Malignant atrophic papulosis (Degros disease). A report of two cases with clinical and histological studies

Abstract

Two women had malignant atrophic papulosis that affected the skin only. Immunologic studies showed normal amounts of serum immunoglobulins and complement and normal delayed hypersensitivity skin test results. Fibrinolysis autografts showed complete loss of fibrinolysis within the lesions but normal fibrinolysis in uninvolved skin. Histopathologic studies showed moderate inflammatory infiltration consisting of lymphocytes in addition to the typical cone-shaped regions of necrobiosis. Large amounts of mucin also were seen. Endovasculitis, thrombosis, and perivascular lymphocytic inflammation were noted in the subcutaneous arterioles of the lesions in only one of the patients. Enzyme histochemical studies showed oxidative enzymes, although in reduced amounts, in the epidermal and dermal cells of the affected skin. Electron microscopic studies of lesions showed paromyxovirus-like inclusions in endothelial cells and fibroblasts. The possibility of an unusual viral causation should be examined further.

Possible involvement of SDF-1/CXCL12 in the pathogenesis of Degos disease

“…This research supports previous findings regarding the existence of perivascular inflammatory cell infiltration without any symptom of vasculitis, 6,25-30 except lymphocytic vasculitis. 28,29,31 The intense staining of SDF-1/CXCL12 in lesions attributed to Degos disease, observed for the first time to our knowledge in this study, suggests the putative role of SDF-1/ CXCL12 in the pathogenesis of Degos disease. Further investigation into the role of SDF-1/CXCL12 and other chemokines that induce thrombosis might challenge current perspectives on Degos disease.…”

Lethal systemic degos disease with prominent cardio-pulmonary involvement

“…Although central porcelain white scar rimmed by erythema and telangiectasia is pathognomonic of DD, but this lesion is not familiar for most physicians. On the basis of this report and multiple similar reports,[81117–19] involvement of soles, palms, and genitalia is not rare in DD. On the other hand, pleural and pericardial involvements have been usually reported as incidental findings at autopsy or minor manifestations of prolonged disease.…”

Benign Cutaneous Degos' Disease

“…8 Elevated fibrinogen levels have also been reported in some patients, 9,10 but not in others. 8,11 Patients with increased platelet aggregation with adrenaline, collagen and serotonin, 12 and ADP and adrenaline 13 have been described. However, other patients have been reported to have normal platelet aggregation, 8,11 and there has also been a report of one patient with reduced platelet aggregation to ADP and adrenaline but normal aggregation to collagen.…”

“…8,11 Patients with increased platelet aggregation with adrenaline, collagen and serotonin, 12 and ADP and adrenaline 13 have been described. However, other patients have been reported to have normal platelet aggregation, 8,11 and there has also been a report of one patient with reduced platelet aggregation to ADP and adrenaline but normal aggregation to collagen. 7 In our patient, there was no evidence of abnormal platelet aggregation or abnormal blood fibrinolytic activity.…”