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Neurological Sciences

, Volume 40, Issue 10, pp 2201–2203 | Cite as

Dramatic neurological debut in a case of Köhlmeier-Degos disease

  • Dario Saracino
  • Francesco Paolo D’Armiento
  • Renata Conforti
  • Maddalena Napolitano
  • Andrea Elefante
  • Simone Sampaolo
  • Gianfranco PuotiEmail author
Letter to the Editor
  • 46 Downloads

Dear Editor,

Köhlmeier-Degos disease (KDD) is a rare thrombo-obliterative vasculopathy involving small- and medium-caliber vessels [1, 2]. Less than 200 cases have been reported and a possible genetic predisposition has been suggested [3]. Vasculitis, endothelial dysfunction, and hyperactivation of complement and coagulation factors are the proposed mechanisms. The disease typically starts between the third and the fifth decade involving the skin with pathognomonic papules surrounded by a telangiectasic rim. In almost 50% of cases, the vasculopathy involves other tissues and organs, such as the gut, lungs, and central nervous system (CNS) with possible fatal complications, hence the definition of malignant atrophic papulosis. We hereby describe a peculiar case of KDD with neurological debut, rapid fatal outcome, and unusual neuropathological findings.

A 44-year-old male affected by diabetes mellitus and HCV-related chronic hepatitis sub-acutely manifested right-sided weakness with...

Notes

Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.

Ethical statement

All procedures performed in this study were in accordance with the ethical standards of the institutional and national research committee and with the 1964 Helsinki declaration.

References

  1. 1.
    Degos R, Delort J, Tricot R (1942) Dermatite papulosquameuse atrophiante. Bull Soc Fr Derm Syph 49:148–150Google Scholar
  2. 2.
    Kohlmeier W (1941) Multiple Hautnekrosen bei Thrombangitis Obliterans. Dermatol Syph (Wien) 181:783–792.  https://doi.org/10.1007/BF01828450 CrossRefGoogle Scholar
  3. 3.
    Conforti R, Cirillo M, Saturnino PP, Gallo A, Sacco R, Negro A, Paccone A, Caiazzo G, Bisecco A, Bonavita S, Cirillo S (2014) Dilated Virchow-Robin spaces and multiple sclerosis: 3T magnetic resonance study. Radiol Med 119:408–414.  https://doi.org/10.1007/s11547-013-0357-9 CrossRefGoogle Scholar
  4. 4.
    Theodoridis A, Makrantonaki E, Zouboulis CC (2013) Malignant atrophic papulosis (Köhlmeier-Degos disease) - a review. Orphanet J Rare Dis 8:10.  https://doi.org/10.1186/1750-1172-8-10 CrossRefGoogle Scholar
  5. 5.
    Ye L, Lekgabe E, Tsui A, Gaillard F (2018) The evolution of cerebrovascular changes in Köhlmeier-Degos disease: an 11-year follow-up case report. J Clin Neurosci 48:114–117.  https://doi.org/10.1016/j.jocn.2017.11.006 CrossRefGoogle Scholar
  6. 6.
    High WA, Aranda J, Patel SB, Cockerell CJ, Costner MI (2004) Is Degos’ disease a clinical and histological end point rather than a specific disease? J Am Acad Dermatol 50:895–899.  https://doi.org/10.1016/j.jaad.2003.11.063 CrossRefGoogle Scholar
  7. 7.
    Gmuca S, Boos MD, Treece A, Narula S, Billinghurst L, Bhatti T, Laje P, Perman MJ, Vossough A, Harding B, Burnham J, Banwell B (2016) Degos disease mimicking primary vasculitis of the CNS. Neurol Neuroimmunol Neuroinflamm 3:e206.  https://doi.org/10.1212/NXI.0000000000000206 CrossRefGoogle Scholar

Copyright information

© Fondazione Società Italiana di Neurologia 2019

Authors and Affiliations

  1. 1.Department of Advanced Medical and Surgical SciencesUniversity of Campania “Luigi Vanvitelli”CasertaItaly
  2. 2.Department of Advanced Biomedical SciencesUniversity of Naples “Federico II”NaplesItaly
  3. 3.Department of Precision MedicineUniversity of Campania “Luigi Vanvitelli”CasertaItaly
  4. 4.Department of Clinical Medicine and SurgeryUniversity of Naples “Federico II”NaplesItaly

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