Clinical and laboratory prognosticators of atrophic papulosis (Degos disease): a systematic review

Abstract

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.

Keywords: Benign atrophic papulosis; Degos disease; Inflammatory cytokines; Malignant atrophic papulosis.

Fig. 1

Clinical images of patient with atrophic papulosis. Porcelain white atrophic papules with a partially-blanchable red ring of are seen on the elbow (a) and lateral thigh (b)

Fig. 2

Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) flow diagram for identifying cases of Degos disease

Fig. 3

MAP survival and organ involvement. a MAP organ involvement predominately affects the gastrointestinal (GI) tract and central nervous system (CNS) (75% and 68.5% respectively). b Time to systemic involvement from first instance of cutaneous onset, 50% of patients had systemic involvement after 1 year (n = 30). c MAP survival from cutaneous onset, 53% of patients died by 2 years in cases that reported mortality with timeline data (n = 34)