Dramatic neurological debut in a case of Köhlmeier-Degos disease
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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 . 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.
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Conflict of interest
The authors declare that they have no conflict of interest.
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.
- 1.Degos R, Delort J, Tricot R (1942) Dermatite papulosquameuse atrophiante. Bull Soc Fr Derm Syph 49:148–150Google Scholar