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)

Raynaud's phenomenon, uncommon in childhood, often heralds connective tissue disorder. Since microvascular abnormalities can be detected at an early stage of the connective tissue disease, especially in scleroderma, a specific diagnosis can be made in patients presenting with Raynaud's phenomenon alone or Raynaud's phenomenon associated with symptoms suggestive of connective tissue disease. Raynaud's phenomenon was studied in 11 consecutive children, 10 girls and 1 boy, ages 6 to 15. One child had a definite diagnosis of cutaneous polyarteritis nodosa. In 6 others connective tissue disease was suspected: 4 had arthritis, 2 has telangiectasia, leg ulcers and antinuclear antibodies. Of the remaining 4, one had hemiplegia and 3 Raynaud's phenomenon only. Oscillometry of the radial artery was reduced in 7 of 9. Decreased capillary resistance was found in 2 of 6, while abrupt thinning in conjunctival vessels was seen in 3 of 7. On nailfold capillaroscopy, reduced vascularity was noted in 5 of 11, dilated capillaries in 4 of 11, tortuousity in 2 of 11, capillary thinning in 1 of 11, capillary spasm in 1 of 11 and normal pattern in 3 of 11. Two patients presenting with Raynaud's phenomenon were found to have "scleroderma-like pattern" on nailfold capillaroscopy. One of them died 2 years later of cardiopulmonary sclerosis, and another developed esophageal stricture and Barrett's esophagus. Neither has sclerodermatous skin. In childhood Raynaud's phenomenon, nailfold capillaroscopy is a non-invasive examination enabling early diagnosis of "systemic scleroderma sine scleroderma".
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PMID:Raynaud's features in childhood. Clinical, immunological and capillaroscopic study. 149 54

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

Peripheral ulcerative keratitis (PUK) is a sight-threatening condition characterized by an epithelial defect, crescent-shaped stromal inflammation, and progressive stromal thinning. Peripheral ulcerative keratitis as a purely inflammatory entity is most commonly associated with collagen vascular diseases, including rheumatoid arthritis, polyarteritis nodosa, Wegener granulomatosis, systemic lupus erythematosus, and relapsing polychondritis. PUK can also be associated with infectious and inflammatory conditions such as hepatitis, syphilis, herpes simplex keratitis, fungal keratitis, Mooren ulcer, and marginal keratitis. We describe a case report of PUK associated with the inflammatory condition of sarcoidosis.
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PMID:Peripheral ulcerative keratitis in association with sarcoidosis. 2399 5