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Updated: Jun 3, 2026

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Published on: October 13, 2023
Skin involvement in Susac's syndrome
Guillaume Turc1, Dominique Monnet, Nicolas Dupin
1Pole Raymond Garcin, Department of Neurology, Hôpital Sainte-Anne, Paris and Paris Descartes University, Inserm UMR 894, France. g.turc@ch-sainte-anne.fr
Susac's syndrome (SS), a rare condition affecting brain, retina, and inner ear, can also involve the skin. This case highlights skin biopsy findings consistent with SS, suggesting it is a systemic disease.
Area of Science:
- Neurology
- Ophthalmology
- Dermatology
- Rheumatology
Background:
- Susac's syndrome (SS) is a rare autoimmune endotheliopathy affecting the brain, retina, and inner ear.
- It is characterized by microangiopathy of precapillary arterioles.
Observation:
- A 24-year-old male presented with encephalopathy, retinal artery occlusions, and hearing loss.
- Livedo racemosa preceded neurological and auditory symptoms by two weeks.
- Skin biopsies revealed arteriolar thrombi and inflammation, mirroring findings in SS.
Findings:
- Histological examination of skin lesions confirmed findings consistent with Susac's syndrome.
- The patient experienced complete recovery with corticosteroid treatment.
- Follow-up MRI revealed characteristic "holes" in the centro-callosal region.
Implications:
- This case demonstrates that Susac's syndrome can manifest with skin involvement.
- Skin examination should be considered in the diagnostic workup of suspected SS.
- Findings support SS as a systemic vasculopathy with potential multi-organ impact.
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