HOMEPRODUCTSCOMPANYCONTACTFAQResearchDictionaryPharmaSign Up FREE or Login

Pyogenic arthritis, pyoderma gangrenosum, and acne

A hereditary autosomal dominant disorder characterized by pyogenic (infectious) arthritis, pyoderma gangrenosum, and severe cystic acne. Onset is in childhood; pyoderma gangrenosum; and severe cystic acne occur in adolescence and beyond. Other less common features may include adult-onset DIABETES MELLITUS, TYPE I; PROTEINURIA, ABSCESS formation at the site of parenteral injections, and cytopenias attributable to SULFONAMIDE medications. Mutations in the PSTPIP1 gene have been identified. OMIM: 604416
Also Known As:
Familial recurrent arthritis; PAPA syndrome; Pyogenic Sterile Arthritis, Pyoderma Gangrenosum, and Acne; Pyogenic arthritis, pyoderma gangrenosum, and severe cystic acne
Networked: 68 relevant articles (0 outcomes, 4 trials/studies)

Disease Context: Research Results

Related Diseases

1. Pyogenic arthritis, pyoderma gangrenosum, and acne
2. Familial Mediterranean Fever (Periodic Disease)
3. Cryopyrin-Associated Periodic Syndromes
4. Acquired Immunodeficiency Syndrome (AIDS)
5. Mevalonate Kinase Deficiency

Experts

1. Aksentijevich, Ivona: 8 articles (01/2022 - 01/2003)
2. Kastner, Daniel L: 8 articles (01/2022 - 01/2003)
3. Gattorno, Marco: 6 articles (01/2021 - 05/2013)
4. Chae, Jae Jin: 3 articles (01/2022 - 01/2003)
5. Stone, Deborah L: 3 articles (01/2022 - 12/2018)
6. Caorsi, Roberta: 3 articles (01/2021 - 11/2014)
7. Martini, Alberto: 3 articles (01/2016 - 05/2013)
8. Wise, Carol A: 3 articles (11/2010 - 04/2002)
9. Arostegui, Juan I: 2 articles (01/2022 - 11/2015)
10. Ombrello, Amanda: 2 articles (01/2022 - 11/2015)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Pyogenic arthritis, pyoderma gangrenosum, and acne:
1. Interleukin-18 (Interleukin 18)IBA
2. Proteins (Proteins, Gene)FDA Link
3. Proline (L-Proline)FDA Link
4. Phosphoprotein Phosphatases (Phosphatases, Protein)IBA
5. Interleukin-1 (Interleukin 1)IBA
6. Tumor Necrosis Factor Receptors (Tumor Necrosis Factor Receptor)IBA
03/01/2011 - "To name some of them like erysipelas-like erythema in FMF; urticaria-like rashes in tumor necrosis factor receptor 1- or cryopyrin-associated periodic syndromes (TRAPS, CAPS), hyperimmunoglobulin D syndrome (HIDS) or Schnitzler syndrome; pyoderma gangrenosum and acne in PAPA syndrome; or behçetoid aphthous ulcerations in HIDS and PFAPA syndrome. "
01/01/2022 - "Objective: In this study, we aimed to evaluate other interleukin-1b-mediated monogenic autoinflam- matory diseases (AIDs) (tumor necrosis factor receptor-1-associated periodic syndrome, hyperimmuno- globulin D syndrome, cryopyrin-associated periodic syndrome (CAPS), pyogenic arthritis, pyoderma gangrenosum, and acne syndrome) by the next-generation sequencing method (NGS) in cases with clinical Familial Mediterranean Fever symptoms, and no variant detected in the MEFV gene. "
06/01/2009 - "These include the crypyrinopathies, familial Mediterranean fever, TNF-receptor associated periodic fever syndrome (TRAPS), hyper-IgD and periodic syndrome (HIDS), pyogenic sterile arthritis, pyoderma gangrenosum and acne (PAPA) syndrome, NALP12-HPF, and the Blau syndrome. "
01/01/2020 - "Monogenic autoinflammatory syndromes are those with identified genetic mutations, such as familial Mediterranean fever, tumor necrosis factor receptor-associated periodic fever syndrome (TRAPS), mevalonate kinase deficiency or hyperimmunoglobulin D syndrome, cryopyrin-associated periodic fever syndromes (CAPS), pyogenic arthritis pyoderma gangrenosum and acne (PAPA) syndrome, interleukin-10 and interleukin-10 receptor deficiencies, adenosine deaminase 2 deficiency and pediatric sarcoidosis. "
7. GlobulinsIBA
8. human PSTPIP1 proteinIBA
9. PyrinIBA
10. Interleukin-1 Receptors (Interleukin 1 Receptor)IBA
01/01/2018 - "Similar IL-1β-mediated autoinflammatory pathomechanisms also occur in deficiency of IL-1 receptor antagonist (DIRA) and deficiency of IL-36 receptor antagonist (DITRA), whose cutaneous appearance is characterised by pustular lesions, as well as in pyogenic arthritis, pyoderma gangrenosum and acne (PAPA) syndrome. "
01/01/2011 - "Genetic disorder of inflammasome-IL-1 system cause autoinflammatory diseases such as cryopyrin-associated autoinflammatory disease, familial Mediterranean fever, deficiency of IL-1 receptor antagonist, and PAPA syndrome. "
01/01/2018 - "There are also case reports revealing vasculitic features in Cryopyrin-Associated Periodic Fever Syndrome (CAPS), Tumor Necrosis Factor Receptor-Associated Periodic Syndrome (TRAPS), Mevalonate Kinase Deficiency (MKD), also known as Hyper IgD syndrome (HIDS), Deficiency of IL-1 Receptor Antagonist (DIRA) and Pyogenic Arthritis, Pyoderma gangrenosum, and Acne (PAPA) patients. "
09/01/2016 - "Skin symptoms include the main features of monogenic inflammasomopathies, such as Cryopyrin-Associated Periodic Syndromes (CAPS), Familial Mediterranean Fever (FMF), Schnitzler Syndrome, Hyper-IgD Syndrome (HIDS), PAPA Syndrome, and Deficiency of IL-1 Receptor Antagonist (DIRA). "
01/01/2013 - "To date, there are twelve known MAISs: familial Mediterranean fever, tumor necrosis factor receptor-associated periodic syndrome, familial cold urticaria syndrome, Muckle-Wells syndrome, CINCA syndrome, mevalonate kinase deficiency, NLRP12-associated autoinflammatory disorder, Blau syndrome, early-onset sarcoidosis, PAPA syndrome, Majeed syndrome, and deficiency of the interleukin-1 receptor antagonist. "

Therapies and Procedures

1. Therapeutics
2. Ligation
3. Biological Therapy
4. General Anesthesia
5. Treatment Delay