Related Experiment Videos
Scleromyxedema with corneal deposits
H M Goldin1, A J Axelrod, D M Bronson
1Department of Medicine, Cook County Hospital, Chicago, IL 60612.
Ophthalmology
|October 1, 1987
Summary
Scleromyxedema, a rare skin disease, can affect the cornea, causing hyaluronic acid and amyloid P deposits. This is the first documented case of corneal involvement in scleromyxedema.
Area of Science:
- Ophthalmology
- Dermatology
- Pathology
Background:
- Scleromyxedema (Arndt-Gottron syndrome) is a rare mucopolysaccharidosis characterized by dermal deposition of hyaluronic acid.
- The condition typically presents with skin thickening, papules, and sometimes systemic involvement.
Observation:
- This report details a unique case of scleromyxedema with concurrent corneal deposits.
- Ocular examination revealed significant abnormalities within the cornea.
Findings:
- Corneal biopsy confirmed the presence of hyaluronic acid deposition within the corneal stroma.
- Amyloid P component was identified in Bowman's membrane, a novel finding independent of primary corneal amyloidosis.
Implications:
- This case expands the known clinical manifestations of scleromyxedema to include ocular involvement.
- The presence of amyloid P in the cornea, unrelated to amyloid deposits, suggests a potential new pathway for corneal pathology in certain systemic diseases.