Atrophic papulosis (Köhlmeier-Degos disease): a cross-sectional study on 105 patients

Abstract

Introduction & Objective Atrophic papulosis (Köhlmeier-Degos disease/Degos disease) is a rare thrombo-obliterative microangiopathy classified in a malignant and a benign phenotype with significant prognostic variability. Large epidemiological studies contributing to better understanding of the course and prognosis of the disease as well as therapeutic studies are still lacking. The aim of the study was to assess the demographics, epidemiological data, and prognosis of patients with atrophic papulosis. Materials & Methods Present work is a continuation of the study published by Theodoridis et al. 2014. In total, 105 patients with atrophic papulosis, diagnosed between 2000 and 2021, were included after expert physician diagnosis in our center or with the help of the Degos Support Network, an international patient’s organization (www.degosdisease.com). Data was collected by utilizing the web-based REDCap platform (Vanderbilt University, Nashville, USA). Results The mean age of disease onset was 33.3 ± 18.3 years and male-to-female ratio was 1:1.6. Family history of Degos disease was documented in 5 cases (8.1%). Benign atrophic papulosis (BAP) with cutaneous symptoms were present in 43/105 cases (41.0%), while malignant atrophic papulosis (MAP) was diagnosed in 62/105 patients (59.0%). The median time from cutaneous lesions onset to systemic symptoms in MAP was 0.54 years (minimum -6, maximum 20). Gastrointestinal tract and nervous system were involved with equal frequency in 38/62 patients (61.3%); however neurological causes of death were slightly more frequent. The overall mortality rate in MAP was 32.3%. No case of death in BAP was observed. Conclusions To our knowledge this is the largest epidemiologic study on atrophic papulosis until yet. Although the MAP phenotype of the disease has been diagnosed with an increased frequency in the last years, overall mortality rate has remained stable.