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

Intravascular Papillary Endothelial Hyperplasia (Contd.)

HISTOPATHOLOGY This lesion lies within a blood vessel of the dermis or subcutis. It is composed of numerous, small, eosinophilic papillae covered by a layer of endothelial cells that are often flattened. The endothelial cells lack cytological atypia and mitotic activity. Thrombus is often adjacent to the lesion and is commonly in varying stages of organization.

DIFFERENTIAL DIAGNOSIS The most important lesion in the differential diagnosis is angiosarcoma from which it must be distinguished. Other similar lesions include pyogenic granuloma, hemangioma, intravascular fasciitis, and organized thrombus.

TREATMENT Biopsy is often required for diagnosis. Management is surgical by opening the vessels and removing the mass. For lesions in areas that are not readily accessible surgically, like the deep orbit, radiosurgery has proven effective.

REFERENCES

Barras C, Olver JM, Cole C, Seet JE. Intravascular papillary endothelial hyperplasia (IPEH) mimicking a lacrimal sac mass. Eye 2001; 15:685–687.

Cagli S, Oktar N, Dalbasti T, et al. Intravascular papillary endothelial hyperplasia of the central nervous system—four case reports. Neurol Med Chir 2004; 44:302–310.

Font RL, Wheeler TM, Boniuk M. Intravascular papillary endothelial hyperplasia of the orbit and ocular adnexa. A report of five cases. Arch Ophthalmol 1983; 101:1731–1736.

Ohshima T, Ogura K, Nakayashiki N, Tachibana E. Intravascular papillary endothelial hyperplasia at the superior orbital f issure: report of a case successfully treated with gamma knife radiosurgery. Surg Neurol 2005; 64:266–269.

Patt S, Kaden B, Stoltenburg-Didinger G. Intravascular papillary endothelial hyperplasia at the fissure orbitalis superior: a case report. Clin Neuropathol 1992; 11:128–130.

Shields JA, Shields CL, Eagle RC Jr, Diniz W. Intravascular papillary endothelial hyperplasia with presumed bilateral orbital varices. Arch Ophthalmol 1999; 117:1247–1249.

Sorenson RL, Spencer WH, Stewart WB, Miller WW, Kleinhenz RJ. Intravascular papillary endothelial hyperplasia of the eyelid. Arch Ophthalmol 1983; 101:1728–1730.

Truong L, Font RL. Intravascular papillary endothelial hyperplasia of the ocular adnexa. Report of two cases and review of the literature. Arch Ophthalmol 1985; 103:1364–1367.

Weber FL, Babel J. Intravascular papillary endothelial hyperplasia of the orbit. Br J Ophthalmol 1981; 65:18–22. Werner MS, Hornblass A, Reifler DM, Dresner SC, Harrison W. Intravascular papillary endothelial hyperplasia:

collection of four cases and a review of the literature. Ophthal Plast Reconstr Surg 1997; 13:48–56.

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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 nontender mass. There are no epithelial changes or erythema. The lesion is mobile and can be elongated and tubular to palpation.

HISTOPATHOLOGY This lesion occurs in veins and represents the intravenous analog of pyogenic granuloma of the skin or conjunctiva. There is a proliferation of blood vessels within the vessel lumen, and sometimes a stalk connecting the lesion to the vein wall can be identified. On other occasions, the lesion appears to be floating within the vessel lumen or it may completely occlude the lumen. In addition to the endothelial cells, smooth muscle cells can be demonstrated immunohistochemically within the tumor and are presumed to represent residua of the vein wall.

DIFFERENTIAL DIAGNOSIS The differential diagnosis includes lesions such as intravascular papillary endothelial hyperplasia, pyogenic granuloma, angiosacoma, hemangioma, Kaposi’s sarcoma, and organized thrombus.

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Intravascular Pyogenic Granuloma (Contd.)

TREATMENT Treatment is often required for diagnosis. Removal is accomplished by opening the vein with removal of the mass which shells out easily.

REFERENCES

Hung CH, Kuo HW, Chiu YK, Huang PH. Intravascular pyogenic granuloma arising in an acquired arteriovenous malformation: report of a case and review of the literature. Dermatol Surg 2004; 30:1050–1053.

Inaloz HS, Patel G, Knight AG. Recurrent intravascular papillary endothelial hyperplasia developing from a pyogenic granuloma. J Eur Acad Dermatol Venereol 2001; 15:156–158.

Truong L, Font RL. Intravascular pyogenic granuloma of the ocular adnexa. Report of two cases and review of the literature. Arch Ophthalmol 1985; 103:1364–1367.

Ulbright TM, Santa Cruz DJ. Intravascular pyogenic granuloma: case report with ultrastructural findings. Cancer 1980; 45:1646–1652.

Inverted Follicular Keratosis

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.

CLINICAL PRESENTATION Inverted follicular keratosis presents as a small, solitary, well-demarcated, hyperkeratotic or wart-like keratotic mass most commonly on the upper eyelid and cheek, Rarely, it may be pigmented simulating a melanocytic tumor. This lesion may show scaling and exophytic projections presenting as a cutaneous horn. The lesion typically appears weeks to months before presentation, but sometimes may be present for many decades. Inverted follicular keratosis shows a growth pattern with epidermis extending over the base and sides of the lesion and then taking an abrupt inverted or downward turn towards the central epithelial mass.

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Inverted Follicular Keratosis (Contd.)

HISTOPATHOLOGY This lesion resembles seborrheic keratosis except that the proliferating epidermis protrudes into the dermis instead of being exophytic. It is often classified as a subtype of seborrheic keratosis, distinguished by its endophytic growth and the presence of whorls of maturing squamous epithelial cells (“squamous eddies,” illustrated on p. 47). There are variable numbers of horn cysts, and there may be an intense infiltrate of chronic inflammatory cells, as seen in the photomicrograph on the left.

DIFFERENTIAL DIAGNOSIS The differential diagnosis includes verruca vulgaris, papilloma, senile keratosis, seborrheic keratosis, cutaneous horn, keratoacanthoma, and squamous cell carcinoma.

TREATMENT Complete surgical excision is recommended, as recurrences are common with incomplete removal.

REFERENCES

Azzopardi JG, Laurini R. Inverted follicular keratosis. J Clin Pathol 1975; 28:465–471.

Boniuk M, Zimmerman L. Eyelid tumors confused with squamous cell carcinoma, II: inverted follicular keratosis. Arch Ophthalmol 1963; 69:698–707.

Lever WF. Inverted follicular keratosis is an irritated seborrheic keratosis. Am J Dermatopathol 1983; 5:474. Mehregan AH. Inverted follicular keratosis is a distinct follicular tumor. Am J Dermatopathol 1983; 5:467–470. Sassani JW, Yanoff M. Inverted follicular keratosis. Am J Ophthalmol 1979; 87:810–813.

Schweitzer JG, Yanoff M. Inverted follicular keratosis. Ophthalmology 1987; 94:1465–1468.

Sim-Davis D, Marks R, Wilson-Jones E. Inverted follicular keratosis: surprising variant of seborrheic wart. Acta Dermatol Venereol (Stockh) 1976; 56:337–344.

Yanoff M. Most inverted follicular keratoses are probably verrucal vugares. Am J Dermatopathol 1983; 5:475.

Juvenile Xanthogranuloma

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 granulomatous 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 is no sexual or racial predilection. Other sites of ocular involvement include the orbit, conjunctiva, cornea, episclera, iris, and ciliary body. Iris lesions are associated with spontaneous hyphema, unilateral glaucoma, uveitis, and heterochromia iridis. Although lesions are common on the face

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Juvenile Xanthogranuloma (Contd.)

and neck, they may also occur on the trunk or extremities. Visceral involvement is rare, however, with a predilection for lung, spleen, testis, pericardium and gastrointestinal tract.

CLINICAL PRESENTATION The eyelid is the most frequent site of ocular involvement. Lesions on the lid often appear quite suddenly. They typically present as a rapidly growing solitary papular or nodular lesion with a rubbery or cartilaginous consistency, varying in size from few millimeters to 1.5 cm. The lesions are typically yellow in color, frequently with an orange overtone. With time they may enlarge and become yellow-brown. New lesions can arise within a few months of the appearance of the first lesion, near or distant to the primary site. The lesions may resolve spontaneously within months or a few years, leaving a slightly atrophic area that may be hyper or hypopigmented. Very large lesions may show ulceration and crusting. Intraocular lesions can be associated with glaucoma and even blindness.

HISTOPATHOLOGY Lipid-laden macrophages (foam cells) and Touton giant cells are the distinguishing features of xanthogranulomas. The Touton giant cells have multiple nuclei forming a circle near the middle of the cell surrounding a core of brightly eosinophilic cytoplasm. The cytoplasm around the outside of the nuclei is vacuolated and clear or palely eosinophilic. Lymphocytes, plasma cells, and occasional eosinophils are scattered among the foam cells and Touton giant cells.

DIFFERENTIAL DIAGNOSIS Xanthomas or xanthelasmas, syringoma, and molluscum contagiosum most commonly mimic eyelid involvement of JXG.

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Juvenile Xanthogranuloma (Contd.)

TREATMENT Surgical biopsy may be necessary to establish the diagnosis. Treatment of juvenile xanthogranuloma should be tailored to the individual case. The majority of lesions are asymptomatic, and may sometimes resolve spontaneously. For isolated skin lesions limited to the eyelid or the epibulblar tissue, observation alone may be appropriate. Skin lesions may be removed surgically for improved cosmesis. For intraocular lesions subconjunctival or systemic corticosteroid therapy, irradiation, and surgery have been reported to give a good response. With systemic visceral involvement, chemotherapy is indicated.

REFERENCES

Cadera W, Silver MM, Burt L. Juvenile xanthogranuloma. Can J Ophthalmol 1983; 4:169–174.

Chalfin S, Lloyd WC. Juvenile xanthogranuloma of the eyelid in an adult. Arch Ophthalmol 1998; 116:1546–1547. Hamdani M, El Kettani A, Rais L, et al. Juvenile xanthogranuloma with intraocular involvement. A case report. J Fr

Ophtalmol 2000; 23:817–820.

Harley RD, Romayananda N, Chan GH. Juvenile xanthogranuloma. J Pediatr Ophthalmol Strabismus 1982; 19:33–39. Nakatani T, Morimoto A, Kato R, et al. Successful treatment of congenital systemic juvenile xanthogranuloma

with Langerhans cell histiocytosis-based chemotherapy. J Pediatr Hematol Oncol 2004; 26:371–374.

Nasour AM, Traboulsi E, Frangieh G. Multiple Recurrences of juvenile xanthogranuloma of the eyelid. J Pediatr Ophthalmol Strabismus 1985; 22:156–157.

Schwartz TL, Carter KD, Judisch GF, Nerad JA, Folberg R. Congenital marcronodular juvenile xanthogranuloma of the eyelid. Ophthalmology 1991; 98:1230–1233.

Kaposi’s 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 associated with exogenous immunosuppression. Some authors have theorized that this lesion may have a viral etiology.

CLINICAL PRESENTATION Kaposi’s sarcoma usually presents as a red to brown macule that enlarges to become a more elevated, highly vascular, purple or red nodule on the cutaneous aspect of the

(Courtesy of Richard S. Smith, M.D.)

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Kaposi’s Sarcoma (Contd.)

eyelids, the caruncle, or on the conjunctiva. Rarely, it may involve the lacrimal sac and orbit. In milder cases, skin lesions often wax and wane, with some regressing completely, leaving atrophic, pigmented scars.

HISTOPATHOLOGY The patch stage of Kaposi’s sarcoma has a proliferation of irregular, often jagged, vascular channels within the dermis. The vessels are thin walled, lined by plump or inconspicuous endothelial cells, some have perivascular lymphocytes and plasma cells, and there may be extravasated erythrocytes and hemosiderin deposits. The plaque and nodular stages of Kaposi’s sarcoma have interlacing bundles of spindle cells and poorly defined, slit-like vessels within the dermis. Chronic inflammatory cells are present focally, and there are extravasated erythrocytes and deposits of hemosiderin. Small, eosinophilic hyaline globules may be intracellular or extracellular and provide a clue to the diagnosis; the globules are positive using periodic acid-Schiff (PAS) stain and they are bright red with Masson’s trichrome stain.

DIFFERENTIAL DIAGNOSIS Lesions that may mimic a Kaposi’s sarcoma include pyogenic granuloma, hemangiopericytoma, angiosarcoma, glomus tumor, lymphoma, metastatic tumor, cavernous hemangioma, chalazion, foreign body granuloma, and conjunctival hemorrhage.

TREATMENT The goal of therapy is to relieve ocular irritation, mass effect, and disfigurement. Accepted treatment options include surgical excision, cryotherapy, irradiation, chemotherapy, and immunotherapy such as intralesional injection with interferon alpha-2a. For patients receiving chemotherapy or for whom chemotherapy is planned for systemic Kaposi’s sarcoma, local treatment often proves unnecessary. Although therapy may reduce or clear the visible lesions, it is not curative. Low dose radiotherapy in the range of 2000 to 3000 cGy can be effective for localized lesions.

REFERENCES

Alexander C. Kaposi’s sarcoma with ocular manifestations. Am J Ophthalmol 1963; 55:625.

Bookstead JH, Wood GS, Gletcher V. Evidence for the origin of Kaposi’s sarcoma from lymphatic endothelium. Am J Pathol 1985; 119:292–300.

Brun SC, Jakobiec FA. Kaposi’s sarcoma of the ocular adnexa. Int Ophthalmol Clin 1997; 37:25–38.

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Kaposi’s Sarcoma (Contd.)

Dugel PU, Gill PS, Frangieh GT, Roa NA. Treatment of ocular adnexal Kaposi’s sarcoma in acquired immunodeficiency syndrome. Ophthalmology 1992; 99:1127–1132.

Friedman-Kien AE, Saltzman BR. Clinical manifestations of classical, endemic African and epidemic AIDS-associated Kaposi’s sarcoma. J Am Acad Dermatol 1990; 22:1237–1250.

Holecek MJ, Harwood AR. Radiotherapy of Kaposi’s syndrome. Cancer 1978; 41:1733–1738.

Kalinske M, Leone CR. Kaposi’s sarcoma involving eyelid and conjunctiva. Ann Ophthalmol 1982;14:497–499.

Munteanu G, Munteanu M, Giuri S. Conjunctival-palpebral Kaposi’s Angiosarcoma: report of a case. J Fr Ophtalmol 2003; 26:1059–1062.

Pang C, Kong L. Kaposi’s sarcoma of eyelid and conjunctiva. Zhonghua Yan Ke Za Zhi 1999; 35:262–264.

Shields JA, DePotter P, Shields CL, Komarnicky LT. Kaposi’s sarcoma of the eyelids: response to radiotherapy. Arch Ophthalmol 1992; 110:1689.

Shuler JD, Holland GN, Miles SA, et al. Kaposi sarcoma of the conjunctiva and eyelids associated with the acquired immunodeficiency syndrome. Arch Ophthlamol 1989; 107:858–862.

Soll DB, Redovan EG. Kaposi’s sarcoma of the eyelid as the initial manifestation of AIDS. Ophthal Plast Reconstr Surg 1989; 5:49–51.

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 histologically, with prolongation of the proliferative phase of wound repair they have been described as “incomplete tumors.” Although any body area can be affected, keloids commonly develop on the face, neck, chest, shoulders, and back. Keloids on the eyelid skin are relatively rare, even in patients who are prone to keloid formation. These scars tend to occur with greater frequency in patients with darker skin tones, but patients of any skin type can develop this exuberant clinical response.

CLINICAL PRESENTATION Keloids are generally painless proliferations of dermal collagen that appear as red or purple, raised, firm nodules which grow beyond the margins of the original sites of injury. There texture is shiny, with minimal to no epithelial markings.

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Keloid (Contd.)

HISTOPATHOLOGY Mature keloids are composed of haphazardly arranged, broad, brightly eosinophilic, glassy, collagen bundles. The collagen bundles tend to form variably sized nodules. Fibroblasts are along the collagen bundles, but the overall cellularity is reduced compared to other scars due to the space occupied by the broad collagen bundles. Early keloids have greater cellularity, abundant fibrillary collagen, and fewer of the thick collagen bundles.

DIFFERENTIAL DIAGNOSIS Keloids are most commonly confused with hypertrophic scars.

TREATMENT The treatment of keloid can be very difficult. Traditionally keloids have been dermabraded, chemically peeled, excised, irradiated, and injected with corticosteroids. Cryotherapy has been reported to be effective in reducing the size of the scar. Although CO2 laser resurfacing may not be suitable in darker skin types, and in fact may promote further keloid formation, the 585-nm pulsed dye laser may be helpful in eradicating these lesions. Newer treatment modalities include injections of interferon and 5-fluorouracil. Silicone gel sheeting may reduce the incidence and degree of scarring by modulating expression of basic fibroblast growth factor.

REFERENCES

Al-Attar A, Mess S, Thomassen JM, Kauffman CL, Davison SP. Keloid pathogenesis and treatment. Plast Reconstr Surg 2006; 117:286–300.

Atiyeh BS, Costagliola M, Hayek SN. Keloid or hypertrophic scar: the controversy: review of the literature. Ann Plast Surg 2005; 54:676–680.

Chen MA, Davidson TM. Scar management: prevention and treatment strategies. Curr Opin Otolaryngol Head Neck Surg 2005; 13:242–247.

Hanasono MM, Lum J, Carroll LA, Mikulec AA, Koch RJ. The effect of silicone gel on basic fibroblast growth factor levels in fibroblast cell culture. Arch Facial Plast Surg 2004; 6:88–93.

Marneros AG, Krieg T. Keloids—clinical diagnosis, pathogenesis, and treatment options. J Dtsch Dermatol Ges 2004; 2:905–913.

Meshkinpour A, Ghasri P, Pope K, et al. Treatment of hypertrophic scars and keloids with a radiofrequency device: A study of collagen effects. Lasers Surg Med 2005; 37:343–349.

O’Brien L, Pandit A. Silicone gel sheeting for preventing and treating hypertrophic and keloid scars. Cochrane Database Syst Rev 2006; CD003826.

Poochareon VN, Berman B. New therapies for the management of keloids. J Craniofac Surg 2003; 14:654–657. Ragoowansi R, Cornes PG, Moss AL, Glees JP. Treatment of keloids by surgical excision and immediate postoperative

single-fraction radiotherapy. Plast Reconstr Surg 2003; 111:1853–1859. Tanzi EL, Alster TS. Laser treatment of scars. Skin Therapy Lett 2004; 9:4–7. Thompson LD. Skin Keloid. Ear Nose Throat 2004; 83:519.

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Keratoacanthoma

INTRODUCTION Keratoacanthoma is a relatively common squamoproliferative neoplasm that occurs on sunexposed 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 carcinoma. However, the two lesions have been shown to be distinct on immunohistochemical grounds. Although multiple, aggressive and giant varieties have been described, most are solitary and self-limited. The lesion may also occur as part of Muir-Torre syndrome (skin lesion associated with an internal malignancy). The etiology is unknown but sunlight, trauma, chemical carcinogens, UVB exposure, and immunocompromised status have been implicated as etiologic factors.

CLINICAL PRESENTATION The classic lesion appears spontaneously as a small flesh-colored papule that evolves very rapidly over three to six weeks to a 0.5 to 2.5 cm dome-shaped violaceous or brownish nodule. The nodule is usually umbilicated with a distinctive central crater filled with a keratin plug. The edges of the crater may have elevated rolled margins. Lesions have a predilection for the lower eyelid where more than half are located. It typically undergoes spontaneous involution within four to six months to leave an atrophic scar. Lesions that occur on the eyelids may produce ectropion or ptosis, and occasionally may cause destructive changes.

HISTOPATHOLOGY Keratoacanthomas have a proliferation of well-differentiated, keratinizing squamous epithelium at the sides and bottom of the lesion, with a central keratin-filled crater that enlarges as the lesion matures. A key diagnostic feature is a lip of epidermis (collarette) overlapping the central crater. The lesion usually has a relatively even lower border that remains superficial to

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