Melanocytic Nevus
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...
Blue Nevus
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...
Epidermal Necrolysis Disease Spectrum
INTRODUCTION Erythema multiforme is an acute mucocutaneous hypersensitivity reaction. Although once believed to be distinct diseases, many observers currently consider erythema multiforme, Stevens-Johnson syndrome, and toxic epidermal necrolysis TENS to represent a mild to severe continuum of the same process. Erythema multiforme minor represents the mildest form. These three entities share certain clinical, histologic, and etiologic characteristics in common. Although ocular involvement in...
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...
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...
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....
Retraction of the Eyelid
INTRODUCTION Eyelid retraction may be seen as a sequel to trauma, surgery, or orbital disease. The most common cause is Graves' disease. This is a systemic autoimmune disease in which the primary target of the immune response is directed toward the TSH receptor of the thyrocyte. Extrathyroidal manifestations principally involve specific fibroblasts of the orbit and of the pretibial dermis. The immune reaction is mediated by anti-TSHR T-cells and results in the release of a Th-1 cytokine...
Capillary Hemangioma
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...
Intravascular Pyogenic Granuloma
INTRODUCTION Pyogenic granuloma, also known as lobular capillary hemangioma, is a benign vascular tumor. The intravascular variant is an angiomatous proliferation that occurs within a blood vessel sometimes associated with an underlying arteriovenous malformation or hemangioma. The lesion is a polypoid mass projecting into the lumen of a dilated vein. CLINICAL PRESENTATION Intravascular pyogenic granuloma occurs within a vein such as the angular vein. It presents as a slowly enlarging deep firm...
Lupus Erythematosus
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...
Amyloidosis
INTRODUCTION Amyloidosis represents a variety of disorders that result in abnormal glycoprotein deposition. It can be associated with certain infections, inflammatory processes, and plasma cell dyscrasias. Primary amyloidosis occurs in the setting of no known predisposing disease. Secondary amyloidosis is typically superimposed on a chronic inflammatory disease such as leprosy, osteomyelitis, or rheumatoid arthritis. Amyloidosis occurring with multiple myeloma is considered a distinct entity....
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...
Leukemia Cutis
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...
Cylindroma
INTRODUCTION Cylindromas are rare tumors of primitive sweat gland origin. They occur in two settings either as nonfamilial solitary lesions or as dominantly inherited multiple tumors. There is a well-established association of multiple cylindromas in association with trichoepitheliomas in some familial cases. Rarely, cylindromas usually of the multiple type undergo malignant degeneration capable of lymph node and visceral metastases. Brooke-Spiegler syndrome is an autosomal dominantly inherited...
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...
Cellulitis
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...
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...
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...
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...
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...
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...
Dermolipoma
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....
Cutaneous Horn
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...
Chalazion and Hordeolum
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...
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...
Eccrine Nodular Hidradenoma
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...
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...
Oromandibular Dystonia
INTRODUCTION Oromandibular dystonia OMD is a focal dystonic movement disorder affecting the mid and lower face, particularly the jaw and tongue. It is characterized by spasms along the sides of the nose, the cheek, mouth, and chin. Uncontrolled spasms make opening and closing of the mouth difficult and can have a profound influence on eating and speaking. Meige's syndrome is a term used for a regional dystonia consisting of the two adjacent focal dystonias, benign essential blepharospasm, and...
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....
Clinical
Microblepharon is a very rare congenital anomaly that should be distinguished from cryptophthalmos where the eyelids are not formed but skin extends across and over a deformed eye or are fused to a disrupted globe. In microblepharon the eyelid margins are formed and the underlying globe is normal. CHARACTERISTICS In microblepharon the eyelid margins are normal but there is a vertical shortening of the lids to varying degrees. The condition may be unilateral or bilateral and can involve both...
Insect Bite
INTRODUCTION Insect bites or stings result in the introduction of venom or toxins into the skin, which in turn cause the release of vasoactive amines. This results in marked swelling and erythema of the thin tissues of the eyelid. Unless secondary infection occurs, these reactions tend to resolve rapidly. Dermatitis nodosa is a specific type of insult due to barbed cilia acquired from caterpillar contact. The barbs result in the cilia working their way into the skin where they set up a...
Eccrine Hidrocystoma
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...
Chondroid Syringoma
INTRODUCTION Chondroid syringoma is also known as pleomorphic adenoma or mixed tumor of the skin. These benign lesions are of possible eccrine gland or hair follicle origin, although several reports showed apocrine differentiation. They usually occur on the head and neck. They only occasionally involve the eyelid. Rarely, malignant variants have been reported. CLINICAL PRESENTATION These lesions present as an asymptomatic solitary nodule 0.5 to 2.0 cm in diameter. They generally show slow...
Epibulbar Osseous Choristoma
INTRODUCTION Epibulbar osseous choristoma is a choristomatous lesion of the conjunctiva containing bone in an otherwise normal eye. It is usually a congenital lesion arising as an abnormal development of embryonic pleuripotential mesenchyme, presenting in childhood. However, some lesions may be associated with trauma, presenting in adulthood, and possibly related to inflammation. 70 of cases are in females and 80 occur in the superotemporal quadrant. Lesions can sometimes be associated with...
Actinic Keratosis
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...
Arteriovenous HemangiomaMalformation
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 as...
Acquired Melanosis
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...
Abscess
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...
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...
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,...
Brow Ptosis
INTRODUCTION Brow ptosis results from sagging of forehead skin and loss of fascial support of the eyebrows to the frontal bone. It is a common deformity in the aging face and frequently accompanies laxity of other periorbital structures, such as eyelid skin and canthal tendons. A downward displacement of the eyebrows can accentuate the degree of redundancy in upper eyelid skin and result in significant loss of superior visual field. The ptosis may be general, involving the entire width of the...
Horners Syndrome
INTRODUCTION Lesions in either the central or peripheral sympathetic pathways to the eye and face lead to the triad of symptoms known as Horner's syndrome. Loss of sympathetic tone to Muller's accessory eyelid muscle results in upper eyelid ptosis, loss to the dilator muscle of the iris causes pupillary miosis, and absence of sympathetic innervation to facial sweat glands produces anhidrosis. In addition, parasympathetic innervation is unopposed resulting in overaction of parasympathetic...
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...
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...
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...
Essential Blepharospasm
INTRODUCTION Essential blepharospasm EB is a bilateral focal dystonia of idiopathic origin. The condition usually affects patients in their fifth to sixth decades but can be seen in younger individuals, even in their teens. It is two to three times more common in females. In 33 of cases some familial occurrence of dystonia can be elicited, suggesting a possible genetic predisposition. Some evidence suggests a central defect in the coordination of ocular and periocular sensory information and...
References Byc
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Dufek MA, Shechtman DL. Floppy eyelid syndrome a diagnostic dilemma. J Am Optom Assoc 1999 70 450-454. Dutton JJ. Surgical management of floppy eyelid syndrome. Am J Ophthalmol 1985 99 557-560. Klapper SR, Jordan DR. Floppy eyelid syndrome. Ophthalmology 1998 105 1582. Langford JD, Lindberg JV. A new physical finding in floppy eyelid syndrome. Ophthalmology 1998 105 165-169. Liu DT, Di Pascuale MA, Sawai J, Goa YY, Tseng SC. Tear film dynamics in floppy eyelid syndrome. Invest Ophthalmol Vis...