The pathology and pathogenesis of malignant atrophic papulosis (Degos' disease). A case study with reference to other vascular disorders.

[malignant atrophic papulosis]

The morphology and immunohistology in a case of malignant atrophic papulosis (Degos ' disease ), a rare vascular disorder of unknown etiology , are described . The vascular lesions affected middle class and small arteries and veins throughout the body and were histologically characterized by intimal proliferation in the absence of any appreciable inflammation . The lesions were categorized as early , intermediate or late . Early lesions consisted of cellular proliferation and edema of the intima with signs of immune complex deposition (IgM , C 3 ). Thrombosis was occasionally present as a secondary phenomenon in the affected vessel segments . In intermediate lesions the edema decreased and smooth muscle proliferation became apparent . Late lesions consisted of acellular intimal sclerosis with hyalinization and narrowing or obliteration of the vascular lumen . The media of the vessels remained always intact . In comparing these features to the pathology and pathogenesis of other vascular disorders they resembled the vascular lesions in a murine model of lupus erythematodes in which also considerable intimal proliferation occurred with thrombotic occlusion , but without appreciable inflammation . The murine model is associated with sustained low levels of circulating immune complexes and it is tempting to assume the same for Degos ' disease . The notion of an immune complex mediated non-inflammatory condition underlying this severe and often fatal vascular disorder of mainly young males may contribute to the eventual finding of a successful therapeutical regimen .

Diseases
Validation

Diseases presenting "successful therapeutical regimen" symptom

malignant atrophic papulosis

You can validate or delete this automatically detected symptom