Elastólise pós-inflamatória e cutis laxa (doença de James Marshall): estudo de casos

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.