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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: 31 relevant articles (0 outcomes, 4 trials/studies)

Disease Context: Research Results

Related Diseases

1. Hyperplasia
2. Pulmonary Fibrosis (Hamman Rich Syndrome)
3. Hypertrophy
4. Pneumonia (Pneumonitis)
5. Lipidoses

Experts

1. Devi, Pratheeba N: 1 article (08/2015)
2. Mukherjee, Bipasha: 1 article (08/2015)
3. Basha, Mohamed Ahmed: 1 article (03/2014)
4. Houla, Nanees Shawky: 1 article (03/2014)
5. Samaka, Rehab Monir: 1 article (03/2014)
6. Bakry, Ola Ahmed: 1 article (03/2014)
7. Knudsen, Lars: 1 article (12/2013)
8. Boxler, Laura: 1 article (12/2013)
9. Clark, Howard W: 1 article (12/2013)
10. Mackay, Rose-Marie: 1 article (12/2013)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Lipoid Proteinosis of Urbach and Wiethe:
1. Silicon Dioxide (Sand)FDA LinkGeneric
2. Pulmonary Surfactant-Associated Protein D (Surfactant Protein D)IBA
3. RetinoidsIBA
4. Periodic AcidIBA
5. QuartzIBA
6. 5-HT2A Serotonin Receptor (5 HT2A Receptor)IBA
7. Interleukin-13IBA
8. Extracellular Matrix ProteinsIBA
9. Sphingomyelin Phosphodiesterase (Sphingomyelinase)IBA
10. PhospholipidsIBA

Therapies and Procedures

1. Dermabrasion
2. Gas Lasers
3. Homologous Transplantation (Allograft)