Reliability of Conjunctival Biopsy for Diagnosis of Ocular Mucous Membrane Pemphigoid: Redetermination of the Standard for Diagnosis and Outcomes of Previously Biopsy-Negative Patients.

Purpose: To demonstrate the reliability of conjunctival biopsy analyzed by direct immunofluorescence (DIF) and supplemented with avidin-biotin complex immunoperoxidase (ABC) in diagnosing oMMP, and report therapy response in biopsy-positive patients, particularly when previously biopsy-negative elsewhere.Methods: Retrospective outcomes review of 136 consecutive patients after conjunctival biopsy for suspected oMMP.Results: Among 136 patients, 66% were diagnosed with oMMP by DIF and 13% via supplemental ABC immunoperoxidase. Sensitivity increased from 79.6% with DIF to 95.6% with supplemental ABC. Among 57 biopsy-positive patients, 77% were in remission at 1-year follow-up and 88% after 2 years. Of 34 previous biopsy-negative but now biopsy-positive patients with a 2-year follow-up, 91% achieved remission, including all 16 diagnosed via DIF and ABC.Conclusion: Conjunctival biopsy analyzed by histopathology and DIF supplemented by ABC has high reliability for diagnosing oMMP and is a useful tool to use before starting long-term immunomodulatory therapy in a patient with suspected oMMP.

On the ocular findings in ochronosis: a systematic review of literature.

BACKGROUND: Ochronosis/Alkaptonuria is a tyrosine metabolism disorder where accumulation of homogentisic acid, in eye, skin, cartilage and several other connective tissues leads to a black pigmentation of the affected tissues. It is autosomal-recessive inherited in men with a frequency of 1-9/1,000,000. While it is clear that pigment deposits lead to joint destruction, renal stone formation and cardiac valvulopathy respectively, the significance of ocular findings is still unclear. We therefore aim to evaluate the frequency and clinical significance of ocular findings in ochronosis and discuss possible therapeutic options. METHODS: Systematic review of literature via Medline and Web of Science. Only case reports in English, German, French, Spanish or Italian documenting detailed ophthalmologic examination were included. RESULTS: Our search revealed 36 case reports including 40 patients. Average age at the onset of ocular signs was 40.6 years. The most frequent sign was symmetric brown sclera pigmentation present in 82.5 percent of the patients. "Oil-drops", brown pigment spots in the limbus are generally considered pathognomonic but were a little less frequent (75 percent). Vermiform pigment deposits at the level of the conjunctiva or increased conjunctival vessel diameter is also frequent. We found an increased incidence of central vein occlusion and elevated intraocular pressure going along with chamber angle hyperpigmentation. Another condition observed twice is rapid progressive astigmatism attributable to corneoscleral pigment accumulation. CONCLUSION: Our observations suggest that ocular findings are of double relevance. First, characteristic ocular findings can anticipate the time of diagnosis and second, ocular findings may complicate to various conditions putting sight at risk. Opthalmologists and general physicians should be aware of both. Therapeutic options include protein restriction, administration of high dose vitamin C or nitisonone. Evidence for all of them is limited.

Epidemiología de las lesiones conjuntivales y su correlación clínico-patológica / Epidemiology of conjunctival lesions and clinical-pathological correlation

Se realizó una revisión de los 1166 casos de lesiones conjuntivales recibidos por el Laboratorio de Patología Ocular del Hospital Clínico de la Universidad de Chile entre los años 1968-2005. En base a ella se describen los distintos diagnósticos histopatológicos según su frecuencia, distribución por sexo, edad, localización y tiempo de evolución. La edad promedio para los casos fue de 36 años con un 51,5 por ciento de hombres afectados y un tiempo de evolución promedio de 68 meses. Las lesiones más frecuentes fueron las pigmentarias (44,2 por ciento), seguidas de las epiteliales (22,7 por ciento) y los cuadros infecciosos (10,9 por ciento). La localización más frecuente para todas las lesiones fue bulbar. Se estableció, además, una correlación entre los diagnósticos clínicos e histopatológicos, la cual resultó ser positiva en un 60,9 por ciento, siendo marcadamente superior para las lesiones pigmentarias e inferior para lesiones degenerativas, cuadros infecciosos y tumores clasificados como pre-malignos o malignos.

We made a retrospective review of 1166 cases of conjuntival lesions, accepted for its study at the Laboratory of Ocular Pathology, University of Chile, from 1968 to 2005. Based on this survey, different histopathologic diagnosis were described, characterized by its frequency, gender distribution, age, location and time of evolution. The average age was 36 years, with a 51,5 percent being male, and an average time of evolution of 68 months. Most frequent lesions were pigmented (44,2 percent), followed by epithelial (22, 7 percent), and infectious diseases (10,9 percent). The most frequent location was bulbar. A clinical - pathologic correlation was established, being positive in 60,9 percent of cases. It was noticeable superior for pigmented lesions, and inferior for degenerative lesions, infectious disease and tumors classified as pre malignants or malignants.

[Surgical worm extraction in loa loa ophthalmia]. / Chirurgische Wurmentfernung bei Loa-Loa-Ophthalmie.

BACKGROUND: Loa Loa is a chronic parasitemic disease which is endemic in the tropical rain forests of Western Africa. Vector of this disease is a mangrove fly with the name Chrysops. Besides the eye worm and skin affections a systemic infection with microfilariae is common. PATIENT: A West African tourist from Bangibe showed up at the university eye clinic. His complaints were a red eye and a mobile subconjunctival tumor (Fig. 1) that showed vermiform movements. The worm was transparent and 4-5 cm in length. After topical anaesthesia and the attempt to paralyze the worm (1) with Pilocarpine 2% it vanished. Two days later the patient showed up in the morning for the planned blood test. No worm was visible at that time but at noontime the blood test was carried out and at that time the worm was visible in the nasal conjunctiva. This time the worm was removed without delay under topical anaesthesia. The worm was fixed with a forceps through the conjunctiva which was opened for 0.5 cm. The worm was grasped with a second forceps and drawn out under massive vermiform movement. Systemic therapy was recommended with Hetrazan (Diethylcarbamazine) using Corticosteroides and Antihistamine to minimize allergic side effects by the therapy due to the systemic microfilariae blood load. Eosinophilia was 8%. CONCLUSIONS: A subconjuctival Loa Loa worm can be removed under topical anaesthesia by fixing it with a forceps through the conjunctiva and opening it and grasping the worm with a second forceps. According to our experience the paralyzation with Pilocarpine cannot be realized. Careful systemic therapy avoiding reported allergic side effects with Hetrazan which is not available in Germany is necessary.