4 results
IgG4-Related Disease
Clinical history:  Man, 60 years, multiorgan involvement, unique or multiple inflammatory pseudotumor
4 phenotypes:
 •
IgG4-Related Disease ... Clinical history ... , lung, lymph node ... concentration PET-CT ... 40% supporting long-term
Plain radiography of the abdomen revealed calcification of both adrenal glands. A homozygous mutation in LIPA
confirmed the diagnosis ... deficiency, or Wolman’s disease ... #clincial #peds ... #infant #NEJM # ... Wolmans #radiology #xray
Lichen Planus- ...On examination, numerous purple papules were noted on the anterior surface of the forearms
confirming the diagnosis ... an autoimmune disease ... used in primary treatment ... #NEJM #clinical ... #derm #wickhams
Anti-NXP-2 Dermatomyositis

What?
DM with generalized subcutaneous edema
Pts have more myalgias, more severe weakness, and an increased prevalence
Violaceous papules or plaques ... auricular papules Clinical ... had more severe disease ... Non-small-cell lung ... positive patients Treatment