Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0851184 (thinning)
 11,252 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Ophthalmological lesions enter in the definition of Cogan's syndrome, associated with vestibulo-auditory symptoms. They are present in almost one half of patients with Wegener's granulomatosis and in 10 to 20 p. 100 of patients with periarteritis nodosa, where they may be the initial symptom. Yet they are seldom described in leucocytoclastic angiitis of small vessels, 3 cases of which are reported here. The first case concerns a 71-year old woman with cutaneous leucocytoclastic angiitis which regressed within a few days under dapsone. However, the drug had to be withdrawn on account of acute haemolysis. Thereafter, new skin lesions and severe pain in the left eye with fall in visual acuity developed simultaneously. Eye examination showed a large, marginal ulceration of the cornea extending to the adjacent sclera and reflecting deep involvement of the sclerocorneal limbus. Systemic corticosteroid therapy in doses of 1 mg/kg/day was instituted, resulting in gradual disappearance of the skin lesions and stabilization of the ophthalmic lesion. There remains, however, thinning of the cornea and sclera which entails a definite risk of perforation should the angiitis recur. The second patient was a 54-year old man seen for fever, polyarthralgia, purpura of the lower limbs and rapidly extending necrotic ulceration of the scrotum. A few days previously, inflammatory chemosis of the right eye had suddenly developed. Under systemic corticosteroids (1.5 mg/kg/day), the ophthalmic symptoms rapidly regressed, but the patient died of digestive tract haemorrhage. The third case was that of a 36-year old woman with urticaria associated with arthralgias and diffuse myalgia.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Ophthalmologic manifestations of leukocytoclastic vasculitis. Apropos of 3 case reports]. 357 12

The purpose of this report is to describe the effective treatment of severe anterior segment inflammation due to Cogan syndrome through the use of topical administration of cyclosporin A. A 47-year-old female patient had been experiencing headaches and difficulties with her vision. Subsequent examination revealed the sudden onset of bilateral conjunctival injection and swelling of bilateral auricles. Despite the multiple treatment (systemic and topical corticosteroid and antibiotic therapy), necrotizing scleritis had appeared bilaterally and the scleral wall was thinning. Topical administration of 1% cyclosporin A was applied to both eyes 4 times a day. After 2 months of this therapy, the epithelial tissue covered the necrotizing tissue and her symptom of ocular pain was relieved and her corrected visual acuity was improved. This is the first case exhibiting that topical cyclosporin A is an effective treatment for severe anterior segment inflammation associated with Cogan syndrome.
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PMID:Effective treatment with topical cyclosporin A of a patient with Cogan syndrome. 1105 5