Case Report: Pediatric Malignant Atrophic Papulosis With Small Bowel Perforation and Positivity of Anticardiolipin Antibody

Malignant atrophic papulosis (MAP) is a life-threatening vasculopathy affecting the skin, gastrointestinal tract, central nervous system, pleural membrane, and pericardium. MAP carries a poor prognosis primarily because of its systemic involvement. It is extremely rare in children. Herein, we report a pediatric case of MAP with small bowel perforation and anticardiolipin antibody positivity.

A Case of Degos Disease Complicated By Constrictive Pericarditis In Remote Phase

Background: Degos disease, also known as malignant atrophic papulosis, is characterized by cutaneous manifestations due to chronic thrombo-obliterative vasculopathy. There have been reports of rare late-onset Degos disease complicated by constrictive pericarditis (CP). We report a case of CP caused by Degos disease that developed 20 years after diagnosis.Case presentation: A 62-year-old woman who has been taking aspirin for 20 years for Degos disease was hospitalized for worsening heart failure. The patient was diagnosed with CP and underwent pericardiectomy. Pathological findings suggested the involvement of Degos disease. The postoperative course was uneventful, and her heart failure and Degos disease did not worsen.Conclusions: This report suggests that Degos disease can cause long-term CP. Aspirin effectively inhibited the progression of Degos disease, and surgical treatment is necessary when heart failure due to CP is refractory to treatment.

Clinical and Laboratory Prognosticators of Atrophic Papulosis (Degos Disease): A Systematic Review

Background: Degos disease is a rare vascular disorder with a cutaneous-limited form, benign atrophic papulosis (BAP), and a systemic variant, malignant atrophic papulosis (MAP). Despite the poor prognosis of MAP, no study has established features associated with systemic disease.Objectives: The aims of this systematic review were to: (1) summarize clinical features and treatments implemented for patients with MAP and BAP (2) identify clinical and laboratory factors associated with the development of MAP, compared to BAP.Methods: We systematically searched MEDLINE and Embase from inception to April 2020. Demographic and clinical features of Degos patients were presented descriptively; multivariable logistic regression was performed to identify associations with MAP.Results: We identified 99 case studies, comprising 105 patients. MAP (64%) had a 2.15 year median survival time from cutaneous onset, most often with gastrointestinal or central nervous system involvement. We found that elevations in either of erythrocyte sedimentation rate (ESR) or C-reactive protein (CRP) were associated with systemic involvement (OR 2.27, p=0.023). Degos secondary to an autoimmune connective tissue disease was found to be inversely associated with MAP (OR 0.08, p=0.048).Conclusions: Elevated ESR or CRP is associated with MAP and may be a predictor of systemic involvement for patients with Degos disease. In addition, secondary Degos disease is associated with a favourable prognosis. Clinicians should be aware of the differences between primary and secondary Degos and the utility of ESR or CRP in identifying disease evolution to systemic involvement. The utility of ESR and CRP to identify systemic involvement should be further explored.

Systemic Dego`s Disease. Our Approach to the Diagnosis and the Follow-Up. A Case reports.

Degos’ disease, also known as “malignant atrophic papulosis” is a rare vasculopathy characterized by typical cutaneous lesions with an unknown etiology which was first described by Dego in 1942 (1), but another case, reported in 1941 by Köhlmeier, who interpreted it as thromboangiitis obliterans of the mesenteric vessels (2). It is an occlusive arteriopathy involving small-caliber vessels. Specifically, it is a progressive, small- and medium-size arterial occluding disease, leading to tissue infarction and initially involving the skin. Degos disease occurs both in a limited benign, cutaneous form and in a potentially lethal multiorgan, systemic variant. The disease has a male predominance (3:1), and sporadic cases of familial involvement have been recorded. (3-7). The involvement of the gastrointestinal tract and other organs has been noted in approximately 60% of reported cases (8). There are fewer than 50 living patients presently known worldwide, and fewer than 200 reported in medical literature. However, many individuals may go undiagnosed due to rarity of the disease (9,10). Most individuals develop symptoms between the ages of 20-50; however, cases outside of this age range have been reported as well, even as early as 8 months (1,6).