Ординатура / Офтальмология / Английские материалы / Diagnostic Atlas of Common Eyelid Diseases_Dutton, Gayre, Proia_2007‎

Cellulitis (Contd.)

age 5 was often associated with bacteremia, septicemia, and meningitis caused by Haemophilus influenzae, however, this cause of preseptal and orbital cellulitis has virtually been eliminated by the introduction of the HIB vaccine. Currently, most cases of preseptal and orbital cellulitis in children are due to gram-positive cocci. In teenagers and adults preseptal cellulitis usually arises from a superficial source such as traumatic inoculation, or a chalazion. The site of the infected focus is often difficult to find because the eyelid tissues become markedly swollen.

CLINICAL PRESENTATION The initial skin lesion is often a small, erythematous focus, suggesting an early furuncle, but instead of localizing, it rapidly spreads through the adjacent subcutaneous tissues after 5 to 10 days. Eyelid edema, erythema, and inflammation may be severe. Unless the infection spreads to the post-septal orbit, the globe is uninvolved; pupillary reaction, visual acuity, and ocular motility are not disturbed; pain on eye movement and chemosis are absent. Complications can result in lagophthalmos, ectropion, and lid necrosis.

HISTOPATHOLOGY Cellulitis, or diffuse inflammation of the connective tissue of the skin or deeper soft tissues, may be acute or chronic. Acute cellulitis is characterized histopathologically by an infiltrate of neutrophils throughout the dermis and/or subcutaneous tissue. There may be subepidermal edema and vascular ectasia. In chronic cellulitis the inflammatory infiltrate consists mostly of lymphocytes and macrophages and is accompanied by fibrosis and angiogenesis.

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Cellulitis (Contd.)

DIFFERENTIAL DIAGNOSIS The differential diagnosis includes orbital cellulitis, erysipelas, Mucormycosis, and ruptured dermoid or epidermoid cyst.

TREATMENT Initial antibiotic selection is based on the history, clinical findings, and initial laboratory studies. With positive culture, prompt sensitivity studies are indicated so that the antibiotic selection can be revised, if necessary. Staphylococcus aureus is the most common pathogen in patients with preseptal cellulitis from trauma. The infection usually responds quickly to penicllinase-resistant penicillin. Imaging studies should be performed to rule out underlying sinusitis if no direct inoculation site is identified. If the patient does not respond quickly to oral antibiotics or if orbital involvement becomes evident, prompt hospital admission, CT scanning and intravenous antibiotics are usually indicated. Surgical drainage may be necessary if the preseptal cellulitis progresses to a localized abscess. Incision and drainage can usually be performed directly over the abscess. The orbital septum should not be opened to avoid contaminating the orbital soft tissue. Affected children should be treated in consultation with a pediatrician, and hospitalization and intravenous antibiotics may be indicated.

REFERENCES

Baker C. Group B streptococcal cellulitis-adenitis in infants. Am J Dis Child 1982; 136:631.

Casady DR, Zobal-Ratner JL, Meyer DR. Eyelid abscess as a presenting sign of occult sinusitis. Ophthal Plast Reconstr Surg 2005; 21:368–370.

Donahue SP, Schwartz G. Preseptal and orbital cellulitis in childhood. A changing microbiologic spectrum. Ophthalmology 1998; 105:1902–1905.

Feingold D. Gangrenous and crepitant cellulitis. J Am Acad Dermatol 1982; 6:289–299.

Harris GJ. Subperiosteal abscess of the orbit: age as a factor in the bacteriology and response to treatment. Ophthalmology 1994; 101:585–595.

Harris GJ. Subperiosteal abscess of the orbit: computed topography and the clinical course. Ophthal Plast Reconstruct Surg. 1996; 12:1–8.

Parunovic A. Proteus mirabilis causing necrotic inflammation of the eyelid. Am J Ophthalmol 1973; 76:543–544.

Rao VA, Hans R, Mehra AK. Pre-septal cellulitis—varied clinical presentations. Indian J Ophthalmol 1996; 44:225–227.

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 chalazion results. A hordeolum is an acute bacterial abscess filled with pus and associated with pain and inflammatory signs. They can involve the meibomian glands (deep hordeolum) or the Zeis and Moll glands ( superficial hordeolum). Superficial hordeola are usually found near the eyelid margin where the Zeis glands are concentrated. Two-thirds of chalazia show mixed-cell cytology, and one-third are suppurating granulomas. The latter tend to occur in older patients with a longer duration of symptoms and larger lesions. When the impacted gland ruptures, extravasated lipid material produces a surrounding chronic lipogranulomatous inflammation. Both chalazia and hordeola often occur in patients with blepharitis and rosacea.

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Chalazion and Hordeolum (Contd.)

CLINICAL PRESENTATION A hordeolum presents acutely with pain, eyelid edema, and erythema, and it evolves into a subcutaneous nodule which may point anteriorly to the skin surface or through the posterior surface of the lid where the tarsus is closer to the conjunctival surface. Exuberant lesions will sometimes erode through the conjunctiva presenting as a type of pyogenic granuloma. They may drain spontaneously or under medical therapy. In contrast, chalazia usually present insidiously as a firm, painless mass. Multiple lesions of both types are not uncommon and very large lesions on the upper lid may even induce astigmatism and amblyopia in children.

HISTOPATHOLOGY A chalazion is a localized lipogranulomatous reaction to the sebaceous glands of the eyelids (either the meibomian glands or the glands of Zeiss). Obstruction of sebaceous gland ducts results in a granulomatous response surrounding vacuoles that remain when lipid is dissolved during histological processing. Epithelioid cells predominate in the granulomas, and the number of multinucleated giant cells is highly variable. Neutrophils may be prominent in early lesions, while lymphocytes and varying degrees of fibrosis are seen in more chronic chalazia. Chalazia arising from the meibomian glands may rupture into the conjunctival substantia propria or involve the dermis of the eyelid. Chalazia developing from a gland of Zeiss usually remain localized to the eyelid margin.

DIFFERENTIAL DIAGNOSIS About 6 % to 7% of chalazia and hordeola are misdiagnosed at presentation, with about 1 to 2% actually being malignant. The differential diagnosis includes chronic inflammation, abscess, sebaceous cell carcinoma, and basal cell carcinoma.

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Chalazion and Hordeolum (Contd.)

TREATMENT Small hordeola may resolve spontaneously. Acute lesions are initially treated with hot compresses to encourage localization and drainage, combined with a topical steroid-antibiot- ic preparation. When further treatment is required the technique varies according to the stage and nature of a lesion. Chronic chalazia may be treated using intralesional corticosteroid injection or surgical drainage. Injection of soluble steroids such as tiramcinolone acetonide, can be effective but carries a very small risk of central retinal artery obstruction, the result of retrograde arterial infusion. It can also induce focal depigmentation in darker skinned patients. Inadvertent ocular penetration has been reported. If a viral etiology is suspected, steroids should be used only cautiously. Suppurating granulomas in long-standing chalazia respond better to surgery than to steroid injections so that larger, long-standing lesions ( 8 months duration) are best treated surgically. When medical therapy or steroids fail to resolve the lesion surgical drainage is performed. This is best accomplished with vertical transconjunctival incisions that allow adequate curettage of the lesion while limiting damage to surrounding meibomian glands. Thermal cautery of the cyst wall has no demonstrable effect on recurrence rate. In recurrent lesions, biopsy is necessary to exclude sebaceous gland carcinoma.

REFERENCES

Ben Simon GJ, Huang L, Nakra T, et al. Intralesional tiramcinolone acetonide injection for primary and recurrent chalazia: is it really effective? Ophthalmology 2005; 112:913–917.

Dhaliwal U, Bhatia A. A rationale for therapeutic decision-making in chalazia. Orbit 2005; 24:227–230.

Donaldson MJ, Gole GA. Amblyopia due to inflamed chalazion in a 13-month old infant. Clin Experiment Ophthalmol 2005; 33:332–333.

Goldberg RA, Shorr N. ‘Vertical slat’ chalazion excision. Ophthalmic Surg 1992; 23:120–122.

Hosal BM, Zilelioglu G. Ocular complications of intralesional corticosteroid injection of a chalazion. Eur J Ophthalmol 2003; 13:798–799.

Lempert SL, Jenkins MS, Brown SI. Chalazia and rosacea. Arch Ophthalmol 1979; 97:1652–1653.

Ozdal PC, Codere F, Callejo S, Caissie AL, Burbier MN. Accuracy of the clinical diagnosis of chalazion. Eye 2004; 18:135–138.

Sendrowski DP, Maher JF. Thermal cautery after chalazion surgery and its effect on recurrence rates. Optom Vis Sci 2000; 77:605–607.

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.

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Chondroid Syringoma (Contd.)

CLINICAL PRESENTATION These lesions present as an asymptomatic solitary nodule 0.5 to 2.0 cm in diameter. They generally show slow growth over 2 to 10 years or longer before coming to medical attention. When they occur on the eyelid they tend to be fixed to the underlying tarsus, but they do not cause changes in the overlying epidermis except occasionally for some pigmentation.

(Courtesy of J. Justin Older, M.D.)

HISTOPATHOLOGY Epithelial cells are distributed singly or form tubules or ducts that are usually lined by two or more rows of epithelial cells. The outer layer of epithelial cells lining the tubules tends to be more flattened than the inner layer of cells. The epithelial cells are within a stroma that varies in appearance from chondroid to myxoid to fibrous. The chondroid stroma is bubbly and blue in H&E stained sections and contains abundant glycosaminoglycans (mucopolysaccharides) that stain positively using alcian blue, colloidal iron, and mucicarmine techniques.

DIFFERENTIAL DIAGNOSIS The differential diagnosis includes epidermoid cyst, neurofibroma, and pilomatrixoma.

TREATMENT Treatment is not indicated except for cases of cosmesis or when the lesion interferes with vision. Surgical excision within its capsule is curative.

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Chondroid Syringoma (Contd.)

REFERENCES

D’Hermies F, Mourier L, Wastl JP, et al. Palpebral form of mixed tumor of the lacrimal gland. Apropos of a case. J Fr Ophtalmol 1992; 15:220–223.

Jordan DR, Nerad JA, Patrinely JR. Chondroid syringoma of the eyelid. Can J Ophthalmol 1989; 24:24–27.

Mandeville JT, Roh JH, Woog JJ, et al. Cutaneous benign mixed tumor (chondroid syringoma) of the eyelid: clinical presentation and management. Ophthal Plast Reconstr Surg 2004; 20:110–116.

Martorina M, Capoferri C, Dessanti P. Chondroid syringoma of the eyelid. Int Ophthalmol 1993; 17:285–288. Mencia-Gutierrez E, Bonales-Daimiel JA, Gutierrez-Diaz E, et al. Chondroid syringomas of the eyelid: two cases. Eur J

Ophthalmol 2001; 11:80–82.

Meythaler H, Koniszewski G. Pleomorphic adenoma of Moll’s glands. Klin Monatsbl Augenheilkd 1979; 175:825–828. Saini JS, Mukherjee AK, Naik P. Pleomorphic adenoma of Krause’s gland in the lower lid. Indian J Ophthalmol 1985;

33:181–182.

Tong JT, Flanagan JC, Eagle RC Jr, Mazzoli RA. Benign mixed tumor arising from an accessory lacrimal gland. Ophthal Plast Reconstr Surg 1995; 11:136–138.

Tyagi N, Abdi U, Tyagi SP, Maheshwari V, Gogi R. Pleomorphic adenoma of skin (chondroid syringoma) involving the eyelid. J Postgrad Med 1996; 42:125–126.

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 25% of cases and takes one of two forms: a recurrent nonscarring vesiculobullous eruption, mainly involving the extremities and inguinal region, or in the form of localized erythematous plaques with associated vesicles and bullae on the face and scalp. The latter variant heals with small atrophic scars. Another variant of localized pemphigoid known as Brunsting-Perry type has skin lesions limited to the face and neck but with no mucosal involvement. Significant scarring of affected areas often occurs, sometimes involving the eyelids. The average age of onset of cicatricial pemphigoid is in the seventh decade, but it can occur at any age, including childhood. The incidence is approximately 1:30,000 and there is a slight female predominance.

CLINICAL PRESENTATION In ocular cicatricial pemphigoid the conjunctival bullae are evanescent and therefore are seldom visualized. The initial symptoms usually include irritation and tearing due to ruptured conjunctival bullae. Subepithelial scarring with gradual obliteration of tear gland openings and loss of mucous glands ultimately results in keratoconjunctivitis sicca. In addition, surfaces on the bulbar and palpebral conunctiva fuse together, resulting in symblepharon. Eventually these symblephara obliterate the conjunctival fornices impairing lid closure. A band of symblepharon extending across the medial part of the lower cu-de-sac is typical of early ocular pemphigoid. The resulting entropion, tear deficiency, and lagophthalmos conspire to cause corneal opacification and loss of vision. Although a few unilateral cases have been seen, cicatricial pemphigoid almost always eventually becomes bilateral. However, involvement of the second eye may be delayed for up to two years.

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Cicatricial Pemphigoid (Contd.)

(Courtesy of Charles S. Soparkar, M.D.)

HISTOPATHOLOGY By routine light microscopy, cicatricial pemphigoid has subepidermal vesicles containing fibrin, edema fluid, and inflammatory cells. The dermis has a perivascular infiltrate of mainly lymphocytes and plasma cells, along with lesser numbers of neutrophils and eosinophils. Scarring may be prominent. Subepithelial bullae are not commonly seen in conjunctival lesions. Light microscopic features are nonspecific; diagnosis rests on demonstrating by direct immunofluorescence the linear deposition of immunoglobulin (usually IgG) and often of complement component C3 along the basement membrane zone.

DIFFERENTIAL DIAGNOSIS The differential diagnosis includes porphyria, bullous pemphigoid, sebaceous gland carcinoma, dermatitis herpetiformis, pemphigus vulgaris, Sjogren’s disease, beta-hemolytic streptococcal infections, diphtheria, adenoviral and herpes simplex infections, trachoma, prolonged use of oral practolol, and reactions to epinephrine, pilocarpine, and/or phospholidine iodide eye drops.

TREATMENT Diagnosis can sometimes be established with a biopsy of an oral mucosal bulla, with histopathologic examination showing a sub-epidermal locus. Immunofluorescent antibodies fixed to conjunctiva basement membrane can be demonstrated in up to 80% of cases. Assays for circulating auto-antibodies also exist, but are positive in only about 10% of affected patients. During exacerbations, topical steroids will reduce the severity and perhaps diminish scarring. Because up to half of patients will harbor staphylococci, the lids and conjunctivae should be periodically cultured, and a course of appropriate antibiotics started if necessary. Artificial tears often help the signs and symptoms of aqueous tear deficiency. In severe cases combined therapy using systemic steroids and immunosuppressive agents is usually of benefit. Dapsone

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Cicatricial Pemphigoid (Contd.)

(diaminodiphenylsulfone) in doses of 25–50 mg daily can show early benefit, but its effect appears to diminish after one to two years. Conjunctival surgery, including cryosurgery, should be avoided because it often precipitates exacerbations, ultimately leaving the patient worse off. Marked loss of conjunctiva can be repaired with mucous membrane or amniotic membrane grafts. Eyelid surgery for trichitic lashes should avoid insults to the conjunctiva if possible, and eyelid margin rotation through an anterior approach is preferred for entropion. No matter the intervention, some patients will inexorably progress despite all therapeutic measures.

REFERENCES

Bartley GB. Surgical reconstruction of the ocular surface in advanced ocular cicatricial pemphigoid and Stevens-Johnson syndrome. Am J Ophthalmol 1996; 122:752–753.

Bean S, Furey N, West C, Andrews T, Esterly NB. Ocular cicatricial pemphigoid (immunologic studies). Trans Sect Ophthalmol Am Acad Ophthal Otoloaryngol 1976; 81:806–812.

Dutton JJ, Tawfik HA, DeBacker CM, Lipham WJ. Anterior tarsal V-wedge resection for cicatricial entropion. Ophthal Plast Reconstr Surg 2000; 16:126–130.

Elder MJ, Collin R. Anterior lamellar repositioning and grey line split for upper lid entropion in ocular cicatricial pemphigoid. Eye 1996; 10:439–442.

Elder MJ, Collin R. Lid surgery: the management of cicatricial entropion and trichasis. Dev Ophthalmol 1997; 28:207–218. Elder MJ, Collin R. The eyelid sequelae of chronic progressive conjunctival cicatrisation. Dev Ophthalmol 1997; 28:176–181. Griffith MR, Fukuyama K, Tuffanelli D, Silverman S Jr. Immunofluorescent studies in mucous membrane pemphigoid. Arch

Dermatol 1974; 109:195–199.

Holsclaw DS. Ocular cicatricial pemphigoid. Int Ophthalmol Clin 1998; 38:89–106.

Mondino BJ, Brown SI, Lempert S, Jenkins MS. The acute manifestations of ocular cicatricial pemphigoid: Diagnosis and treatment. Ophthalmology 1979; 85:543–555.

Mondino BJ, Brown SI. Ocular cicatricial pemphigoid. Ophthalmology 1981; 88:95–100.

Mondino BJ, Brown SI. Immunosuppressive therapy in ocular cicatricial pemphigoid. Am J ophthalmol 1983; 96:453–459. Mondino BJ, Ross AN, Rabin BS, Brown SI. Autoimmune phenomena in ocular cicatricial pemphigoid. Am J Ophthalmol

1977; 83:443–450.

Rogers RS, Perry HO, Bean SF, Jordan RE. Immunopathology of cicatricial pemphigoid: studies of complement deposition. J Invest Dermatol 1977; 58:39–43.

Shore JW, Foster CS, Westfall CT, Rubin PA. Results of buccal mucosal grafting for patients with medically controlled ocular cicatricial pemphigoid. Ophthalmology 1992; 383–385.

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 more commonly in males and in patients 8 to 10 years older than those with benign diagnoses. The most common inciting diagnoses are seborrheic keratosis, actinic keratosis, and squamous cell carcinoma.

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Cutaneous Horn (Contd.)

CLINICAL PRESENTATION Cutaneous horns are usually seen on areas of exposed skin. They may attain a very large size causing mechanical ptosis or other eyelid malpositions, and may completely occlude vision. Lesions present as a dry, white to yellowish, firm, hornlike projection of keratin that extends upward from the skin surface. It may have several distinct projections that coalesced at the base.

HISTOPATHOLOGY Cutaneous horn is a clinical diagnosis that corresponds histologically to a protuberant mass of keratin. To be designated a “horn”, the height should exceed at least one-half of the greatest diameter of the lesion from which it arises. Cutaneous horns are most commonly associated with actinic (solar) keratosis, verruca vulgaris, seborrheic keratosis, squamous cell carcinoma, inverted follicular keratosis, or tricholemmoma.

DIFFERENTIAL DIAGNOSIS The cutaneous horn is easy to diagnose, but its significance is determining the underlying lesion. They can develop from a variety of underlying lesions, including seborrheic keratosis, actinic keratosis, inverted follicular keratosis, verruca vulgaris, tricholemmoma, subepidermal calcified nodules, basal cell carcinoma, squamous cell carcinoma, metastatic tumors, and other epidermal tumors.

TREATMENT Treatment is dependent on the underlying cause. For benign lesions simple excision or shave biopsy may be adequate. However, when the basal lesion is malignant, surgical excision with

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Cutaneous Horn (Contd.)

adequate clear margins will be necessary to affect a cure. In most cases, however, the diagnosis is not apparent until the specimen has been submitted for histologic examination. Because of the possibility of an underlying malignant lesion, complete surgical excision should be performed.

REFERENCES

Brauninger GE, Hood CI, Worthen DM. Sebaceous carcinoma of lid margin masquerading as cutaneous horn. Arch Ophthalmol 1973; 90:380–381.

Copcu E, Sivrioglu N, Culhaci N. Cutaneous horns: are these lesions as innocent as they seem to be? World J Surg Oncol 2004; 2:18.

D’Hermies F, Gerolami-Favreul I, Meyer A, et al. A cutaneous horn of the free margin of the eyelid: an anatomical and clinical observation. J Fr Ophtalmol 2003; 26:534–537.

Ferry AP. Subepidermal calcified nodules of the eyelid. Am J Ophthalmol 1990; 109:85–88.

Mencia-Gutierrez E, Gutierrez-Diaz E, Redondon-Marcos I, Ricoy JR, Garcia-Torre JP. Cutaneous horns of the eyelid: a clinicopathological study of 48 cases. J Cutan Pathol 2004; 31:539–543.

Thappa DM, Laxmisha C. Cutaneous horn of eyelid. Indian Pediatr 2004; 41:195.

Yu RC, Pryce DW, Macfarlane AW, Stewart TW. A histopathologic study of 643 cutaneous horns. Br J Dermatol 1991; 124:449–452.

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 disease characterized by cutaneous adnexal neoplasms, most commonly cylindromas and tirchoepitheliomas. Lesions may show histologic hybrid features of apocrine, follicular, and sebaceous differentiation. The occurrence of combined sebaceous and trichoblastic features suggests that cylindromas are not eccrine tumors but neoplasms of the folliculosebaceousapocrine unit.

CLINICAL PRESENTATION Solitary lesions usually appear as dome-shaped, smooth, firm dermal nodules of varying size on the eyelid or brow. The color ranges from flesh toned to pinkish-blue with white specks. The multiple, inherited variety shows numerous nodules of varying size, mainly on the scalp, but some may be found on the forehead and face. Such extensive involvement is known as a “turban tumor.” A case of dermal cylindroma has been reportred in the superomedial orbit.

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