Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Case Reports
. 2022 Jan 1;79(1):84-85.
doi: 10.1001/jamaneurol.2021.4194.

Acute Myelopathy in a Man With Cutaneous Papules

Shermyn Neo  1 , Chee Seng Sim  2 , Chia Chun Ang  3
Affiliations
Case Reports

Acute Myelopathy in a Man With Cutaneous Papules

Shermyn Neo et al. JAMA Neurol. .

Erratum in

  • Error in Figure.
    [No authors listed] [No authors listed] JAMA Neurol. 2022 Mar 1;79(3):313. doi: 10.1001/jamaneurol.2021.5455. JAMA Neurol. 2022. PMID: 35099512 Free PMC article. No abstract available.

Abstract

A 46-year-old man presented with acute-onset weakness of his bilateral lower limbs for 3 days, accompanied by ascending numbness to just below his nipples. Neurological examination revealed weakness in bilateral lower limbs, extensor plantar responses, and a sensory level at T6. What is your diagnosis?

PubMed Disclaimer

Conflict of interest statement

Conflict of Interest Disclosures: None reported.

Figures

Figure 1.
Figure 1.
T2-weighted magnetic resonance imaging (MRI) of the thoracic spine showing an area of hyperintensity in the left posterolateral cord at T7 (A) (arrowhead). Multiple papules were seen on the patient’s abdomen. These consisted of hypopigmented papules with central hyperpigmented umbilication and larger papules with typical central atrophic porcelain white scarring (B).
Figure 2.
Figure 2.
Biopsy from the central atrophic scar of a skin papule, showing thinned epidermis with mild interface vacuolar dermatitis, papillary dermal fibrosis, and increased dermal mucin deposition. Inset shows a vascular deposit of C5b-C9 membranolytic attack complex on immunostaining (hematoxylin-eosin; original magnification x400).
See this image and copyright information in PMC

References

    1. Magro CM, Poe JC, Kim C, et al. . Degos disease: a C5b-9/interferon-α-mediated endotheliopathy syndrome. Am J Clin Pathol. 2011;135(4):599-610. doi:10.1309/AJCP66QIMFARLZKI - DOI - PubMed
    1. Subbiah P, Wijdicks E, Muenter M, Carter J, Connolly S. Skin lesion with a fatal neurologic outcome (Degos’ disease). Neurology. 1996;46(3):636-640. doi:10.1212/WNL.46.3.636 - DOI - PubMed
    1. Matsuura F, Makino K, Fukushima T, et al. . Optic nerve and spinal cord manifestations of malignant atrophic papulosis (Degos disease). J Neurol Neurosurg Psychiatry. 2006;77(2):260-262. doi:10.1136/jnnp.2005.066134 - DOI - PMC - PubMed
    1. Label LS, Tandan R. Myelomalacia and hypoglycorrhachia in malignant atrophic papulosis. Neurology. 1983;33(7):936-939. doi:10.1212/wnl.33.7.936 - DOI - PubMed
    1. Theodoridis A, Konstantinidou A, Makrantonaki E, Zouboulis CC. Malignant and benign forms of atrophic papulosis (Köhlmeier-Degos disease). Br J of Dermatol. 2013;170(1):110-115. doi:10.1111/bjd.12642 - DOI - PubMed

Publication types