Abstract Details

Title Neurologic manifestations of Degos disease

Topic Autoimmune Neurology

Presentation(s) P17 - Poster Session 17 (11:45 AM-12:45 PM)

Poster/Presentation
Number 9-003

Objective To describe neurologic manifestations of Degos disease (malignant atrophic papulosis) involving central and peripheral nervous system

Background Degos disease is a rare occlusive vasculopathy most commonly involving skin, gastrointestinal tract and brain. Rare cases of neuropathy have been reported as well. Exact pathophysiology of Degos disease is still not well understood, but it may involve vascular depositon of C5b-9 complement components.

Design/Methods Case report

Results A 53-year-old man developed asymmetric painless leg weakness and numbness in summer of 2018. This was preceded by the onset of diffuse papular rash 4 years earlier. He has also developed episodic bowel incontinence and asymmetric sensorineural hearing loss. Electrodiagnostic testing showed chronic L4-5 radiculopathy and mild neuropathy. CSF studies revealed elevated protein content at 234 mg/dl, glucose of 49 mg/dl, with 3 white blood cells and negative cytology. He later developed horizontal diplopia and contrast-enhanced brain MRI showed 15-20 enhancing leptomeningeal foci. Repeat electrodiagnostic testing at 2.5 years after the onset showed radiculoplexus neuropathy, mildly improved when compared to earlier studies. There was also mild clinical improvement after treatment with prednisone up to 80 mg/day and azathioprine up to 150 mg/day. Extensive workup was otherwise non-diagnostic including laboratory testing , lumbar puncture, nerve and muscle biopsy, MRIs of total spine and brain and PET/CT. Repeat MRI of pelvis showed subtle enhancement of cauda equina. However, repeat skin biopsy showed evidence of Degos disease.

Conclusions Degos is a rare and often fatal multisystemic disease which may involve both central and peripheral nervous system. The hallmark of the disease is papular skin rash lending its name to malignant atrophic papulosis. Patients may also have abdominal pain and diarrhea as a sign of gastrointestinal involvement. Immunosuppressive therapy provides only limited benefits in Degos disease, and the extensive complement deposition in the skin and other tissues suggests potential use of complement inhibitors.

Authors/Disclosures
Sasha Zivkovic, MD, FAAN (Yale University) PRESENTER	Dr. Zivkovic has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Astra Zeneca. Dr. Zivkovic has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Pfizer. Dr. Zivkovic has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Applied Therapeutics. Dr. Zivkovic has received personal compensation in the range of $500-$4,999 for serving as a Consultant for WCG Clinical services.
Tawfiq Al-Lahham, MD (UPMC)	Dr. Al-Lahham has nothing to disclose.
David Lacomis, MD, FAAN (UPMC Neurology)	Dr. Lacomis has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Cytokinetics. The institution of Dr. Lacomis has received research support from Mitsubishi Tanabe. The institution of Dr. Lacomis has received research support from NIH. Dr. Lacomis has received publishing royalties from a publication relating to health care.

��ɫ��

��ɫ��