08-30-2021-1941 - P2A2 syndrome is an acronym for pyogenic arthritis, pyoderma gangrenosum and acne. It is a rare genetic disorder characterised by its effects on skin and joints.[2][3] P2A2 v. Petersen-USA-et. al., etc. gangar fever pyo pyogene interleukin 1 interlukin tumor necrosis factor immunosuppression immunosuppressant genetic blood hematology osteology rheumatology radiology oncology immunology genology myology neurology cardiology pathology histology aphthous Hyper-IgD syndrome Hyperimmunoglobulin D syndrome Cryopyrin-associated periodic syndrome D Deficiency of the interleukin-1–receptor antagonist F Familial cold autoinflammatory syndrome Autoimmune disease Axial spondyloarthritis Autoinflammatory diseases Autoinflammatory syndromes osteitis inflammation chronic inflammatory disease chronic inflammation layng down

 P2A2 syndrome is an acronym for pyogenic arthritis, pyoderma gangrenosum and acne. It is a rare genetic disorder characterised by its effects on skin and joints.^[2][3]

PAPA syndrome is inherited in an autosomal dominant fashion, which means that if one parent is affected, there is a 100% chance that a child will inherit the disease from a homozygous affected parent and a 50% chance that a child will inherit the disease from an affected heterozygous parent.

The responsible gene has been identified on Chromosome 15.^[4][5][6] Two mutations have been found in a protein called CD2 binding protein 1(CD2BP1).^[7] This protein is part of an inflammatory pathway associated with other autoinflammatory diseases such as familial Mediterranean fever, Hyperimmunoglobulinemia D with recurrent fever, Muckle–Wells syndrome, neonatal onset multisystem inflammatory disease, and familial cold urticaria.^[8]

Acne treatment may require oral tetracycline antibiotics or isotretinoin. Treatments directed at tumor necrosis factor (TNF) (infliximab, etanercept) and interleukin-1 (anakinra) have shown a good response in resistant arthritis and pyoderma gangrenosum.^[9][10][11] Other traditional immunosuppressant treatments for arthritis or pyoderma gangrenosum may also be used.

External links[edit]

Classification	D OMIM: 604416 MeSH: C536253 DiseasesDB: 32724 SNOMED CT: 724015007
External resources	Orphanet: 69126

Categories: 

• Autosomal dominant disorders
• Rare diseases
• Syndromes
• Arthritis
• Acneiform eruptions
• Reactive neutrophilic cutaneous conditions
• Autoinflammatory syndromes

https://en.wikipedia.org/wiki/PAPA_syndrome

P2A2 v. Petersen-USA-et. al., etc.

08-30-2021-1941 -  P2A2 syndrome is an acronym for pyogenic arthritis, pyoderma gangrenosum and acne. It is a rare genetic disorder characterised by its effects on skin and joints.[2][3] P2A2 v. Petersen-USA-et. al., etc. gangar fever pyo pyogene interleukin 1 interlukin tumor necrosis factor immunosuppression immunosuppressant genetic blood hematology osteology rheumatology radiology oncology immunology genology myology neurology cardiology pathology histology 

Category:Autoinflammatory syndromes

From Wikipedia, the free encyclopedia

Jump to navigationJump to search

Autoinflammatory syndromes are a group of inherited disorders characterized by bouts of inflammatory skin lesions and periodic fevers.

The main article for this category is Periodic fever syndrome.

Pages in category "Autoinflammatory syndromes"

The following 24 pages are in this category, out of 24 total. This list may not reflect recent changes (learn more).

 

• Autoinflammatory diseases
• Autoinflammatory syndromes

A

• Autoimmune disease
• Axial spondyloarthritis

B

• Behçet's disease
• Blau syndrome

C

• Chronic infantile neurologic cutaneous and articular syndrome
• Cryopyrin-associated periodic syndrome

D

• Deficiency of the interleukin-1–receptor antagonist

F

• Familial cold autoinflammatory syndrome
• Familial Mediterranean fever

H

• Hyper-IgD syndrome
• Hyperimmunoglobulin D syndrome

K

• Keratoendotheliitis fugax hereditaria

M

• Majeed syndrome
• Muckle–Wells syndrome

P

• PAPA syndrome
• Periodic fever syndrome
• Periodic fever, aphthous stomatitis, pharyngitis and adenitis

S

• Schnitzler syndrome

T

• TNF receptor associated periodic syndrome
• Tumor necrosis factor receptor associated periodic syndrome

V

• Vogt–Koyanagi–Harada disease

Y

• Yao syndrome

https://en.wikipedia.org/wiki/Category:Autoinflammatory_syndromes