Light and Electron Microscopy the Uveo-retinal Tissues in Behcet's Disease

Huh, Warne; Bong, Woo Sik; Kim, Jae Ho; Rhee, Sang Wook

Abstract

In 1937, Behcet's, a Turkish dermatologist, identified firstly as a distinct disease entity presenting the three cardinal symptoms of relapsing iridocyclitis with hypopyon, aphthous lesions in the mouth, and the ulceration of the genitalia, but although these may be the main features of the condition the numerous reports of involvement of other systems of the body such as the blood vessels, the nervous system, the skin and the joints make it clear that it is not restricted to the eyes, the skin and mucous membranes but is systemic in its incidence. Here, authors observed one typical case of Behcet's disease and its uvea-retinal tissues of an enucleated eyeball was studied by light and electron microscopies.
MATERIALS AND METHODS
Left eye ball was enucleated from a patient with Behcet's disease in the stage of remission. This twenty yr. old boy was admitted due to visual disturbance of both eyes, ocular pain and left frontal headache. He was been suffered from recurrent attacks of aphthous lesion of mouth, ulceration of urogenital area, erythema nodosum on leg skin and lately uveitis for the last five years. And that time, he was been treated with Depo-Medrol and gamma globulin at local clinic. At admission, the visual acuity of both eyes were light perception with signs of uveitis and secondary glaucoma, the lens was also opacified mildly on anterior capsular and subcapsular areas due to the long exsisting posterior synechia. And the ocular tension of the right eye was in hypotony but of the left eye was increased up to 40 mmHg. He was been treated systemically at our Department. But his ocular conditions were not improved, inspite of combination surgery of cyclocryothermy especially for glaucomatous eye. The left eye was finally enucleated for pain control. For light and electron microscopy, this enucleated eye ball was sent to the pathologic laboratory. The half of sectioned eye ball was fixed in formalin and the tissues was then stained with hematcxylin-eosin and periodic acid-Schiff stains. Another half of anterior-posterior sectioned eyeball was fixed in 2.5% glutaraldehyde and 1% csmium acid for electron microscopy. And after routine procedures of preparation, this specim.
RESULTS
Anterior chamber was filled moderately with some fibrinoid exudate and round cells especially atthe angle. The Schlemm's canal was patent but the trabecular meshwork was infiltrated with round cells. And anterior surface of the iris was covered with fibrinoid exudates. Perivascular round cell infiltration of iris stromal vessels and some atrophic changes of the iris stroma were prominent findings In ciliary body, there was evidenced diffuse non-specific inflammation and degenerative changes of the ciliary muscle. By electron microscopy, vacuolization of the basement membrane of capillaries and perivascular infiltration of plasma cells and round cells were presented in the ciliary body. Atrophic and degenerative changes of the choroid were another features with the roundu cell infiltration in perivascular area. The retina was totally detached from its pigment layer. Diffuse retinal degeneration with gliosis and focal hemorrhagic infarcts revealed in this case. The retina and the vitreous body showed pronounced permeation by round cells and polymorphoneutrophile leucocytes and by fibrinoid exudates. By electron microscopy, picknosis and necrobiosis of the outer nuclear layer was characterized with some thickened capillary walls which were accompanied with degenerated smooth muscles fi bers. COMMENTS: In 1953, Sezer stated that the severe necrosis in the ciliary body and peripheral choroid of nonspecific character were the specific findings. In all cases there was a chronic inflammatory mononuclear inflammation of the granulomatous type particularly around the vessels, mainly in the uveal tract where areas of necrosis result, and also around the retinal vessels. The retina is often detached, hemorrhagic and necrotic in course (Duke-Elder, 1966). Mano and Baghadassarian (1964) found a perivascular infiltration with lymphocytes and plasma cells which is marked in the uveal tract Berlin (1944) noted peri vascular round cell infiltration around the central retinal artery and in the choroid. In 1960, Fishop's case with enucleated eye showed massive hemorrhagic infarction of the retina secondary to a recent thrombosis of the central retinal vein. Shikano (1971) reported that the histopathology of iris tissues in Behcet's disease revealed almost total absence of tissue destruction or of focallized inflammatory center, with the extravasated leucocytes which show minimal tendency to leucoclasia. And in the retina there showed strong permeation by leucocytes and by exudates with some evidence of thinning its thickness due probably to characteristic lytic process of its visual elements. Sugiura et al (1974) described round wondering cells which were often accumulated around the vessels assuming perivascular cuffs. In the retina acellular capillaries filled with the processes of Muller cells were often observed, and tubular structure resembling the nucleocapsid of myxovirus in shape and size, were detected with tubular bodies in the endothelial cell of blood vessels in all tissues examined. In authors case, There was evidenced diffuse nonspecific inflammation of whole ocular tissues, with the accompany of perivascular infiltration of round cells and plasm cells in the uveo-retinal tissues.

Light and Electron Microscopy the Uveo-retinal Tissues in Behcet's Disease

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