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Topical retinoic acid in dysplastic and metaplastic keratinization of corneoconjunctival epithelium.

We report four cases of corneoconjunctival keratinization that were successfully treated with topical retinoic acid ointment. In two cases keratinization was due to squamous metaplasia and in two others it was secondary to intraepithelial corneoconjunctival neoplasia. Treatment reversed severe keratinization in a case of drug-induced pseudopemphigoid and stabilized the disease in one of the two affected eyes without additional treatment. In a case of ocular cicatricial pemphigoid, retinoic acid was useful as an adjuvant therapy to immunosuppression, by reversing keratinization of the conjunctiva. In two cases of corneoconjunctival neoplasia, lesions regressed markedly. Long-term treatment was well tolerated in three patients. Our findings suggest that retinoic acid ointment is effective in treating severe squamous metaplasia in cicatrizing diseases of the conjunctiva. Our findings indicate further that retinoic acid seems to inhibit growth of corneoconjunctival neoplasias and thus might be useful complementary therapy in this situation.

Administration, Topical↗

Visible corneal nerve fibers and neuromas of the conjunctiva--a syndrome of type-3 multiple endocrine adenomatosis in two generations.

A case report of multiple mucosal neuroma syndrome (multiple endocrine adenomatosis type 3) is presented in a mother and her two children. In all of them the eye involvement consisted of highly visible corneal nerves, neuromas of the conjunctiva, and thickened eye lids. The mother had mucosal neuromas of the tongue, and in the 4-year-old girl these were seen to appear during the follow-up period. The mother and children had medullary carcinoma of the thyroid. Marfanoid physiognomy was also one characteristic clinical feature.

Adult↗

Irradiation for conjunctival granulocytic sarcoma.

CASE HISTORY AND FINDINGS: A 73-year-old woman with a history of myeloproliferative syndrome (MPS) presented with bilateral chemosis, redness and burning of the eyes. The ocular motility was severely impaired. Ophthalmological examination revealed markedly distended conjunctivas on both sides. Biopsy disclosed conjunctival granulocytic sarcoma as an initial symptom of acute myelogenous leukemia (AML). Diagnosis was confirmed by peripheral blood smear and bone marrow aspiration. TREATMENT AND OUTCOME: The orbital tumor disappeared completely after local external beam irradiation with a total dose of 30 Gy and no further orbital recurrence occurred. With chemotherapy following irradiation transient hematological remission was achieved. 5 months after diagnosis the patient died of respiratory failure following atypical pneumonia as a consequence of her underlying disorder. CONCLUSION: Detection of orbital granulocytic sarcoma, even in the absence of typical leukemic symptoms is of practical importance, because treatment with irradiation can lead to stabilization or improvement in the patient's vision.

Aged↗

[Schimmelpenning-Feuerstein-Mims syndrome (SFM syndrome)].

We report on the case of a 70-year-old caucasian woman with a large linear sebaceous naevus with multifocal intralesional basaliomas and a lipodermoid of the left eye. This rare finding has been preserved because of the special history of the patient. The naevus spread on the left side from parietal to retroauricular, nuchal and sternal. Because of the patient's cardio-pulmonary insufficiency we excised the basaliomas under local anaesthesia. We also made a partial stripped excision of the left-sided parietal parts of the naevus.

Aged↗

[Immunochemical demonstration of human herpes virus 8 in conjunctival Kaposi's sarcoma].

BACKGROUND: Angiomatous lesions of the conjunctiva and chronic conjunctivitis are frequently difficult to differentiate. CASE: A 28 year old white male sought help because of a conjunctival swelling. A biopsy made elsewhere had revealed a capillary haemangioma of the conjunctiva. Recently striking alterations of the skin led to the suspicion of a Kaposi's sarcoma. Immunohistochemical and dermatological examinations confirmed the diagnosis of a Kaposi's sarcoma and viral testing of serum revealed an HIV-infection. Besides intensive antiviral therapy, our patient underwent local irradiation of the right eye, the nose and of the left shoulder with an external beam. CONCLUSIONS: Kaposi's sarcoma is the most frequent Aids-associated malignant tumor. In about 30% of all patients with Aids, a Kaposi's sarcoma is diagnosed. The angiomatous character may lead to the histopathological diagnosis of a haemangioma. Human herpes virus 8 is known to be the transforming element.

AIDS-Related Complex↗

[Management of conjunctival malignant melanoma associated with primary acquired melanosis (PAM) using 0.02% mitomycin C eyedrops].

BACKGROUND: The therapy of malignant diseases of the conjunctiva with local chemotherapy is an extension of the therapeutic options in this field. We report on our experience in the therapy of malignant melanomas of the conjunctiva associated with primary acquired melanosis (PAM). METHODS AND PATIENTS: Between March 1998 and April 2001, 13 patients with malignant melanoma of the conjunctiva associated with PAM (6 female; 7 male; mean age 57+/-13 years) were treated with local chemotherapy. The tumor was classified as stage pT2 (pN0, pM0) in seven patients, stage pT3 (pN0, pM0) in three patients and in the remaining three patients the lid was involved in the malignant process (pT4, pN0, pM0). Local chemotherapy (mitomycin C 0.02% eyedrops 5 times a day) was applied after incisional biopsies in 2 cycles for 14 days with a 14-day break. In 4 patients a third cycle was included. RESULTS: Regression of the tumor was observed after completion of the therapy in all cases. Severe ocular or systemic secondary effects were not seen in our patients. Nine patients were without recurrence within the follow-up time. In three patients, a recurrence of the disease was observed. In these cases, the eyelid was involved in the process. CONCLUSION: Local chemotherapy with mitomycin C is a useful option in the treatment of malignant melanomas of the conjunctiva associated with PAM if the tumor stage is pT3 or less. From our point of view the combination with incisional biopsy is of great benefit. Prognosis of conjunctival malignant melanomas involving the lid margin (pT4) is poor.

Administration, Topical↗

[Atopic keratoconjunctivitis: probably a risk factor for the development of conjuntival carcinoma].

BACKGROUND: Almost half of the patients with atopic dermatitis experience chronic inflammation of the eyelids, the conjunctiva and the cornea. Chronic inflammation is a possible cause for the development of malignancies, especially if associated with some kind of immunological defect as in atopic patients. So far, a correlation between atopic conjunctivitis and conjunctival malignancies has not yet been reported. Here, we present 7 atopic patients with conjunctival carcinoma or carcinoma in situ detected between February 2000 and August 2001. PATIENTS: All 7 patients had a long history of atopic dermatitis and chronic of inflammation of the conjunctiva and cornea. In all patients smears were examined by cytology and DNA cytometry. Furthermore, in 6 of the 7 patients a histopathological examination of conjunctival biopsies was performed. RESULTS: In 4 of the 7 patients invasive conjunctival carcinoma and in 2 carcinoma in situ were detected. Cytology and cytometry revealed conjunctival carcinoma or carcinoma in situ in the remaining patient. Histopathological examination could not be performed since the patient refused to have a conjunctival biopsy. CONCLUSIONS: These results suggest that atopic keratoconjunctivitis might be a risk factor for the development of conjunctival carcinoma.

Adult↗

[Progressive peripunctal nevus in an adult].

Nevi arise from migration of melanocytes into the epithelium. Over the years they experience a typical maturation, with the possibility of an increased thickness due to accompanying inflammation or fibrosis but actual growth of nevi is rare in adults. A 70-year-old patient presented with a growing tumour around the punctum lacrimale. The histological examination revealed a conjunctival nevus without any transformation. Peripunctal nevi are rare. The localisation of a nevus in the area of the punctum lacrimale was first published in 1931 and only 10 other patients with such a nevus have been reported. If at all, an enlargement of a peripunctal nevus has been described in younger patients. The patient presented here showed significant growth of the nevus at an atypically high age without any histological signs of malignancy or proliferation. The reason for this uncommon nevus enlargement remains unclear.

Aged↗

[Lymphoproliferative lesions of the ocular adnexa. Differential diagnostic guidelines].

The ocular adnexal lymphomas represent the malignant end of the spectrum of lymphoproliferative lesions which occur in the conjunctiva, eyelids, lacrimal gland and orbit. The new "W.H.O. Classification of Tumours of Haemopoietic and Lymphoid Tissues" is the most suitable for subdividing the ocular adnexal lymphomas, whereby the extranodal marginal zone B-cell lymphoma (EMZL) represents the most common lymphoma subtype. Management of patients with ocular adnexal lymphomas includes a systemic medical examination to establish the clinical stage of the disease. Most patients have stage IE disease and current recommended therapy for this is radiotherapy, while disseminated disease is treated with chemotherapy. Despite usually demonstrating an indolent course, EMZLs are renowned for recurrence in extranodal sites, including other ocular adnexal sites. Furthermore, Blastic transformation of EMZL with a corresponding aggressive clinical course has been described. Long-term follow-up with half-yearly examinations are therefore recommended. Major prognostic criteria for the ocular adnexal lymphomas include the age of the patient, anatomical location of the tumour, stage of the disease at first presentation, serum lactate dehydrogenase level at the time of diagnosis, lymphoma subtype as determined using W.H.O. lymphoma classification and the tumour cell growth rate. The clinical symptoms and histopathological findings of the differential diagnosis of lymphoproliferative lesions of the ocular adnexa are discussed.

Adult↗

[HIV seroprevalence in ophthalmologic patients of Cameroon].

BACKGROUND: It is difficult to estimate the exact HIV infection rates in countries such as Cameroon because of diagnostic and statistical problems. The majority of people seek help from traditional healers outside the health system. PATIENTS AND METHODS: A screening for human immunodeficiency virus type 1 (HIV-1) and type 2 (HIV-2) was performed on 2452 patients of the western province of Cameroon. All data were evaluated regarding HIV seroprevalence and ophthalmological findings in HIV-seropositive patients. The test covered all patients who came for cataract surgery (group 1), all outpatients with suspicious ophthalmological findings (group 2), and all remarkable patients of the collaborating department of general medicine (group 3) between 20 September 2000 and 20 September 2001. RESULTS: Of the 2452 screened patients, 467 (19.0%) were HIV seropositive. A positive test result was obtained in 29 (5.5%) of the 525 patients in group 1, 154 (35.6%) of the 433 patients in group 2, and 284 (19.0%) of the 1494 patients in group 3. The main ocular manifestations of the 154 HIV-seropositive patients in group 2 were uveitis (17.6%), squamous cell carcinoma of the conjunctiva (14.9%), zoster ophthalmicus (14.9%), and corneal ulcers (11.0%). CONCLUSIONS: This study shows that the seroprevalence of the screened population of Cameroon lies between 5.5% (results of group 1) and 19.0% (results of group 3).

AIDS-Related Opportunistic Infections↗

[Association of malignant tumors of the conjunctiva and HIV infection in Kinshasa (D. R. Congo). First results].

BACKGROUND: Conjunctival carcinomas are tumors especially of the 6th/7th decades. They occur in Germany with a frequency of 1/20,000. For years it has been well known that in Africa the number of malignant tumors of the conjunctiva has been increasing. The reasons for that are not well known. HIV is a possible pathogenetic cofactor. METHOD: A histological examination of 34 specimens was performed in Rostock on conjunctival tumors removed at the Ophthalmologic Department of the University of Kinshasa in 2001. The study included 23 female and 11 male patients aged 20-75 years (mean 42). All patients underwent serological investigation for HIV infection. RESULTS: Of 34 patients 18 were HIV positive. Of the HIV-positive patients, six had a squamous cell carcinoma, seven a CIN (conjunctival intraepithelial neoplasia) I-III, and five a pinguecula. Of the HIV-negative patients, five showed a squamous cell carcinoma, eight a CIN I-III, one a pinguecula, one a sebaceous gland carcinoma, and one a papilloma. The average age of the HIV-positive patients with a squamous cell carcinoma was 41 years compared to the HIV-negative patients with 58 years. CONCLUSIONS: There was no difference in frequency of conjunctival intraepithelial neoplasia between HIV-positive or HIV-negative patients. HIV-positive patients with a squamous cell carcinoma were on average 17 years younger. UV radiation is the main risk factor and HIV infection is a possible carcinogenic cofactor. Investigations on additional viral infections as possible pathogenetic factors and on changed tumor suppressor genes are needed.

AIDS Serodiagnosis↗

[Neurodermatitis. A risk factor for the development of squamous cell carcinoma].

Squamous cell conjunctival carcinomas (SCCC) are rare. We report on a 34-year-old patient with neurodermatitis and chronic conjunctivitis who presented with a therapy-resistant corneal ulcer induced by mechanical irritation diagnosed as SCCC. Chronic blepharoconjunctivitis seems to sustain the development of SCCC in patients with neurodermatitis because of their insufficient immune response.

Adult↗

[Adjuvant topical interferon-alpha-2b treatment in epithelial tumors of the ocular surface].

PURPOSE: The aim of this study was to evaluate the role of topical interferon-alpha-2b in the adjuvant treatment of corneal and conjunctival tumors. METHOD: In this noncomparative, prospective, interventional case series, five patients with histologically proven conjunctival intraepithelial neoplasia (CIN) after primary excision and amniotic membrane transplantation were treated with interferon (IFN)-alpha-2b eye drops five times daily over a period of 6 weeks (1 million IU/ml Intron A, Schering). An in situ hybridization technique was used to detect human papillomavirus (HPV) in all specimens. Frequent follow-up was undertaken clinically and photographically for evidence of tumor recurrence. RESULTS: In the follow-up period (13.2+/-4,97 months) no clinical evidence of recurrence with only limited treatment side effects such as mild conjunctival hyperemia were recorded after 6 weeks of interferon. In one CIN specimen HPV 16/18 could be detected. CONCLUSIONS: The combination of excisional biopsy and topical interferon-alpha-2b application seems to be an effective and safe treatment for conjunctival intraepithelial neoplasias. Therefore, we prefer this combined treatment to topical interferon-alpha-2b treatment alone, more destructive approaches such as radiation and cryotherapy, or treatment with antimetabolites such as 5-fluorouracil or mitomycin C.

Administration, Topical↗