Primary Cutaneous Amyloidosis

An autosomal dominant type of familial amyloidosis that is characterized by PRURITIS and skin scratching and histologically by the finding of deposits of AMYLOID staining on keratinous debris in the papillary DERMIS. Mutations in the OSMR gene have been identified. OMIM: 105250

Also Known As:

Networked: 100 relevant articles (3 outcomes, 7 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Pruritus (Itching)
2.	Amyloidosis
3.	Amyloid Plaque
4.	Skin Diseases (Skin Disease)
5.	Rare Diseases (Rare Disease)

Experts

1.	Chander, Ram: 2 articles (01/2022 - 08/2017)
2.	Yang, Bin: 2 articles (01/2022 - 01/2022)
3.	Manjunath, G V: 2 articles (04/2021 - 04/2012)
4.	Esmat, Samia M: 2 articles (01/2021 - 07/2015)
5.	Gawdat, Heba I: 2 articles (01/2021 - 07/2015)
6.	Tey, Hong Liang: 2 articles (10/2018 - 01/2016)
7.	Fujimoto, Norihiro: 2 articles (10/2014 - 03/2002)
8.	Tajima, Shingo: 2 articles (10/2014 - 03/2002)
9.	Chang, Y-T: 2 articles (04/2014 - 12/2006)
10.	Lee, D-D: 2 articles (04/2014 - 12/2006)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Primary Cutaneous Amyloidosis:

1.	Amyloid (Amyloid Fibrils)IBA 08/01/2023 - "Previous studies on primary cutaneous amyloidosis (PCA) have mainly focused on exploring genetic mutation and components of amyloid in patients with PCA. " 09/27/2021 - "Primary cutaneous amyloidosis (PCA) is characterized by the extracellular deposition of amyloid in the skin without systemic involvement. " 01/01/2021 - "Primary cutaneous amyloidosis (PCA) is a pruritic disease characterized by amyloid deposition in the skin. " 10/08/2019 - "Primary localized cutaneous amyloidosis (PLCA) occurs when amyloid is deposited only within the skin and there is no evidence of systemic involvement. " 05/01/2019 - "Primary localized cutaneous amyloidosis is a group of rare conditions where amyloid deposition is limited to the skin without systemic manifestations. "
2.	Keratins (Keratin)IBA 09/01/1980 - "Skin specimens obtained from two cases of primary localized cutaneous amyloidosis (PLCA) were subjected to study with an indirect immunofluorescence technique using anti-keratin antibody. " 02/01/1999 - "Amyloid deposits in primary cutaneous amyloidosis (PCA) may be initially derived from cytokeratin. " 10/01/1987 - "A monoclonal anti-keratin antibody reactive with amyloid deposit of primary cutaneous amyloidosis." 01/01/2022 - "LC-MS/MS and immuno-electron subtyping combined with genetics show that OSMR mutations cause amyloid deposition of keratins 5/14 in familial primary localized cutaneous amyloidosis." 01/01/1999 - "The results of histochemical reactions confirm the keratin-derived nature of amyloid in primary cutaneous amyloidosis."
3.	Dimethyl Sulfoxide (DMSO)FDA LinkGeneric 03/01/2002 - "To evaluate dimethylsulphoxide (DMSO) as a topical therapeutic agent for the treatment of two major variants of primary, localized, cutaneous amyloidosis -- macular amyloidosis (MA) and papular/lichen amyloidosis (LA) -- using a monocentric, open, prospective trial. " 02/01/1996 - "The skin lesions and amyloid deposition regressed with topical application of dimethyl sulfoxide in one patient, suggesting that DSP is one of the clinical phenotypes of primary localized cutaneous amyloidosis. "
4.	Apolipoproteins E (ApoE)IBA 09/01/1998 - "These results indicate that apoE is a component of primary localized cutaneous amyloidosis, and that it might play an important role in primary localized cutaneous amyloidosis." 09/01/1998 - "In this study, we investigated whether apoE is associated with amyloid deposits of primary localized cutaneous amyloidosis using immunohistochemistry, immunogold electron microscopy, and immunoblotting. " 09/01/2001 - "Apolipoprotein E (apoE) is present in a variety of biochemically different amyloid deposits, including Alzheimer's disease, systemic amyloidosis and primary cutaneous amyloidosis (PCA). "
5.	Peroxidase (Myeloperoxidase)IBA 10/01/1976 - "Primary cutaneous amyloidosis : clinical, histological, histochemical immunofluorescent and peroxidase studies of 134 cases in 2 years."
6.	Amyloidogenic ProteinsIBA 01/01/2022 - "Primary localized cutaneous amyloidosis (PLCA) is defined by the deposition of amyloid protein in the skin without systemic involvement. " 10/01/2017 - "Primary localized cutaneous amyloidosis (PLCA) is characterized by extracellular deposition of heterogenic amyloid proteins in the skin without systemic involvement. " 12/15/2020 - "Lichen amyloidosis is a subtype of primary localized cutaneous amyloidosis characterized by deposition of amyloid protein in the skin without visceral involvement. " 02/01/2016 - "Nodular primary localized cutaneous amyloidosis (PLCA) is a rare subtype of localized cutaneous amyloidosis in which amyloid protein is derived from immunoglobulin light chains. " 09/01/1990 - "This is the third case of nodular primary cutaneous amyloidosis which has been studied at the level of sequence analysis of purified amyloid fibril proteins, and the first case of nodular primary cutaneous amyloidosis in which a lambda III amyloid protein has been shown to be present by sequence analysis."
7.	Proteins (Proteins, Gene)FDA Link 05/01/2023 - "Primary localized cutaneous amyloidosis (PLCA) is characterized by extracellular deposition of pathological fibril aggregation of proteins in the skin without systemic involvement. " 07/01/2016 - "Primary cutaneous amyloidosis (PCA) is a condition characterized by tissue deposition of misfolded proteins. " 09/01/1990 - "An amyloid fibril protein (MA) was purified as a 17,000-dalton protein from a case of nodular primary cutaneous amyloidosis, and its partial amino acid sequence (22 residues from N-terminal) was determined. " 07/01/2009 - "Nodular amyloidosis is a primary cutaneous amyloidosis characterized by the deposition of amyloid L-type fibril proteins in the dermis. " 09/01/1990 - "This is the third case of nodular primary cutaneous amyloidosis which has been studied at the level of sequence analysis of purified amyloid fibril proteins, and the first case of nodular primary cutaneous amyloidosis in which a lambda III amyloid protein has been shown to be present by sequence analysis."
8.	Congo RedIBA 08/01/2006 - "A comparative study of two Congo red stains for the detection of primary cutaneous amyloidosis." 04/01/2021 - "There was 100% sensitivity with Congo red and CK5/6. Thus, CK5/6 can be used as an adjunct tool to Congo red stain in the diagnosis of primary cutaneous amyloidosis." 02/01/2006 - "Because these materials were positive for both Congo red and Dylon, we diagnosed the lesion as primary cutaneous amyloidosis of the auricular concha. " 04/01/2012 - "Of the 45 cases of clinically suspected amyloidosis, 32 cases were proven histopathologically as primary cutaneous amyloidosis and confirmed by congo red stain under polarized light which showed apple green birefringence. "
9.	Oncostatin M Receptors (Oncostatin M Receptor)IBA 12/01/2013 - "Familial primary localized cutaneous amyloidosis with an oncostatin M receptor-β mutation, Pro694Leu." 01/01/2012 - "A new c.1845A→T of oncostatin M receptor-β mutation and slightly enhanced oncostatin M receptor-β expression in a Chinese family with primary localized cutaneous amyloidosis." 10/01/2009 - "Identification of an oncostatin M receptor mutation associated with familial primary cutaneous amyloidosis."
10.	AntibodiesIBA 05/01/1998 - "In nodular primary cutaneous amyloidosis, amyloid deposits were not labelled with any antikeratin antibodies. " 01/01/1984 - "Thirty-one cases of primary cutaneous amyloidosis were studied by the immunoperoxidase technique (PAP method) employing anti-keratin antibodies. " 05/01/1998 - "The expression of keratins was investigated immunohistochemically on formalin-fixed and snap-frozen primary cutaneous amyloidosis tissue with a panel of monospecific and polyspecific antikeratin antibodies, with recognized keratins K1, K5, K6, K7, K8, K10, K14, K16, K17, K18 and K19. " 10/01/1982 - "Primary localized cutaneous amyloidosis: dermal amyloid deposits do not bind antibodies to amyloid A protein, prealbumin or fibronectin."

Therapies and Procedures

1.	Gas Lasers 07/01/2015 - "Efficacy of different modes of fractional CO2 laser in the treatment of primary cutaneous amyloidosis: A randomized clinical trial." 06/01/1986 - "Nodular primary localized cutaneous amyloidosis: immunohistochemical evaluation and treatment with the carbon dioxide laser." 01/01/2021 - "Fractional Carbon Dioxide Laser is Effective in Amelioration of Pruritus in Primary Cutaneous Amyloidosis: A Clinical and Biochemical Study."
2.	Photochemotherapy (Photodynamic Therapy) 01/01/2019 - "Our case is the first report demonstrating the efficacy of daylight photodynamic therapy in primary cutaneous amyloidosis. " 01/01/2019 - "Daylight photodynamic therapy may provide a convenient and cost-effective therapeutic option in primary cutaneous amyloidosis, and its efficacy should be further confirmed in prospective trials." 01/01/2019 - "Therefore, we speculate that type II photochemical reaction is responsible for the observed therapeutic activity of methyl aminolevulinate photodynamic therapy in our patient with primary cutaneous amyloidosis. "
3.	Radiotherapy 02/01/2016 - "Radiotherapy appears ineffective in treating this type of primary cutaneous amyloidosis, and surgical treatment, where possible, is a good option, especially with MMS, which allows both controlled excision and minimal margins." 01/01/2021 - "Majority of these lesions do not require any treatment but surgical excision, shave excision, laser therapy, and radiotherapy can be considered for symptomatic nodular localized primary cutaneous amyloidosis (NLPCA). "
4.	Lasers (Laser) 03/01/2022 - "The therapeutic role of lasers in primary localized cutaneous amyloidosis: a systematic review." 07/01/2016 - "Treatment of primary cutaneous amyloidosis with laser: a review of the literature."
5.	Solid-State Lasers 01/01/2021 - "Fractional Erbium-Doped Yttrium Aluminum Garnet Laser in the Treatment of Primary Cutaneous Amyloidosis."