A 40-year-old individual presents for evaluation of the following lesions on his skin pictured below.
Which of the following is the most likely cause of mortality associated with this condition?
A. CNS bleed
B. Myocarditis
C. Pulmonary involvement
D. Gastrointestinal perforation
E. Ocular involvement
Rationale: The pictured findings above show multiple atrophic white papules with surrounding erythema which are pathognomonic for malignant atrophic papulosis (Degos disease). Degos disease is not completely understood but represents a vaso-occlusive vasculopathic disorder. Three categories of Degos lesions exist: 1) classic Degos disease with systemic involvement 2) "benign" Degos disease and 3) Degos-like lesions which can be seen with multiple connective tissue disorders.
Correct answer: D Gastrointestinal perforation
Classic Degos disease is associated with systemic involvement and the most common site of involvement leading to death is intestinal perforation. Systemic symptoms at the time of presentation portend a poor prognosis.
Incorrect answers: A, B, C, E
Classic Degos disease can also be associated with CNS, pulmonary, cardiac, ocular, and renal involvement however to a lesser degree.
Additional reading at Chapter 146: Malignant Atrophic Papulosis (Degos Disease)
References:
1. A. Theodoridis, A. Konstantinidou, E. Makrantonaki, C.C. Zouboulis, Malignant and benign forms of atrophic papulosis (Köhlmeier–Degos disease): systemic involvement determines the prognosis, British Journal of Dermatology, Volume 170, Issue 1, 1 January 2014, Pages 110–115, https://doi.org/10.1111/bjd.12642