Sweet syndrome
- aka acute febrile neutrophilic dermatosis
- pathophysiology: after URI or GI infection, in setting of heme problems such as MDS or myelodysplastic syndrome evolving to AML
- association with medications that stimulates neutrophils: all-trans retinoic acid, GCS-F
- sx:
- high fever
- neutrophilia
- leukocytosis with left shift
- elevated inflammatory markers
- muscle/joint pain
- dense dermal infiltrate on histology
- "juicy" skin lesions: painful, edematous, red to violet
- no infection, responsive to steroids
mimics cellulitis
Backlinks
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Rheumatoid Arthritis
rheumatoid arthritis
Pathophysiology and Risk Factors
- genetic risks:
- 60% of risks
- HLA class II most important, especially HLA-D
- These HLA code for citrullinated peptide antigen that are immunogenic since citruline is not normally in humans
- citrulline formed by peptidylarginine deiminase
- environmental: 40%
- smoking activates PADI and citrullination, increases risk to 2-20x
- infection
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