科研之友
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摘要
background: although rare diseases, one third of all cases of post-inflammatory elastolysis and cutis laxa published in the international literature are from the state of minas gerais, in brazil. reports with long term follow-up of these patients are also scarce. objectives: to report nine cases observed from 1981 to 2004 and compare the findings with 20 cases described in the literature. methods: epidemiological, clinical, pathological and therapeutical variables were assessed. results: the onset of the disease is usually up to the age of four years (93%), and it is more common in african americans (8:1), females (4:1) and in tropical climate (9:1). other features include alpha-1-antitrypsin deficiency (7%), aortitis (7%), related skin conditions (62%), premature facial ageing (97%) and stable atrophic phase during childhood after months or years with inflammatory lesions (97%). biopsies of recent lesions showed a more intense inflammatory infiltrate and less elastolysis than those obtained from older lesions. reconstructive surgery yielded good results during atrophic phase and 0.05% topical tretinoin was ineffective. conclusions: in 62% of patients the acute phase of post-inflammatory elastolysis and cutis laxa is associated with different elastolysis-promoting inflammatoy dermatoses. the correct management of these conditions may reduce severity of atrophic lesions, which can also be treated by reconstructive surgery. alpha-1-antitrypsin deficiency should be investigated.
