Lipoid Proteinosis of Urbach and Wiethe (Lipoidproteinosis)

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22 relevant articles (0 outcomes, 3 trials/studies) found for this Disease

Description: 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; Urbach-Wiethe Lipoid Proteinosis; Urbach-Wiethe Syndrome; Lipoid Proteinosis, Urbach-Wiethe; Urbach Wiethe Disease; Urbach Wiethe Lipoid Proteinosis; Lipoproteinosis; Urbach-Wiethe Disease

Disease Context: Research Results

Respiratory Tract Diseases: 373

Respiration Disorders: 11

Hoarseness: 267

Lipoid Proteinosis of Urbach and Wiethe: 22

Congenital, Hereditary, and Neonatal Diseases and Abnormalities: 422

Inborn Genetic Diseases: 549

Genetic Skin Diseases: 10

Lipoid Proteinosis of Urbach and Wiethe: 22

Skin and Connective Tissue Diseases

Skin Diseases: 5944

Lipoid Proteinosis of Urbach and Wiethe: 22

Related Diseases

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

Experts

1.	Schuchman, Edward H: 1 article (03/2003)
2.	Ikegami, Machiko: 1 article (03/2003)
3.	Dhami, Rajwinder: 1 article (03/2003)
4.	Ma, Bing: 1 article (07/2002)
5.	Gobran, Laurice I: 1 article (07/2002)
6.	Chupp, Geoff: 1 article (07/2002)
7.	Homer, Robert J: 1 article (07/2002)
8.	Rooney, Seamus A: 1 article (07/2002)
9.	Zheng, Tao: 1 article (07/2002)
10.	Chen, Quingshen: 1 article (07/2002)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Lipoid Proteinosis of Urbach and Wiethe:

1.	Silicon Dioxide (Sand)IBA 02/01/1977 - "Light microscopy of biopsy and necropsy material showed small silicotic nodules, silica particles, and alveolar lipoproteinosis, and ultrastructural studies were performed to define changes in alveolar epithelium and macrophages" 10/01/1991 - "Increased accumulation in silica-induced pulmonary lipoproteinosis." 01/01/1983 - "Even 1 year after instillation of silica areas of granulomas, silicotic nodules and alveolar lipoproteinosis may be observed in most of the lungs studied; ie, these responses are not mutually exclusive." 01/01/1983 - "Both strains of rats develop silica-containing granulomas, mature silicotic nodules, and areas of alveolar lipoproteinosis associated with interstitial pneumonitis" 05/01/1982 - "Both strains of rats developed silica-containing granulomas, which ultimately developed into silicotic nodules, as well as areas of alveolar lipoproteinosis associated with interstitial pneumonitis." Order ALL the reference details at left...
2.	Pulmonary Surfactant-Associated Protein D (Surfactant Protein D)IBA 10/01/1991 - "These studies indicate that the extracellular accumulation of SP-D is markedly increased in silica-induced lipoproteinosis, and that SP-D is associated with amorphous components identified by electron microscopy." 01/01/1993 - "SP-D was initially characterized as a biosynthetic product of freshly isolated rat type II cells and first purified in chemical amounts from bronchoalveolar lavage of rats with silica-induced alveolar lipoproteinosis" Order ALL the reference details at left...
3.	QuartzIBA 06/01/1980 - "Microscopically, all exposed groups developed granulomatous pneumonia and alveolar lipoproteinosis; pulmonary fibrosis was most severe in the quartz-exposed groups and progressed with time in these groups" 08/01/2002 - "Histopathological investigations after four weeks and three months revealed that the used PVNO sample was active in the quartz and amorphous SiO2 groups and markedly reduced the incidences or severity of several pulmonary changes such as macrophage accumulation, inflammatory cell infiltration, interstitial fibrosis, bronchiolo-alveolar hyperplasia, alveolar lipoproteinosis and amorphous SiO2 -induced granulomatous alveolitis/interstitial fibrotic granulomas" 01/01/1986 - "These results indicate that pyrite, like only a few other types of dust that are different from quartz, can evoke the development of pulmonary alveolar lipoproteinosis in rats." 01/01/1986 - "Moreover, both pyrite-treated rats and quartz-treated rats developed pulmonary alveolar lipoproteinosis; the lesions were much less prominent and severe in animals injected with pyrite than with quartz" Order ALL the reference details at left...
4.	Periodic AcidIBA 08/01/2008 - "The lungs contained intra-alveolar accumulation of periodic acid-Schiff positive material, foamy macrophages, and hyperplasia of the alveolar cells, consistent with pulmonary alveolar lipoproteinosis" 01/01/2001 - "Lipoid proteinosis (Urbach-Wiethe disease) is a rare autosomal recessive disorder associated with deposition of periodic acid-Schiff (PAS)-positive hyaline material in various tissues including skin, mucous membranes, and internal organs" Order ALL the reference details at left...
5.	Interleukin-13IBA 07/01/2002 - "Pulmonary type II cell hypertrophy and pulmonary lipoproteinosis are features of chronic IL-13 exposure." Order ALL the reference details at left...
6.	Sphingomyelin Phosphodiesterase (Sphingomyelinase)IBA 03/01/2003 - "Alveolar lipoproteinosis in an acid sphingomyelinase-deficient mouse model of Niemann-Pick disease." Order ALL the reference details at left...
7.	PhospholipidsIBA 08/01/2003 - "The alveolar lipoproteinosis (ALP) is a rare pulmonary disease, characterized by an excess of phospholipids in the distal airway, and the most symptom of which is dyspnea" Order ALL the reference details at left...
8.	p-Aminosalicylic Acid (PAS)FDA Link 01/01/2001 - "Lipoid proteinosis (Urbach-Wiethe disease) is a rare autosomal recessive disorder associated with deposition of periodic acid-Schiff (PAS)-positive hyaline material in various tissues including skin, mucous membranes, and internal organs" Order ALL the reference details at left...
9.	Lipoproteins (Lipoprotein)IBA 02/01/1988 - "We confirmed the presence of large multilamellar aggregates of lipoprotein by ultrastructural examination in patients with both interstitial lung disease and idiopathic alveolar lipoproteinosis" 02/01/1988 - "Lipoprotein macroaggregates were present in cytocentrifuge preparations of bronchoalveolar lavage fluid from four patients with diffuse lung diseases other than idiopathic alveolar lipoproteinosis" 02/01/1988 - "Lipoprotein macroaggregates in bronchoalveolar lavage fluid from patients with diffuse interstitial lung disease: comparison with idiopathic alveolar lipoproteinosis." Order ALL the reference details at left...
10.	Dimethyl Sulfoxide (DMSO)FDA LinkGeneric 07/01/1997 - "Oral DMSO therapy in 3 patients with lipoidproteinosis" Order ALL the reference details at left...

Therapies and Procedures

1.	Gas Lasers 03/01/1997 - "Carbon dioxide laser treatment for lipoid proteinosis (Urbach-Wiethe syndrome) involving the eyelids." Order ALL the reference details at left...
2.	Homologous Transplantation (Allograft) 12/01/1997 - "Alveolar lipoproteinosis in lung allograft recipients." Order ALL the reference details at left...

Best Treatments:
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