A case of Degos disease in pregnancy

Abstract

Degos disease is characterized as a rare systemic vaso-occlusive disorder, although the exact pathophysiology is uncertain. Fewer than 200 patients have been reported in the literature, and only two reports describe the course of the disease during pregnancy. Here, we present the first reported case of the course of pregnancy in a woman with the systemic form of Degos disease. The patient had been diagnosed with Degos prior to pregnancy and was monitored throughout the duration of the pregnancy. Her presentation and treatment are described. There was no further exacerbation secondary to the pregnancy itself; the pregnancy course was uncomplicated and the baby unaffected to date.

Degos disease: a case report and review of the literature

“…Kohlmeier first described DD in 1941 [ 2 , 5 ]. The following year, Degos presented DD as a distinct entity and coined its name [ 3 ].…”

“…DD, also known as “malignant atrophic papulosis,” is a rare systemic vaso-occlusive disorder with unknown pathophysiology [ 6 ]. Some authors have stated that DD might result from coagulopathy, vasculitis, or endothelial cell dysfunction [ 5 , 7 ]. Anticoagulation strategies targeted to coagulopathy and immunosuppressive modalities that were successful with vasculitis have proved to be inadequate in treating systemic disease [ 7 ].…”

“…Anticoagulation strategies targeted to coagulopathy and immunosuppressive modalities that were successful with vasculitis have proved to be inadequate in treating systemic disease [ 7 ]. The characteristic raised papules with a red border and a depressed “china white” center are possibly due to dermal capillaries and venules developing a thrombogenic microangiopathy [ 5 ].…”

“…Acutely ill patients have been treated with heparin with success. However, other fibrinolytic agents were ineffective [ 5 ]. Immunosuppression with corticosteroids has been shown to worsen skin lesions and further complicate the course of the disease [ 5 ].…”

“…However, other fibrinolytic agents were ineffective [ 5 ]. Immunosuppression with corticosteroids has been shown to worsen skin lesions and further complicate the course of the disease [ 5 ]. The efficacy of eculizumab (monoclonal antibody targeted against complement C5) and treprostinil (synthetic prostaglandin agonist) has been reported in the literature [ 9 , 12 ].…”