Commentary on 'Degos disease: a C5b-9/interferon-Î±-mediated endotheliopathy syndrome' by Magro et al: a reconsideration of Degos disease as hematologic or endothelial genetic disease.

[malignant atrophic papulosis]

Magro et al in April of 2011 published a new article in the American Journal of Clinical Pathology on the etiology and treatment of Degos Disease (DD ), and importantly , its fatal variant malignant atrophic papulosis (MAP ). Specifically , Magro noted that MAP is a disease involving the complement cascade that can be treated effectively with eculizumab . DD has two variants , a benign variant confined to the skin and a malignant (heretofore fatal ) variant that involves the skin and systemic organs . Five aspects of DD are discussed : (1 ) the clinical findings of DD , (2 ) thrombosis and DD , (3 ) the histology of DD , (4 ) the presence of viral like inclusions in the endothelial cells of patients with DD , and (5 ) the lack of any apparent immune defect that relates to DD . It seems the previous criteria for Degos Disease must be amended . Paroxysmal nocturnal hemoglobinuria (PNH ) is discussed and its relationship with DD explored . Eculizumab has been approved to treat paroxysmal nocturnal hemoglobinuria . A review of the data suggests that MAP is a hematological or endothelial disease like PNH . PNH , eculizumab , and data about DD is discussed to give a basis for understanding DD and speculate why eculizumab may be promising for the treatment of MAP .

Diseases
Validation

Diseases presenting "benign variant" symptom

familial hypocalciuric hypercalcemia

malignant atrophic papulosis

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