Disease Information for PAPA Syndrome

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Clinical Manifestations
Signs & Symptoms
Cutaneous ulcer/Skin ulcer
Cutaneous ulcers/under-cut border
Delayed/poor wound healing
Hyperpathia phenomenon (venipuncture inf)
Skin pathergy/needle stick/sterile pustule/erythema
Asymmetric arthritis/multiple joints
Joint Swelling
Clinical Presentation & Variations
Acne in Adults
Pyogenic Arthritis Pyoderma Gangrenosa Cystic Acne
Demographics & Risk Factors
Population Group
Population/Pediatrics population
Young Adult
Sex & Age Groups
Population/Adolescent
Population/Adult ('twenties')
Population/Child
Laboratory Tests
Microbiology & Serology Findings
Microlab/Joint fluid culture negative/sterile fluid
Abnormal Lab Findings - Increased
SYNOVIAL Leukocytes
SYNOVIAL Neutrophiles
Diagnostic Test Results
Pathology
BX/Skin/Acute Inflammation Polys
PATH/Tissue neutrophiles/microabscessing/necrosis
X-RAY
Xray/Joint abnormal
Associated Diseases & Rule outs
Rule Outs
Job syndrome
Juvenile rheumatoid arthritis/chronic
Associated Disease & Complications
Arthritis Pyogenic Septic
Cystic acne vulgaris
PAPA syndrome
Polyarthritis in Children
Pyoderma gangrenosa
Pyogenic Hip Arthritis
Pyogenic Knee Arthritis
Disease Mechanism & Classification
Class
CLASS/Skin disorder (ex)
CLASS/Joint disorder (ex).
Pathophysiology
Pathophysiology/Autoinflammatory (non-immune)
Pathophysiology/Gene locus Chromosome 15
Pathophysiology/Gene mutation CD2BP1
Pathophysiology/Immune physiology/defective
Process
PROCESS/Autosomal dominant hereditary disease (ex).
PROCESS/Genetic expressivity variable (ex)
PROCESS/Hereditofamilial (category)
PROCESS/INCIDENCE/Rare disease (ex)
PROCESS/Variant expressions/Subsets (ex)
Treatment
Drug Therapy - Indication
RX/Anakinra (Kineret)
RX/Anti-TNF drug
RX/Corticosteroid (Cortisone)
RX/Etanercept (Enbrel)/Anti-TNF
RX/Infliximab (Remicade)
Definition
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External Links Related to PAPA Syndrome
Google
Wikipedia
Merck
Images
PubMed (National Library of Medicine)
NGC (National Guideline Clearinghouse)
Medscape (eMedicine)
Harrison's Online (accessmedicine)
NEJM (The New England Journal of Medicine)
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