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.

Atrophic papulosis (Köhlmeier–Degos disease) revisited: a cross‐sectional study on 105 patients

“…This fact has been mentioned in the literature and requires further insights and vigilance from physicians of various specialties 18 . Concerning the frequency of involvement of particular internal organs, an equal prevalence of the CNS and the GIT manifestations was observed in the current study, which stands in agreement with our previous work, 4 but not with older studies, in which emphasis has been placed on GIT involvement, which has been reported to be more common 5,8,19–21 . Neurological involvement has been reported more rarely, namely in 19–68.5% of patients 5 .…”

“…A striking trend towards a more frequent MAP diagnosis (59%) than in the previous study (29%) 4 was observed. In a literature review, 63.8% of patients had MAP and 36.2% suffered from BAP, 5 rates which are similar to our current results. This fact could be explained by the larger number of patients monitored now and a growing awareness of the disease among physicians.…”

“…A final important aspect of our current work is the follow‐up of the reported patients. With an overall mean follow‐up time of 3.29 years, our results satisfy the recommendation of the current systematic review for the follow‐up duration of prospective treatment studies 5 …”

“…Internal organ involvement is still definitely associated with a poor prognosis, as the median time from its occurrence to disease‐specific death is 0.83 years. In the systematic review by Lu et al ., 5 a 2.15‐year median survival time for MAP after initiation of systemic involvement has been calculated, which is slightly longer than in our cohort. CNS alterations were the most common cause of disease‐specific death in our study, followed by GIT complications, in accordance with previous studies 4,5,8 .…”

“…4 The disease is extremely rare, with a few 100 cases described in the literature to date. 5 Atrophic papulosis usually becomes first apparent between the second and fifth decades of life, with a slight female predominance. There are also a few case reports of atrophic papulosis in children.…”