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Published on: September 19, 2019
Gronblad Strandberg syndrome with vertibrobasilar dolichoectasia
Resmy Susan Babu1, Indu K Nair, M K Suresh
1Medical College Hospital, Trivandrum.
Gronblad Strandberg syndrome, a genetic disorder, involves pseudoxanthoma elasticum affecting skin, eyes, and blood vessels. Early recognition of this condition can help minimize severe complications like hemorrhages and cardiovascular issues.
Area of Science:
- Genetics
- Dermatology
- Ophthalmology
- Cardiology
Background:
- Gronblad Strandberg syndrome is a rare genetic disorder characterized by pseudoxanthoma elasticum.
- This condition involves progressive calcification and fragmentation of elastic fibers.
- It affects multiple organ systems, including the skin, eyes, and cardiovascular system.
Observation:
- Cutaneous lesions of pseudoxanthoma elasticum often begin in childhood but may go unnoticed until adolescence.
- In some cases, skin manifestations can appear later in life.
- Ocular and vascular disturbances are key components of the syndrome.
Findings:
- The syndrome is defined by the triad of pseudoxanthoma elasticum, ocular involvement, and vascular disturbances.
- Elastic fiber degeneration leads to characteristic skin findings and potential retinal complications.
- Cardiovascular system involvement can manifest as progressive calcification.
Implications:
- Early diagnosis of Gronblad Strandberg syndrome is crucial for proactive management.
- Timely intervention can potentially reduce the risk of severe gastrointestinal and retinal hemorrhages.
- Monitoring and management of cardiovascular complications are essential for long-term patient outcomes.
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