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Lipoid Proteinosis of Urbach and Wiethe (Lipoidproteinosis)

An autosomal recessive disorder characterized by glassy degenerative thickening (hyalinosis) of SKIN; MUCOSA; and certain VISCERA. This disorder is caused by mutation in the extracellular matrix protein 1 gene (ECM1). Clinical features include hoarseness and skin eruption due to widespread deposition of HYALIN.
Also Known As:
Lipoidproteinosis; Hyalinosis Cutis et Mucosae; Urbach-Wiethe Lipoid Proteinosis; Urbach-Wiethe Syndrome; Lipoid Proteinosis, Urbach-Wiethe; Urbach Wiethe Disease; Urbach Wiethe Lipoid Proteinosis; Lipoproteinosis; Urbach-Wiethe Disease
Networked: 46 relevant articles (0 outcomes, 6 trials/studies)

Disease Context: Research Results

Related Diseases

1. Hyperplasia
2. Pulmonary Fibrosis (Fibrosing Alveolitis)
3. Hypertrophy
4. Pneumonia (Pneumonitis)
5. Lipidoses

Experts

1. Berthoux, Charlotte: 1 article (02/2021)
2. Gauthier, Clarisse: 1 article (02/2021)
3. Lagier, Jean-Christophe: 1 article (02/2021)
4. Mailhe, Morgane: 1 article (02/2021)
5. Melenotte, Cléa: 1 article (02/2021)
6. Mège, Jean-Louis: 1 article (02/2021)
7. Vély, Frédéric: 1 article (02/2021)
8. Fourie, J: 1 article (06/2016)
9. Koen, N: 1 article (06/2016)
10. Morgan, B: 1 article (06/2016)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Lipoid Proteinosis of Urbach and Wiethe:
1. Silicon Dioxide (Silica)FDA LinkGeneric
2. Pulmonary Surfactant-Associated Protein D (Surfactant Protein D)IBA
3. Surface-Active Agents (Surfactants)IBA
4. Granulocyte-Macrophage Colony-Stimulating Factor (GM-CSF)IBA
5. RetinoidsIBA
6. AutoantibodiesIBA
7. Extracellular Matrix ProteinsIBA
8. Periodic AcidIBA
9. QuartzIBA
10. Phospholipids (Phosphatides)FDA LinkGeneric

Therapies and Procedures

1. Dermabrasion
2. Therapeutics
3. Gas Lasers