Related Experiment Video
Updated: Aug 1, 2026
07:47
Longitudinal In Vivo Imaging of the Cerebrovasculature: Relevance to CNS Diseases
Published on: December 6, 2016
Kohlmeier-Degos disease: a multisystem vasculopathy with progressive cerebral infarction
J N Burrow1, P C Blumbergs, P V Iyer
1Royal Adelaide Hospital, SA.
Australian and New Zealand Journal of Medicine
|February 1, 1991
Summary
Kohlmeier-Degos disease, a rare vasculopathy, presents with skin lesions and often involves the brain and gut, leading to a fatal outcome. This case highlights the disease's progressive nature and lack of effective treatments.
Area of Science:
- Vascular Medicine
- Dermatology
- Neurology
Background:
- Kohlmeier-Degos disease (Malignant Atrophic Papulosis) is a rare, fatal vasculopathy.
- It is characterized by distinctive skin lesions and frequent gastrointestinal and neurological complications.
Observation:
- A case report details a 22-year-old male with progressive cerebral infarction.
- The patient developed characteristic skin lesions, followed by neurological decline over nine months.
Findings:
- Autopsy revealed extensive small vessel occlusive vasculopathy affecting the skin, brain, and bowel.
- Treatments including anti-platelet agents and plasma exchange were ineffective.
Implications:
- The etiology of Kohlmeier-Degos disease remains unknown.
- Diagnosis relies on clinico-pathological findings.
- Increased awareness is crucial for differential diagnosis in young adults with cerebrovascular-arteritic syndromes.
More Related Videos
10:50Visualizing Impairment of the Endothelial and Glial Barriers of the Neurovascular Unit during Experimental Autoimmune Encephalomyelitis In Vivo
Published on: March 26, 2019
04:29A Visual Approach for Inducing Dolichoectasia in Mice to Model Large Vessel-Mediated Cerebrovascular Dysfunction
Published on: May 17, 2024
Related Concept Videos
01:15Ischemic Stroke ll: Pathophysiology
An ischemic stroke occurs when a cerebral blood vessel becomes obstructed, most often by a thrombus or embolus, interrupting the delivery of oxygen and glucose to brain tissue. Because neurons rely on continuous aerobic metabolism, energy failure begins within minutes of reduced perfusion. The region receiving the least blood flow becomes the infarct core, an area of irreversible cellular death. Surrounding this core lies the penumbra, a zone of hypoperfused but still viable tissue that is...
01:29Hemorrhagic Stroke ll: Pathophysiology
A hemorrhagic stroke develops when a cerebral blood vessel ruptures, allowing blood to escape into the surrounding brain tissue, as in intracerebral hemorrhage (ICH), or into the subarachnoid space, as in subarachnoid hemorrhage (SAH). Because the skull is a rigid compartment, the sudden presence of extravascular blood rapidly increases intracranial pressure and compresses adjacent neural structures, leading to immediate tissue injury and impaired cerebral perfusion.Mass Effect and Primary...
01:29Increased Intracranial Pressure ll: Pathophysiology
Increased intracranial pressure (ICP) refers to a potentially life-threatening rise in pressure inside the skull. This usually happens when there is a major change in the volume of brain tissue, blood, or cerebrospinal fluid (CSF) — the three components inside the skull. According to the Monro-Kellie doctrine, if the volume of one component increases, the volumes of the other components must decrease to maintain normal pressure. If this does not happen, ICP rises.The process often begins with...
01:22Cerebral Edema ll: Pathophysiology
Vasogenic edema is a major form of cerebral edema characterized by abnormal accumulation of fluid in the brain’s extracellular space due to disruption of the blood–brain barrier (BBB). The BBB is a specialized structure composed of endothelial cells connected by tight junctions, supported by astrocytic endfeet and a basement membrane. Under normal conditions, it tightly regulates the movement of ions, proteins, and solutes between the bloodstream and brain parenchyma. When this barrier loses...
01:23Alzheimer Disease ll: Pathophysiology
Alzheimer disease involves structural changes in the brain that begin long before symptoms appear. The most distinctive features are extracellular neuritic plaques and intracellular neurofibrillary tangles.Neuritic plaques form in the cerebral cortex and around blood vessels. These plaques contain a dense core of beta-amyloid (Aβ)—a toxic protein fragment that clumps outside neurons. The core is surrounded by damaged neuronal extensions, as well as reactive astrocytes and microglia. Abnormal...
01:22Dementia l: Introduction
Dementia is an acquired, progressive syndrome characterized by a decline in multiple cognitive domains severe enough to impair daily functioning and reduce independence. Although memory loss is a central feature, the diagnosis requires additional deficits involving language, executive function, visuospatial skills, judgment, calculation, or abstract reasoning. These cognitive impairments reflect underlying neurodegenerative or vascular processes that gradually disrupt neuronal networks...