Pathology of the Gastrointestinal Tract

2017 Edition
| Editors: Fátima Carneiro, Paula Chaves, Arzu Ensari

Malignant Atrophic Papulosis (Degos’ Disease)

  • Elisabete Rios
  • Francisco Ferro de Beça
Reference work entry
DOI: https://doi.org/10.1007/978-3-319-40560-5_1651

Synonyms

Atrophic papulosquamous dermatitis; Degos’ disease; Fatal cutaneous-intestinal syndrome; Köhlmeier–Degos disease; Thromboangiitis cutaneointestinalis disseminata

Definition

Malignant atrophic papulosis (MAP) is a rare and often lethal, vaso-occlusive disorder involving small and medium-caliber vessels. It is characterized by narrowing and occlusion of the lumen by intimal proliferation and thrombosis, which leads to ischemia and infarction of the involved organ systems (most commonly the skin, gastrointestinal tract, and central nervous system). It was initially described by Köhlmeier in 1941 as a form of thromboangiitis obliterans, and recognized as a distinct vascular injury syndrome by Degos in 1942, hence the name (Thompson and Rosenbaum 2008).

Two variants have been described (Scheinfeld 2011). A cutaneous or benign variant, that is limited to the skin, with a relatively benign course. A systemic or malignant variant, in which there is a multiorgan involvement, with...

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References and Further Reading

  1. Ahmadi, M., Rafi, S. A., Faham, Z., Azhough, R., Rooy, S. B., & Rahmani, O. (2011). A fatal case of Degos’ disease which presented with recurrent intestinal perforation. World Journal of Gastrointestinal Surgery, 3, 156–158.CrossRefGoogle Scholar
  2. Magro, C. M., Poe, J. C., Kim, C., Shapiro, L., Nuovo, G., Crow, M. K., et al. (2011). Degos disease: A C5b-9/interferon-a-mediated endotheliopathy syndrome. American Journal of Clinical Pathology, 135, 599–610.CrossRefGoogle Scholar
  3. Scheinfeld, N. (2011). Commentary on ’Degos disease: A C5b-9 interferon-alpha;-mediated endotheliopathy syndrome by Magro et al.: A reconsideration of Degos disease as hematologic or endothelial genetic disease. Dermatology Online Journal, 17, 6.PubMedGoogle Scholar
  4. Thompson, O., & Rosenbaum, D. M. (2008). Uncommon causes of stroke. In R. Caplan Louis & J. Bogousslavsky (Eds.), Kohlmeier-Degos disease (malignat atrophic papulosis) (pp. 377–380). Cambridge: Cambridge University Press. Books Online. http://dx.doi.org/10.1017/CBO9780511544897.052
  5. Weedon, D. (2010). Weedon’s skin pathology (3rd ed.). Philadelphia: Churchill Livingstone/Elsevier.Google Scholar

Copyright information

© Springer International Publishing AG 2017

Authors and Affiliations

  • Elisabete Rios
    • 1
    • 2
    • 3
  • Francisco Ferro de Beça
    • 1
    • 2
    • 3
  1. 1.Department of PathologyCentro Hospitalar de São JoãoPortoPortugal
  2. 2.Faculty of Medicine of the University of PortoPortoPortugal
  3. 3.IPATIMUP – Institute of Pathology and Molecular Immunology of the University of PortoPortoPortugal