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Anterior Segment-Optical Coherence Tomography features in Blau syndrome
- Source :
- Photodiagnosis and Photodynamic Therapy. 34:102278
- Publication Year :
- 2021
- Publisher :
- Elsevier BV, 2021.
-
Abstract
- Blau syndrome (BS) is a rare granulomatous auto-inflammatory disease, characterized by the classic clinical triad of joints, skin and ocular involvements. Ocular manifestation usually consists in a bilateral insidious chronic anterior uveitis with a potential evolution to panuveitis. We describe the case of two siblings, an 8-years old female and a 5-years old male, with a diagnosis of BS, evaluated by Anterior Segment-Optical Coherence Tomography (AS-OCT). In the female patient, slit-lamp examination revealed bilateral anterior granulomatous uveitis and inflammatory sequelae. AS-OCT revealed high intensity reflective layers in the anterior cornea, hyperreflective dots both in the aqueous humor and in the posterior corneal surface. In the male, no signs of inflammation were detected both on slit-lamp examination and AS-OCT scans. AS-OCT is a valuable, non-invasive tool that could improve the diagnosis of ocular involvement, better characterize and follow-up corneal alterations and anterior segment features in pediatric patients with BS.
- Subjects :
- Male
medicine.medical_specialty
Sarcoidosis
genetic structures
030303 biophysics
Biophysics
Aqueous humor
Dermatology
Uveitis
030207 dermatology & venereal diseases
03 medical and health sciences
0302 clinical medicine
Optical coherence tomography
Anterior Eye Segment
Ophthalmology
Cornea
Female patient
Humans
Medicine
Pharmacology (medical)
Anterior cornea
Child
Blau syndrome
0303 health sciences
Photosensitizing Agents
Synovitis
medicine.diagnostic_test
business.industry
Arthritis
Panuveitis
medicine.disease
Anterior Segment-Optical Coherence Tomography
eye diseases
medicine.anatomical_structure
Photochemotherapy
Oncology
Child, Preschool
Chronic anterior uveitis
Female
sense organs
business
Tomography, Optical Coherence
Subjects
Details
- ISSN :
- 15721000
- Volume :
- 34
- Database :
- OpenAIRE
- Journal :
- Photodiagnosis and Photodynamic Therapy
- Accession number :
- edsair.doi.dedup.....1e8de36c5ff4a1c8e338aef6b57116f7