Cases reported "Eyelid Diseases"

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1/11. Phakomatous choristoma: a case report and review of the literature.

    Phakomatous choristoma is a rare congenital lesion of the eyelid that can be clinically and/or histologically mistaken for a cyst, cutaneous adnexal neoplasm, or an ocular adnexal oncocytoma. Only 13 such cases have been previously described, mostly in the English language ophthalmic literature. Zimmerman reported the first case in 1971 and proposed the lesion to be of lenticular anlage origin, a theory that has been widely accepted. We report an additional case occurring in an 8-week-old male infant with a firm nodule of the right lower eyelid that was present since birth. A 15 x 12 x 2 mm circumscribed solid nodule with a homogenously white cut surface was surgically excised. Histologically, this lesion was comprised of cuboidal cells forming cystically dilated and irregularly branched ducts and cords within a densely fibrotic stroma. Also present were eosinophilic basement membranelike material, psammoma body-like calcifications and intraluminal degenerated ghost cells. The immunohistochemical profile of the epithelial cells included strong immunoreactivity for vimentin, focal weak staining for S-100, and negative staining for cytokeratin, epithelial membrane antigen, synaptophysin, and chromogranin. The irregularity of the ducts and cords of epithelial cells within the densely fibrotic stroma resembled an infiltrative neoplasm of cutaneous adnexal or lacrimal duct origin. However, the site of involvement, the peculiar basement membrane material, ghost cells, and immunohistochemical profile were features that helped to distinguish phakomatous choristoma from an infiltrative carcinoma. The correct identification of this lesion is essential to avoid an aggressive surgical excision, thus sparing the eyelid and lacrimal system. The purpose of this article is to bring attention to this rare entity, because it has not been described in either the dermatology or dermatopathology literature and furthermore, is not mentioned in any of the major dermatopathology texts.
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2/11. Phakomatous choristoma of the eyelid.

    This article describes the first report of phakomatous choristoma of the eyelid in korea. A six-month-old boy underwent excision of a congenital inferonasal orbital mass arising from the left lower lid. A dermoid cyst was suspected, however a diagnosis of phakomatous choristoma was made following conventional histology. An immunohistochemical study of this rare benign congenital tumor was conducted. The cuboidal epithelial cells comprising this choristoma showed strongly positive cytoplasmic staining with S-100 protein and vimentin. They also showed focally positive staining with a neuron-specific enolase, while they showed no immunoreactivity to cytokeratin or epithelial membrane antigen. The results of the immunohistochemical study support the conclusion that this tumor is of lenticular anlage origin.
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3/11. molluscum contagiosum, involving the upper eyelids, in a child infected with hiv-1.

    BACKGROUND: infection with molluscum contagiosum has been reported in pediatric and adult patients with acquired immune deficiency syndrome (AIDS), but rarely affecting eyelids. We have studied the viral phenotype, hiv-1 plasma viremia, p24 antigenemia, alterations of cellular immune function, and the ophthalmological status in a 5-year old human immunodeficiency virus type 1 (hiv-1)-infected girl, who developed multiple molluscum lesions, bilaterally involving upper eyelids with extension over the face and nose. methods: Detailed ophthalmological examination and immunological and virological studies were performed in a pediatric patient with hiv-1 vertical infection having extensive infection with molluscum contagiosum. RESULTS: The pediatric patient was emetropic; tricomegalia was present bilaterally, and alteration of the microvessels of the conjunctiva (microangiopathy) was observable in both eyes and structural (fibrilar) degeneration of the vitreous architecture in both eyes. There was no ophthalmoscopic sign of infectious retinitis or retinal microangiopathy. She had lymphopenia, very low percentage and absolute number of CD4 T cells but increased percentage of CD8 T cells. The in vitro lymphocyte proliferative response to phytohemagluttinin (PHA) was depressed as compared to healthy controls. She had high levels of viral HIV rna in her plasma and of p24 antigen in her serum, and the phenotype of the isolated hiv-1 was determined to be syncytium-inducing (SI). CONCLUSION: Although healthy persons may develop molluscum contagiosum, usually unilateral, as far as we are aware this report is the first to document a case of molluscum contagiosum with bilateral eyelid involvement in an hiv-1-infected pediatric patient. Our observations suggest that this type of infection may be present in HIV-infected children, associated with high viral load and possibly an SI viral phenotype, severe immunoregulatory abnormalities, and poor clinical status.
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4/11. Eyelid phakomatous choristoma.

    PURPOSE: To report a case of congenital phakomatous choristoma (PC) of the eyelid, a rare tumor of lenticular anlage in the subcutaneous tissue and dermis. CASE: A boy had a mass in the right lower eyelid near the inner canthus at birth. At age 10 months, the tumor was excised. RESULTS: The tumor cells showed intense immunoreactivity positive for S-100 protein, vimentin, and periodic acid-Schiff. Keratin markers, epithelial membrane antigen, glial fibrillary acidic protein, muscle specific actin, and epithelial membrane antigen were negative. The histopathologic features were consistent with those of a PC. CONCLUSIONS: This histopathologic and immunoreactivity study supports the proposal that PC is a rare entity of lenticular anlage origin. Only 18 such cases have been described previously.
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5/11. Acquired trichomegaly of the eyelashes: a cutaneous marker of acquired immunodeficiency syndrome.

    Acquired trichomegaly of the eyelashes was found in seven patients infected with human immunodeficiency virus type 1. These patients had advanced infection with severe T-helper cell depletion, high levels of p24 antigenemia, and inability to tolerate zidovudine therapy. Control of infection with other antiretroviral agents caused the trichomegaly to regress. Acquired trichomegaly may be a useful clinical marker for assessment of severity of infection.
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6/11. Allergic granulomatous nodules of the eyelid and conjunctiva. The XXXV Edward Jackson Memorial Lecture.

    We studied the clinical and pathologic features of 22 cases of granulomas of the conjunctiva or eyelids. All cases showed the histologic features of the Splendore-Hoeppli phenomenon, that is, a giant cell and eosinophil granulomatous reaction to an antigen-antibody precipitate originally described in relation to parasites or fungi. In four of seven typical cases selected for detailed description unidentified nematodes were found to be the cause of the condition. In light of these findings together with a review of similar "allergic granulomas" reported both in ocular tissues and elsewhere in the body, we considered the cause in the remaining cases. All 22 cases may have been caused by nematodes, as seems, probable in 14 of them, or the causative antigens may have been of widely different kinds. Although in our cases all ocular granulomas had an identical histology, this study did not resolve the problem of those cases where no causative agent was found. Thorough investigation of such cases in the future and the demonstration of their cause may elucidate the wider problem of nonocular allergic granulomas.
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7/11. XXXV Edward Jackson Memorial Lecture. Allergic granulomatous nodules of the eyelid and conjunctiva.

    Twenty-two cases of granulomas of the conjunctiva or eyelids were studied, and the clinical and pathologic features were tabled. All cases showed the histologic features of the Splendore-Hoeppli phenomenon, that is a giant cell and eosinophil granulomatous reaction to an antigen-antibody precipitate originally described in relation to parasites or fungi. Seven typical cases were selected for detailed description; in four of these cases, unidentified nematodes were the cause of the condition.
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8/11. vitiligo patterns simulating autoimmune and rheumatic diseases.

    Several variants of generalised vitiligo can be recognised by their cutaneous distribution. In some, certain anatomical regions--e.g., hands--are predominantly affected while the cutaneous depigmentation in other variants shows a similar anatomical distribution to that of the internal structures affected in some of the autoimmune and rheumatic diseases. Five patients have been seen, and three described by others, with vitiligo affecting skin of eyelids and lower front of neck, reminiscent of the anatomical distribution of lesions in thyrotoxicosis with exophthalmos: two of these patients have thyrotoxicosis. Another variant of vitiligo mimicks the anatomical distribution of lesions in ankylosing spondylitis, Reiter's syndrome, and ulcerative colitis and there are cases simulating lupus erythematosus, rheumatoid arthritis, and psoriasis. It is suggested that the parts of the body affected in each vitiligo variant and its corresponding internal disease constitute a set os mosiac patches with distinctive shared characters. These may have morphogenetic functions during embryonic development and be potentially autoantigenic later in life.
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9/11. Phakomatous choristoma of the eyelid: immunohistochemical observation.

    This article reports the first case of phakomatous choristoma of the eyelid in japan. The tumor occurred in a 2-week-old boy and was located in the left lower lid near the inner canthus. An immunohistochemical study of this rare, congenital tumor was performed. The immunohistochemical analysis revealed that the epithelial cells of this tumor showed positive staining for vimentin, S-100 protein, and neuron-specific enolase, while they had no immunoreactivity for cytokeratin, glial fibrillary acidic protein, epithelial membrane antigen, or a macrophage marker. Both the epithelial cells and the central contents of the islands in this tumor showed positive staining with anti-human alpha crystallin monoclonal mouse IgG. These results strongly indicated that a phakomatous choristoma was of a lenticular origin.
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10/11. Bilateral periocular actinic granuloma in a patient with renal failure: a clinicopathologic study.

    PURPOSE: We report a 50-year-old man with end-stage renal disease who has developed bilateral inner and outer canthal nodules. methods: The nodules were excised, fixed in 10% formalin and 2.5% glutaraldehyde, and processed for light and electron microscopy, respectively. RESULTS: Histological examination showed elastotic changes with associated granulomatous inflammation. The granulomatous reaction was centered around elastotic fibers, and some giant cells engulfed the fibers. Areas of dystrophic calcification were identified. A diagnosis of actinic granuloma was made. The differential diagnosis and histopathology of actinic granuloma, i.e. granuloma annulare, rheumatoid nodule, and necrobiotic xanthogranuloma, are discussed. CONCLUSION: This case of an actinic granuloma originating in the sun-damaged periocular skin provides further evidence that elastotic degeneration may be associated with a giant cell reaction. It is, however, unclear which antigens are responsible for initiating the granulomatous response and whether elastotic material on its own can elicit a granulomatous reaction.
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