these may progress rapidly with a poor prognosis. CLINICAL...

Steatoblepharon

INTRODUCTION In steatoblepharon the orbital septum becomes weakened and redundant. This allows the extraconal orbital fat pockets to herniate forward into the eyelids. In younger individuals it may be seen as a familial condition, not associated with other signs of aging. However, in most cases steatoblepharon is seen as an involutional phenomenon associated with dermatocholasis, eyelid laxity, and ptosis. CLINICAL PRESENTATION The eyelids appear full as the prolapsed orbital fat protrudes...

Necrotizing Fasciitis

INTRODUCTION Necrotizing fasciitis is an uncommon and severe invasive soft tissue infection characterized by cutaneous gangrene, suppurative fasciitis, and vascular thrombosis. The disease is usually preceded by penetrating trauma in patients that have systemic problems, most commonly diabetes, alcoholism, and immunosupression, but may occur after blepharoplasty or other eyelid surgery. Necrotizing fasciitis represents a synergistic polymicrobial soft tissue infection with the release of...

References

Primary acquired melanosis of the conjunctiva. Int Ophthalmol Clin 1997 37 61-72. Folberg R, McLean IW, Zimmerman LE. Primary acquired melanosis of the conjunctiva. Hum Pathol 1985 16 129-135. Folberg R, McLean IW. Primary acquired melanosis of the conjunctiva terminology, classification, and biologic behavior. Hum Pathol 1986 17 652-654. Gloor P, Alexandrakis G. Clinical characteristics of primary acquired melanosis. Invest Ophthalmol 1995 36 1721-1729....

Plasmacytoma

INTRODUCTION Malignant plasma cell tumors are divided according to site of origin. They may be multicentric such as multiple myeloma, or localized originating within bone or soft tissue. The latter form is a malignant tumor that is thought to be a separate neoplasm from multiple myeloma with a much better prognosis. Extramedullary plasmacytomas represent 2 to 10 of plasma cell tumors and males are affected three times more often than females. The age at presentation usually lies between the...

Myxoma

INTRODUCTION Cutaneous myxomas are benign adnexal mesenchymal tumors that may occur in isolation or be associated with Carney syndrome. The latter is a familial disorder characterized by multiple neoplasias including a variety of nonendocrine and endocrine tumors, blue nevi, schwannomas and other neoplasms. CNS aneurysms may be part of the syndrome in some cases. Skin myxomas are seen in 33 of cases, and these often involve the eyelids. Skin lesions are benign with no metastatic potential. The...

Phakomatous Choristoma

INTRODUCTION Phakomatous choristoma, also known as Zimmerman's tumor, is a benign congenital adnexal hamartoma of lens tissue. It is seen at birth or shortly thereafter and three-fourths of patients are male. It likely develops from an abnormal migration of cells from the lenticular anlage into the mesodermal structures of the eyelid. Inferior cells of this presumptive lens tissue may become displaced as choristomatous elements into the embryonic mesenchyme destined to become the lower eyelid...

Euryblepharon

Euryblepharon is a very rare congenital anomaly of the palpebral fissure. It is distinguished from ankyloblepharon where the lid margins are fused together to varying degrees, and from cryptoph-thalmos where the eyelids are not formed but skin extends across and over a deformed eye. It may be unilateral or bilateral and is not typically associated with other congenital anomalies or syndromes, nor does there appear to be any familial tendency. It has been suggested that euryblepharon results...

Molluscum Contagiosum

Verrucae Vulgaris Inclusion Bodies

INTRODUCTION Molluscum contagiosum is a common viral skin disease caused by a large intracytoplasmic DNA poxvirus that causes the epidermal cells to undergo decreased transit time. Infection usually arises from direct contact or fomites in children, and by sexually transmitted routes in young adults. The incubation period is usually about two weeks. Specific antibodies are present in 87 of affected patients by immunofluorescence. CLINICAL PRESENTATION The characteristic molluscum lesion appears...

Capillary Hemangioma

Capillary Hemangioma Upper Lid Cases

INTRODUCTION Also known as a benign hemangioendothelioma or strawberry nevus, this common vascular lesion occurs in 1 to 2 of infants and is the most common orbital tumor found in children. It is felt to represent a vascular hamartoma derived from endothelial rests. Periorbital heman-giomas may present as a superficial cutaneous lesion (strawberry hemangioma), subcutaneous lesion, deep orbital tumor, or can occur in a combination of these different locations. Approximately one-third of lesions...

Keloid

INTRODUCTION Keloids represent exuberant scar formation resulting from proliferation of dermal tissue following skin injury. Mechanisms for keloid formation represent abnormal wound healing and include alterations in growth factors, collagen turnover, tension alignment, and genetic and immunologic contributions. Keloids differ from hypertrophic scars in that they spread beyond the initial site of injury. Because they tend to be invasive into the surrounding normal skin both clinically and...

Epidermoid Cyst

INTRODUCTION The epidermoid cyst is also referred to as infundibular cyst, epidermal inclusion cyst, keratinous cyst, or frequently and erroneously sebaceous cyst. The sebaceous cyst is similar clinically but arises from obstruction in the hair follicle and is referred to as a pilar or trichilemmal cyst. The epidermoid cyst is a very common skin lesion that arises from traumatic entrapment of surface epithelium or from aberrant healing of the infundibular epithelium of the hair follicle...

Distichiasis

Distichiasis is a congenital or acquired condition in which there is an accessory row of eyelash cilia behind the normal row. The disorder may be familial with an autosomal dominant pattern of inheritance, but may also follow severe inflammatory or traumatic injury. It is believed that these abnormal lashes develop as a result of metadifferentiation of primary epithelial germ cells originally intent upon meibomian gland development. The meibomian glands are modified sebaceous glands that are...

Merkel Cell Tumor

INTRODUCTION This tumor is a rare aggressive neuroendocrine neoplasm composed of Merkel cells, nondendritic, and nonkeratinocytic epithelial clear cells of neural crest origin. Merkel cells appear to stimulate dermal nerve plexuses and to be involved in the release of various bioactive substances to the dermis. Merkel cell carcinoma accounts for less than 1 of cutaneous malignancies, and an etiologic role for chronic sun exposure has been proposed. It occurs most commonly in elderly Caucasian...

Cavernous Hemangioma

INTRODUCTION Cavernous hemangioma represents a hamartoma that seldom appears prior to middle childhood, with the majority arising after the second decade. Although this lesion is the most common benign orbital tumor in adults, it only occasionally occurs as an isolated eyelid lesion. A rare syndrome termed the blue rubber bleb nevus syndrome, exists which is characterized by multiple cutaneous cavernous hemangiomas associated with gastrointestinal hemangiomas that often bleed. A subtype of...

Floppy Eyelid Syndrome

Floppy Eyelid Syndrome

INTRODUCTION The Floppy Eyelid Syndrome primarily affects obese individuals with a male predominance. The cause of the disease remains unknown and histological examination of the softened and redundant tarsal plate has not suggested any conclusive etiology. A mild chronic inflammatory infiltrate has been reported in some cases, but it is not clear if this was a primary cause or a secondary effect. The tarsal plate and skin show a decreased amount of elastin fibers. The syndrome and its clinical...

Blue Nevus

Pigmented Lesions The Eyelids

INTRODUCTION The blue nevus was first described by Tieche in 1906. It gets its name from its blue color that results from the concentration of melanin in its location in the deep dermis and the Tyndall effect of differential absorption of long wavelengths of light. It is believed to represent dermal arrest in embryonal migration of neural crest melanocytes that fail to reach the epidermis. The blue nevus is composed of pigmented dermal melanocytes and is represented by two histolog-ic types the...

Arteriovenous Hemangioma Malformation

INTRODUCTION The nomenclature of vascular lesions of the skin remains very unclear and there are no clear-cut guidelines for clinicians. Despite attempts at better classifications hemangiomas and malformations are still often confused. Most authorities classify vascular lesions as hemangiomas (hamartomas) or malformations (developmental anomalies). This is further refined based on endothelial characteristics and flow type. When arteriovenous lesions occur in the skin they are often referred to...

Actinic Keratosis

Seborrheic Keratosis Eyelid Treatment

INTRODUCTION Also known as solar or senile keratoses, these neoplasms are a common form of premalignant skin lesion seen on the face. Actinic keratoses are related to ultraviolet radiation damage of epidermal cells on sun-exposed areas of the face, hands, scalp, and eyelids. They occur more commonly in fair-skinned middle-aged or older individuals. The risk of malignant transformation is low, about 0.25 per year, but the ultimate development of squamous cell carcinoma in untreated lesions is as...

Lentigo Senilis

INTRODUCTION Also known as senile lentigines, solar lentigo, age spots, or liver spots, these are the most common lesions found on sun-exposed areas of light-skinned older individuals. They may also occur in younger individuals after prolonged sun exposure, and are common in patients with xeroderma pigmentosum. They are related to aging phenomena in light-skinned individuals where areas of hypopigmentation may be seen along side of focal hyperpigmentation (lentigo senilis). The...

Apocrine Hidrocystoma

INTRODUCTION Also known as a cystadenoma, sudoriferous cyst, or cyst of the gland of Moll, these lesions arise from apocrine glands of Moll and are true cystic adenomas of the secretory cells rather than retention cysts. These lesions are also associated with Schopf-Schulotz-Passarge syndrome, an ectodermal dysplasia in which patients display multiple periocular apocrine hydrocystomas, hypodontias, hypotrichosis, and palmoplantar hyperkeratosis. CLINICAL PRESENTATION Apocrine hidrocystoma...

Herpes and Varicella Zoster

Impetigo Eyelid

INTRODUCTION Herpes zoster (shingles) and varicella zoster (chickenpox) are both systemic infections with manifestations caused by herpes virus varicellae. The virus is an obligate human parasite requiring person-to-person transmission for its survival. Varicella most commonly occurs in children and is almost always a mild, self-limited disease however, when the disease occurs in adults it is often a much more severe process. Zoster, meaning belt or girdle in Greek, is felt to be a reactivation...

Angioedema and Urticaria

INTRODUCTION Angioedema and urticaria are common transient phenomena that result from mast cell degranulation with the release of mediators that promote vascular permeability, causing proteins and fluids to extravasate into the extracellular space. In urticaria fluid collects within the dermal tissue, whereas in angioedema fluid collects in the deeper subcutaneous space. The causes of mast cell degranulation are varied and include both immunologic and nonimmunologic mechanisms. Systemic...

Blepharoptosis

INTRODUCTION Blepharoptosis, or ptosis, is a drooping of the upper eyelid such that the eyelid margin rests lower with respect to the superior corneal limbus. There are numerous causes for ptosis and these can be classified according to mechanistic etiologies. Aponeurotic ptosis is caused by defects in the levator aponeurosis, either redundancy or frank disinsertion. This can be seen from trauma or surgery, or as an involutional phenomenon which is the most common form of adult acquired ptosis....

Leukemia Cutis

Eyelid Infiltrative Disorders

INTRODUCTION Leukemia is a result of neoplastic proliferation of bone marrow-derived leukocytes, the majority of which are of B-cell origin. The disease may be subdivided into acute or chronic forms. The acute form presents with anemia, thrombocytopenia, hemorrhage, adenopathy, hepatosplenomegaly, and a rapidly fatal course. The chronic indolent form is often incidentally diagnosed following prolonged episodes of fever, weight loss, and infection. The acute leukemias are more likely to show eye...

Tarsal Kink Syndrome

The tarsal kink syndrome is a variant of congenital entropion in which there is a horizontal kink or bend in the tarsal plate of the upper eyelid. The cause is unknown, but may be related to in utero environmental factors. CLINICAL PRESENTATION Typically seen in newborn infants, the eye is red and there may be corneal abrasion. Frank corneal ulceration is not an uncommon sequel, and if not managed early, amblyopia may ensue. The upper eyelid is swollen, with entropion of the lash-bearing...

Lupus Erythematosus

Lupus Ana Levels

INTRODUCTION Lupus erythematosus is a chronic inflammatory autoimmune disease with a spectrum of clinical forms ranging from a benign chronic cutaneous variety (discoid lupus erythematosus) to an often-fatal systemic type with nephritis (systemic lupus erythematosus). Intermediate types, variously known as disseminated discoid lupus erythematosus and subacute cutaneous lupus erythematosus, are characterized by various combinations of widespread cutaneous lesions and mild to severe systemic...

Madarosis

INTRODUCTION Madarosis refers to the loss of eyelashes. It may result from trauma, rubbing the eyelids, or it can follow eyelid surgery with injury to the lash follicles. Madarosis is also associated with systemic diseases such as alopecia areata, but here hair loss is usually seen in other parts of the body as well. Discoid lupus erythematosis involving the eyelids presents with erythema, scarring, and madarosis, but the latter can be the only presenting finding before any other...

Angiosarcoma

INTRODUCTION Angiosarcomas are aggressive malignant tumors of vascular endothelium that may originate anywhere in the body. They are relatively uncommon, with most occurring in the head and neck region of men over the age of 55 years. Only rarely does this tumor involve the eyelid. They show an aggressive course and have a high potential for metastasis. Metastases occur to the preauricular and cervical lymph nodes, lung, or liver and occur in approximately one third of cases. The prognosis is...

Seborrheic Keratosis

Seborrheic Keratosis Diseases

INTRODUCTION Seborrheic keratosis is the most common eyelid tumor and the incidence increases with age. They are more common in light-skinned individuals. Also known as a senile verruca and seborrheic wart, this is a benign epithelial neoplasm that can occur on any part of the body. The reticulated type is usually found on the sun-exposed areas of the face and eyelids, and may develop from solar lentigines. These lesions usually affect middle-aged and older adults. CLINICAL PRESENTATION...

Treatment Of Eye Lid Varix

Varices Palp Brale

INTRODUCTION Varices of the eyelids are usually associated with orbital varices with an extension forward into the lid. A varix is an abnormal dilatation of one or more normal veins. They probably relate to an acquired or congenital weakness of the involved vein, or to an obstruction of the venous circulation, or both. Varices can result from compression by a tumor or an arterial aneurysm over an adjacent vein, an arteriovenous malformation, or any trauma or infection that involves the wall or...

Juvenile Xanthogranuloma

Molluscum Eyelid

INTRODUCTION Juvenile xanthogranuloma (JXG) is a rare systemic childhood disease of non-Langerhans cell histiocytes. It is characterized by cutaneous and, on occasion, intraocular lesions. It may be a gran-ulomatous reaction of histiocytes to an unidentified stimulus. There is special predilection for skin and eye involvement. It affects children below the age of five years with 85 of the cases being under one year of age. Most patients are younger than two years of age at presentation. There...

Mucormycosis

INTRODUCTION Mucormycosis is a rapidly progressive fungal infection that occurs in patients in an immunocompromised state. It is most commonly seen in patients with diabetes complicated by ketoacidosis, leukemia, lymphoma, and severe neutropenia. Occasionally, mucormycosis may be the first manifestation of diabetes. The causative agent of mucormycosis is ubiquitous in nature. The fungus is characterized by the presence of large, pauciseptate hyphae that branch at 90o angles within the involved...

Necrobiotic Xanthogranuloma

INTRODUCTION Necrobiotic xanthogranuloma is associated with paraproteimia and with histocytic proliferative disorders. It may be associated with the presence or subsequent development of multiple myeloma or leukemia. Laboratory findings show dysproteinemia due to an IgG paraprotein, leukopenia, greatly elevated ESR, hyperlipidemia, cryoglobulinemia, and depressed complement levels. Most patients have ophthalmic manifestations, mainly affecting the eyelid skin. CLINICAL PRESENTATION These...

Atopic Dermatitis

INTRODUCTION The eyelids can be affected by various types of dermatitis that can be difficult to diagnose. Of these types 70 result from allergic contact dermatitis, and about 9 to 10 each from irritant contact dermatitis, atopic dermatitis, and seborrheic dermatitis. Atopic dermatitis is a chronically relapsing inflammatory skin disease. It is a genetically fixed disease that remains with the patient all their lives, whether they show symptoms or not. It occurs in approximately 2 of the...

Pilomatrixoma

Pilomatricoma

INTRODUCTION Also known as a calcifying epithelioma of Malherbe, pilomatrixoma is a benign tumor of the hair cortical cells. The lesion tends to occur in children and young adults, with 75 less than 10 years of age. The head and upper extremities are the most common sites of involvement with a significant proportion occurring in the periorbital region, particularly the upper eyelid and brow. Most lesions are misdiagnosed as epidermoid and dermoid cysts, and are unsuspected until histopathologic...

Squamous Cell Carcinoma

Mild Basal Cell Carcinoma

INTRODUCTION Squamous cell carcinoma is a malignant tumor that most commonly affects elderly, fair-skinned individuals. It arises from keratinocytes of the epidermis. Unlike the more common basal cell carcinoma, squamous cell carcinoma tends to arise in precancerous areas of skin alteration or in areas of skin damaged by chronic sun exposure, ionizing radiation, carcinogens (e.g., arsenic), psoralen plus ultraviolet A (PUVA) therapy for psoriasis, and the human papilloma virus. Intrinsic...

Basal Cell Carcinoma

Radiation Lesions Eyelids

INTRODUCTION Basal cell carcinoma is a malignant tumor derived from cells of the basal layer of the epidermis. It represents the most common malignant tumor of the eyelids, comprising 85-90 of all malignant epithelial eyelid tumors. The etiology of basal cell carcinomas is linked to excessive ultraviolet light exposure in fair-skinned individuals. Several types of basal cell carcinoma can occur on the eyelids. The nodular-type is the most common, followed by the morphea variety. Over 99 of...

Ichthyosis

INTRODUCTION Ichthyosis represents a heterogeneous group of disorders of skin keratinization. Four major classes of ichthyosis are recognized. The most common type is ichthyosis vulgaris, an autosomal dominant disease with onset prior to age five years. Fine, light scales with flexural sparing is present. The eyelids and eyelashes are often involved. In X-linked ichthyosis affected males manifest large, dark scales with flexural involvement during the first year of life due to a deficiency of...

Apocrine Adenoma

INTRODUCTION Apocrine adenomas, also known as apocrine cystadenoma, are rare adnexal tumors that arise from apocrine Moll glands and ducts. The cystadenoma is derived from secretory epithelium, whereas the specific subtype tubular apocrine adenoma consists mainly of tubules with apocrine epithelium. More than 90 of such lesions occur on the face and scalp. Cystic spaces develop with lipid-rich decapitation material as found in apocrine cysts. Rarely, these lesions can undergo malignant change....

Trichilemmal Sebaceous Cyst

INTRODUCTION The trichilemmal cyst is also referred to as a sebaceous or pilar cyst. It is derived from the outer root sheath of the deeper parts of a hair follicle and consists of a well-keratinized epidermal wall surrounding semi-solid hair keratin and cholesterol-rich debris, rather than just sebaceous material. Trichilemmal cysts are more common in females and most cases occur in the sixth and seventh decades of life. They differ from epidermoid cysts in that they lack a granular layer in...

Cellular Blue Nevus

Dermal Melanocytosis Pathology

INTRODUCTION Cellular blue nevus is a variant of the common blue nevus, but was first described as a variant of melanoma. Although these can be similar clinically to the common blue nevus, they tend to be larger, elevated, and have more pronounced celluarity composed of nonpigmented spindle-shaped melanocytes. They are most common in Asian populations, and rare in blacks. The cellular blue nevus is believed to represent a dermal arrest of embryonal migration of neural crest melanocytes that...

Lentigo Maligna

INTRODUCTION Also known as Hutchinson's melanotic freckle or precancerous melanosis, lentigo maligna is a pigmented patch most often found on the sun-exposed forehead or malar areas and may involve the lower eyelid and canthal areas. It represents 4 to 5 of all cutaneous melanomas. Lentigo maligna arises from the benign lentigo senilis solar lentigo , and represents a premalignant in situ stage of what later can become invasive lentigo cutaneous malignant melanoma. After a variable period of...

Mucoepidermoid Carcinoma

INTRODUCTION Mucoepidermoid carcinoma, also known as adenosquamous carcinoma, is a tumor of low- and high-grade malignancy. Low-grade tumors can appear at any age and grow slowly. The highgrade lesions are more infiltrative and metastasize aggressively to the regional lymph nodes and to distant sites. They typically arise from the major and minor salivary gland epithelium and ductal elements, where they account for 10 to 30 of all primary carcinomas. However, they can also arise within the...

Cryptophthalmos

INTRODUCTION Cryptophthalmos syndrome is a rare congenital anomaly that manifests with varying degrees of completeness. It can be unilateral or bilateral. The condition occurs as an isolated anomaly in 20 of cases, whereas about 40 are familial. Cryptophthalmos can be seen with multiple congenital systemic malformations such as in Fraser syndrome where it is seen in 80 of affected individuals. Here it is associated with renal agenesis, laryngeal atresia, pulmonary hypoplasia, syn-dactyli, and...

Dermatochalasis

Dermatochalasis Upper Eyelid

INTRODUCTION Dermatochalasis refers to a laxity of eyelid skin and loss of muscle tone. It is a common condition that primarily affects persons over the age of 50 years, although it may occasionally be seen in younger individuals. It can affect both upper and lower eyelids and is frequently associated with fat prolapse, or steatoblepharon due to laxity of the orbital septum. As the condition advances the fascial adhesions between the anterior and posterior eyelid lamellae stretch, exacerbating...

Ankyloblepharon

Ankyblephron

INTRODUCTION Ankyloblepharon is a condition where the eyelid margins are fused together to varying degrees. In congenital ankyloblepharon the fused eyelids fail to completely separate during embryogenesis. It can occur as a sporadic isolated finding or in association with diverse chromosomal and syndromic conditions characterized by failure of separation of apposed tissues suggesting a common defect in the mechanisms that regulate tissue fusion. It has also been associated with trisomy 18. The...

Cicatricial Pemphigoid

INTRODUCTION Cicatricial pemphigoid, also known as benign mucous membrane pemphigoid, essential conjunctival shrinkage, or ocular pemphigus is a progressive inflammatory disease of presumed autoimmune etiology. It variously involves mucous membranes of the mouth, conjunctiva, pharynx, nose, esophagus, vagina, urethra, and anus. Oral bullae and erosions occur in 90 of cases. Strictures of the esophagus, urethra, or anus sometimes occur late in the disease. Skin involvement is seen in less than...

Trichoepithelioma

Trichoepithelioma

INTRODUCTION Trichoepithelioma is a benign adnexal tumor of hair follicle origin that may occur as a solitary lesion or as an inherited form with multiple lesions, each with a predilection for the face. They can occur at any age, but the mean age is 45 years. Solitary lesions tend to present in older patients. These lesions are nonaggressive and assymptomatic, but may cause significant cosmetic disfigurement. Multiple lesions may occur in an inherited, autosomal dominant pattern called...

Keratoacanthoma

INTRODUCTION Keratoacanthoma is a relatively common squamoproliferative neoplasm that occurs on sun-exposed areas of adults, the incidence increasing with advancing years. Males outnumber females by a ratio of 2 1. It resembles squamous cell carcinoma both clinically and pathologically, and in 15 to 17 of cases squamous cell carcinoma is misdiagnosed as keratoacanthoma. Some authors have argued that keratoacanthoma should be classified as a variant of well-differentiated squamous cell...

Granuloma Annulare

Deep Granuloma Annulare

INTRODUCTION Granuloma annulare, also known as pseudorheumatoid nodule, is a benign self-limited lesion of uncertain etiology with characteristic clinical and histopathologic appearance. It has been suggested that lesions are triggered by trauma, insect bites, sun exposure, viral infection, and tuberculin skin tests, but there is no convincing evidence to support these claims. Lesions most often occur on the dorsum of the hands, legs, and trunk, and less frequently on the face and eyelids....

Nodular Fasciitis

INTRODUCTION Nodular fasciitis is a benign reactive proliferation of fibroblasts in the subcutaneous tissues. It is also referred to as subcutaneous pseudosarcomatous fibromatosis or proliferative fasciitis. It is seen most commonly in young individuals between 30 and 40 years of age, with about 10 occurring in children. The cause is unknown, but in some cases there may be a history of trauma. It is believed that they may be triggered by a local injury or inflammatory process. Because of the...

Trichiasis

INTRODUCTION Trichiasis is an acquired condition in which the eyelash cilia are turned backward toward the globe. The lid margin is usually oriented normally with respect to the eye, but the lashes are directed at various angles. Trichiasis usually results from inflammation or scarring of the eyelid following eyelid surgery, trauma, chalazion, or severe blepharitis. It is frequently associated with chronic cicatricial diseases such as ocular pemphigoid, trachoma, and Stevens-Johnson syndrome....

Hughes Tarsoconjunctival Flap Procedure

Eyelid Margin Anatomy

The Hughes procedure is a two-staged operation for reconstruction of total or near total lower eyelid defects. As with the free tarsoconjunctival graft, a block of tarsus and conjunctiva is marked out on the ipsilateral upper lid. However, the upper border is left attached superiorly and a conjunctival flap is dissected off of the underlying Muller's muscle to the superior fornix Fig. 10 . The tarsal flap is advanced down into the lower lid defect and sutured to residual tarsus or canthal...

Histopathologic Terminology

Cytoid Bodies

The use of descriptive terms in histopathology is a valuable method for standard communication which allows both the pathologist and the clinician to understand specific histologic characteristics of biological materials. One or more of these characteristics may be specific for certain lesions, thus allowing a more precise diagnosis. In some cases, knowledge of such characteristics can also help the clinician make a provisional diagnosis that might allow therapeutic decisions such as to biopsy...

Xanthogranuloma

Lipoma Eyelid

INTRODUCTION Xanthogranulomas are lesions of lipid accumulation with foamy histiocytes. Different forms of xanthogranuloma are based on specific histologic findings and on clinical associations such as ocular lesions juvenile xanthogranuloma , paraproteinemia and necrobiosis necrobiotic xanthogranuloma , adult lesions with or without new onset asthma adult onset xanthogranu-loma , and visceral deposits with bone lesions Erdheim-Chester Disease . They occur most commonly on the face, scalp, and...

Sebaceous Adenoma

Sebaceous Cell Carcinoma Upper Eyelid

INTRODUCTION Cutaneous adnexal neoplasms showing sebaceous differentiation are difficult to classify. Because of the intimate relationship of sebaceous glands with other adnexal structures associated with the pilosebaceous unit these lesions often display complex histologic features combining sebaceous, hair follicle, and sweat gland tissues. Sebaceous neoplasms run the gamut from benign to malignant lesions. These include sebaceous gland proliferation sebaceous hyperplasia , congenital...

Evaluation of Eyelid Malpositions

Basal Cell Carcinoma Eyelid

A thorough eyelid examination should be included in the documentation of any eyelid malposition. Accurate diagnosis of eyelid dystopia including some determination of its etiology is essential prior to consideration of surgical or nonsurgical correction. An adequate history can provide useful clues to the cause of the eyelid malposition and may suggest the need for further evaluation. In some cases the history may make immediate surgical intervention unwise. The time of onset of the...

Anatomy of the Eyelids

Orbital Rim Vessels Anatomy

The eyelids serve several valuable functions. Most importantly they provide mechanical protection to the globe. They also provide vital chemical elements to the precorneal tear film, and help distribute these layers evenly over the surface of the eye. During the blink phase the eyelids propel tears to the medial canthus where they enter the puncta of the lacrimal drainage system. The eyelashes along the lid margins sweep air-borne particles from in front of the eye, and the constant voluntary...

Sarcoidosis

Sarcoidosis Disease

INTRODUCTION Sarcoidosis is a noncaseating granulomatous multi-system disease of unknown etiology that most commonly affects young adults. It affects males and females equally, but females are more likely to show ocular involvement. There is a greater prevalence of sarcoidosis in the southeastern United States, and it is believed to occur more commonly among blacks. A bimodal incidence has been reported. A sub-acute presentation in patients less than 30 years of age is more likely to be...

Surgical Management of Eyelid Lesions

Atlas Oculoplastic Surgery

Some eyelid lesions can be identified by their history and clinical appearance, and then treated appropriately. However, many benign lesions can be confused with more aggressive malignant tumors from which they must be differentiated. When doubt exists as to a specific diagnosis, a biopsy should be obtained and submitted for histopathologic evaluation. Based on the findings a more directed therapeutic approach can then be planned. In some cases such as inflammatory lesions medical therapy alone...

Milia On Eyelid

Milia Histopathology

INTRODUCTION Milia are common tiny keratin-filled epidermoid cysts that occur as a result of occlusion of pilosebaceous units. Milia commonly occur in patients of all ages. Primary milia are seen in infants, and are believed to arise in sebaceous glands that are not fully developed. They occur on the face, associated with vellus hair follicles. They are so common as to be considered normal. In adults they may occur spontaneously or secondary to damage to the pilosebaceous unit as from trauma,...

Mycosis Fungoides

Mycosis Fungoides Bilder

INTRODUCTION Mycosis fungoides is a cutaneous T-cell lymphoma with ophthalmic involvement in 30 of cases. The disease typically progresses through three characteristic phases. The first is a pruritic, disseminated, eczematous dermatitis that ultimately progresses to infiltrating and plaque forming lesions and terminates in a tumor phase. Eyelid lesions are usually seen in the later tumor phase. However, the disease may start with skin tumors without a preceding dermatitis. The Sezary syndrome...

Sebaceous Cell Carcinoma

Eyelid Papilloma Treatment

INTRODUCTION Sebaceous cell carcinoma is a highly malignant neoplasm that arises from sebaceous glands, and the vast majority of these occur around the eyelids. It can derive from the meibomian glands, glands of Zeis, and from sebaceous glands associated with the pilosebaceous unit. Sebaceous cell carcinoma is an aggressive tumor with a high recurrence rate, a significant metastatic potential, and a notable mortality rate. Although relatively rare, sebaceous gland carcinoma represents the third...

Lymphangioma

Eyelid Lymphangioma

INTRODUCTION Lymphangiomas are lymphatic malformations that may involve the eyelid, conjunctiva, or orbit. This condition is divided into three different types capillary, or lymphangioma circumscripta, cavernous lymphangioma, and cystic hygroma. Sometimes more than one type will coexist in the same patient. Lesions often present at birth or early in childhood, and only occasionally present in adulthood. The condition may appear as a simple dilatation of lymphatic vessels lymphangiectasia , or a...

Dermolipoma

Eye Lid Disease

INTRODUCTION The dermolipomas are congenital choristomas that typically occur on the superotemporal conjunctiva. They contain more adipose tissue that the solid dermoid, and are more common in patients with Goldenhar syndrome oculoauriculovertebral dysplasia . When they contain variable combinations of ectopic tissues such as cartilage, smooth muscle, and acinar glands, they are referred to as complex choristomas. Dermolipomas account for about 5 of all conjunctival tumors in childhood....

Prolapsed Orbital

Orbital Diseases

INTRODUCTION Prolapse of extraconal orbital fat into the eyelids is a common finding generally related to aging phenomena. The fat pockets bulge forward behind a lax orbital septum. That condition is known as steatoblepharon. Prolapse of intraconal orbital fat beneath the conjunctiva is a different phenomenon that presents as a subconjunctival mass rather than a swelling of the eyelid. It may be related to trauma, prior eyelid surgery, and has been associated with diseases such as cutis laxa...

Blepharitis

Atrophic Vagina Histology

INTRODUCTION Blepharitis is a general term referring to eyelid margin inflammation. The two most prevalent factors appear to be a dysfunction of the sebaceous glands meibomian glands , and colonization by pathogenic staphylococci. Additional common features include a diminished or abnormal tear production, chronic conjunctivitis, and structural changes in the lid margin due to chronic inflammation. Several organisms have at times been implicated in the etiology of blepharitis, including...

Pyogenic Granuloma

Pyogenic Granuloma Eyelid

INTRODUCTION Also known as granuloma pyogenicum, the pyogenic granuloma is a common benign vascular lesion of the skin and mucosa. Its name is a misnomer since it is neither infectious nor granulomatous in nature. These lesions often follow trauma or surgery along the areas of injury, and may also develop in association with inflammatory processes such as chalazia. They may be seen in children and young adults without antecedent events, and are prone to arise on the head, neck, upper trunk, and...

Eccrine Hidrocystoma

Eccrine Hidrocystoma Eyelid

INTRODUCTION Eccrine hidrocystoma represents a common cystic lesion with a lining that resembles that of eccrine sweat glands. Thought to represent ductal retention cysts, they occur commonly on the face with a predilection for the canthal angles. Immunohistochemical studies suggest that these lesions are of eccrine origin. CLINICAL PRESENTATION Such lesions present as solitary or multiple, small translucent 1 to 5 mm fluid filled cysts. The lesions are typically flesh-colored to bluish, tense...

Papilloma

Verrugas Malignant

INTRODUCTION A papilloma is any lesion that is papillomatous in growth pattern that is a smooth, rounded, or pedunculated elevation. The squamous papilloma is a generic term for any papilloma of nonviral origin. Also known as a fibroepithelial polyp, acrochordon, or skin tag, this neoplasm commonly occurs on the eyelid, neck, axilla, and groin. This is a benign tumor of squamous epithelial origin, and this is the most common benign lesion found on the eyelid, representing 15 to 30 of all benign...

Dermatofibroma

INTRODUCTION Dermatofibroma is also known as a fibrous histiocytoma. It is a common benign cutaneous tumor of unknown etiology that is more common in females. Although these tumors occur most commonly on the extremities, they have also been described on the eyelids. These lesions represent multiple variants of tumors derived from fibroblast precursors and are frequently referred to as fibrous histiocytoma, nodular subepidermal fibrosis, and sclerosing hemangioma. CLINICAL PRESENTATION These...

Coloboma

INTRODUCTION Coloboma refers to a condition where part of the eyelid is missing. It may be acquired from trauma or surgery. However, the term usually refers to a congenital developmental condition. Coloboma may be seen as an isolated defect or as part of craniofacial anomalies such as Goldenhar's syndrome and Tessier craniofacial cleft syndromes. They may also be seen with amniotic band syndrome where they result from a mechanical pressure of the band on the fetal eyelid. Alternatively,...

Cutaneous Horn

Cutaneous Horns

INTRODUCTION The term cutaneous horn, also known as cornu cutaneum, is a descriptive designation for a protuberant projection of packed keratin that resembles an animal horn. It is more common in elderly individuals, but can be seen in young adults as well. It is associated with a large variety of benign, premalignant, and malignant lesions at the base, thus masking the true diagnosis. About 60 to 75 of such inciting lesions are benign and 8 to 10 malignant. Malignant diagnoses tend to occur...

Acquired Melanosis

Squamous Cell Carcinoma Confused With

INTRODUCTION Melanocytic lesions of the eyelids run the spectrum from benign nevi and acquired melanosis, to invasive malignant melanoma. Acquired melanosis is very common, with nearly one-third of individuals of European descent having at least one patch of conjunctival melanosis in one eye. It generally appears in middle age. Melanosis consists of abnormally prominent intra-epithelial melanocytes, in contrast to melanocytic nevi where nests of melanocytes occur at the dermal-epidermal...

Nevus Flammeus

Port Wine Stain Eyelid

INTRODUCTION Also known as a port-wine stain, nevus flammeus is not a vascular neoplasm but a vascular capillary malformation composed of mature telangiectatic vessels. It can be seen commonly at birth as a discrete median and symmetrical vascular lesion that disappears within the first year of life. A more striking form of congenital nevus flammeus is asymmetric and persists throughout life. It can be isolated and unilateral, or associated with ocular and leptomeningeal vascular hamartomas as...

Inverted Follicular Keratosis

Seborrheic Keratosis Eyelid

INTRODUCTION Inverted follicular keratosis is a benign skin lesion that is common on the face and less frequently on the eyelids. It occurs in older individuals from the fifth decade on, and is considerably more common in males. It is frequently mistaken for a malignant tumor. These lesions arise from the infundibular epithelium of the hair follicle and therefore are related to epidermoid cysts. Inverted follicular keratosis may be an irritated form of seborrheic keratosis or verruca vulgaris....

Melanocytic Nevus

Eyelid Diseases

INTRODUCTION Also known as nevocellular or nevomelanocytic nevi, these common benign neoplasms or hamartomas are composed of melanocytes. Nevi are nests of melanocytes that may be congenital or acquired. Congenital nevi probably represent malformations or errors in development and migration of these neural crest elements. When nevocytes are sequestered along the palpe-bral fissure of the embryo, this results in the presence of a nevus on both the upper and lower eyelid margin, so-called kissing...

Eccrine Nodular Hidradenoma

Clear Cell Apocrine Hideradenoma

INTRODUCTION Eccrine hidradenoma is also referred to as nodular hidradenoma, eccrine spiradenoma, or clear cell hidradenoma. These lesions are uncommon on the eyelids. They presumably arise from eccrine sweat glands and do not show any apocrine differentiation. These tumors occur primarily in middle-aged females and have a predilection for the head region. Very rarely they may undergo malignant change. CLINICAL PRESENTATION The eccrine hidradenoma presents as a solitary slowly progressive...

Dermoid Cyst

Cysts Skin Appendages

INTRODUCTION Dermoid cysts are congenital choristomas containing components of both the epidermis and skin appendages. They account for 15-20 of all eyelid lesions in childhood. These cysts can occur as superficial, subcutaneous, or deep eyelid and orbital lesions. They presumably result from entrapment of skin along embryonic closure lines. Attachment to underlying bony sutures often is present and most commonly involves the frontozygomatic suture. Lesions may extend posteriorly into the orbit...

Chalazion and Hordeolum

Eyelid Erythema

INTRODUCTION A chalazion and hordeolum are focal inflammatory lesions of the eyelid that results from the obstruction of secretory glands. In a chalazion there is no acute bacterial infection, but rather a chronic inflammatory lesion with circumferential fibrosis. When this involves the meibomian glands they form a deep chalazion, whereas when there is involvement of the more superficial glands of Zeis in the dermis or glands of Moll associated with the pilosebaceous unit a more superficial...

Erysipelas

INTRODUCTION Erysipelas is an acute cellulitis-lymphangiitis usually caused by a group A hemolytic Streptococcus. The organism usually gains access through a break in the skin, or occasionally through a surgical incision, and proceeds along the superficial lymphatics. The disease affects mainly older adults. The most common sites of occurrence are the lower legs and face. If the infection extends into the deep subcutaneous and fascial tissues it may spread remarkably rapidly, and is known as...

Impetigo

INTRODUCTION Impetigo represents a superficial invasion of the skin by pathogenic streptococci, staphylococci, or sometimes a mixture of both. Infections tend to occur in areas of previously compromised or diseased skin, such as skin affected by dermatitis, especially eczema, or in a recently lasered resurfaced skin. Owing to the superficial location there is rarely any systemic reaction of consequence. However, in rare instances the bacterial infection may result in the formation of...

Xanthelasma

Necrobiotic Xanthogranuloma

INTRODUCTION Xanthelasmas are common, plaque-like yellow lipid deposits occurring in middle-aged and older adults, particularly in women. The peak age of occurrence is in the fourth and fifth decades. Eyelid xanthelasmas are the most common cutaneous xanthomas. They occur more commonly near the inner canthus, and involve the upper lid more frequently than the lower lid. Although xanthelasmas often occur in patients with normal serum cholesterol levels, up to 50 of cases are associated with...

Cellulitis

Mucormycosis

INTRODUCTION Preseptal cellulitis is defined as inflammation and infection confined to the eyelids and periorbital structures anterior to the orbital septum. The orbital structures posterior to the septum are not involved, but may be secondarily inflamed. In children, the most common cause of preseptal cellulitis is underlying sinusitis. Preseptal cellulitis in children under age 5 was often associated with bacteremia, septicemia, and meningitis caused by Haemophilus influenzae, however, this...

Verruca Vulgaris

Verruca Vulgaris Treatment

INTRODUCTION Also known as a viral wart, or a viral papilloma, this lesion is a papilloma caused by an epidermal infection with the human papillomavirus, which is spread by direct contact and fomites. Immunocompromised patients are more susceptible to infection. Verruca vulgaris is more common in children and young adults between the ages of 5 and 20 years. They may occur anywhere on the skin, including the eyelids. Two common variants exist Verruca filiformis or filiform warts which include...

Evaluation of Eyelid Lesions

Eyelid Blood Vessels

The eyelids may be affected by benign and malignant lesions. Most of these are common elsewhere on the body, but when occurring on the eyelid they are often different in character, appearance, and behavior because of the unique characteristics of eyelid skin. A large number of cutaneous and systemic disorders may be associated with eyelid lesions. In many instances the eyelid findings are quite specific for a particular disorder, at other times they may be rather non-specific. These ocular...

Malignant Melanoma

Nodular Melanoma Clear

INTRODUCTION Cutaneous malignant melanoma is an invasive proliferation of malignant melanocytes, and accounts for 1 of all eyelid malignancies. The incidence increases with age, but remains relatively stable from the fifth to the seventh decades. Cutaneous malignant melanoma may be classified into four different types lentigo maligna melanoma 5 , superficial spreading melanoma 70 , nodular melanoma 16 , and acral lentiginous melanoma 9 . Nodular melanoma and lentigo maligna melanomas are the...

Abscess

Difference Abscess Cellulitis Skin Layer

INTRODUCTION An abscess is a collection of pus within a cavity formed in soft tissue or bone. It is usually associated with an infection caused by bacteria or parasites that gain access via a break in the skin or sometimes through hematogenous spread. Sometimes a sterile abscess can be induced by retained foreign material. Local tissue cells are destroyed by bacterial action or toxins and this triggers an inflammatory response by attracting large numbers of white blood cells. Regional blood...

Plexiform Neurofibroma

Vagina Diseases

INTRODUCTION Plexiform neurofibromas are the most common benign peripheral nerve tumor occurring in the eyelid and are considered pathognomonic for type 1 neurofibromatosis NF-1 . The lesion arises from and grows along any peripheral nerve. Plexiform neurofibromas typically present in children during the first decade of life. Mechanical ptosis can be profound, and in younger children may cause deprivation amblyopia. Associated systemic and ocular findings in patients with neurofibromas are...

Eyelid Lesions and Tissues of Origin

Excretory Glands

The plethora of lesions that can occur on the eyelids is rather daunting, not to say confusing, to the average clinician. Many names are similar and while they may be meaningful to the pathologist based on details of microscopic findings, they often add little to the clinical recognition or management of these diseases. Placing such lesions into a more or less organized system based on anatomical tissues of origin may be a useful exercise. All lesions that involve the eyelids or any other...

Herpes Simplex

Eosiniphlic Keratynocytes

INTRODUCTION Herpes simplex is caused by a DNA virus that is estimated to infect 60 to 90 of individuals at sometime during their life. Clinically evident infections however are much less common. Involvement of the facial region is predominantly due to type I herpes virus, with the exception of newborns, in whom overwhelming exposure to the type II variety during birth can result in development of typical skin lesions during the first few days of life, often associated with devastating CNS and...