Dysimmune diseases

Articles

• systemic lupus erythematosus
  16 October 2003

  Definition: Systemic lupus erythematosus (SLE) is an autoimmune disease that is characterized by autoantibodies specific for highly conserved nuclear antigens, such as double-stranded DNA, histones and ribonuclear proteins.
  Digital case
  JRC:10585 : Cutaneous lupus erythematosus.
  Images
  LE cell in peritoneal fluid : https://twitter.com/pwraess/status/732709988368416768
  The requirements for the activation and response of self-reactive B cells — the source of autoantibodies — seem to be (...)

• IPEX syndrome
  12 November 2003

  Definition: IPEX is a X-inked fatal disorder characterized by immune dysregulation, polyendocrinopathy, enteropathy and X-linked inheritance (MIM.304930).
  Patients typically present in infancy with severe diarrhea and failure to thrive. Most children die by 1 year of age without therapy. The diagnosis is established by genetic analysis, which often takes several weeks to complete and can sometimes delay crucial immunosuppressive treatment.
  Synopsis
  dysimmune enteropathy (autoimmune (...)

• APECED
  21 November 2003

  Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED); autoimmune polyendocrine syndrome type I; autoimmune polyendocrinopathy syndrome type I; APS1; Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy
  Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED) is a rare autosomal recessive disorder defined by the presence of two of three conditions, namely, Addison’s disease, hypoparathyroidism, and mucocutaneous candidiasis.
  Autoimmune (...)

• hemophagocytic lymphohistiocytosis
  22 February 2005

  FHLs, HPLH, HLH, familial erythrophagocytic lymphohistiocytosis, familial hemophagocytic lymphohistiocytosis, familial hemophagocytic lymphohistiocytoses
  Definition: Familial hemophagocytic lymphohistiocytosis (FHL) is a rare, fatal disorder of early infancy.
  Images
  hepatic erythrophagocytosis in hemophagocytic lymphohistiocytosis
  https://twitter.com/GIPathologyURMC/status/889533077118214144
  See also
  erythrophagocytosis
  hepatic erythrophagocytosis
  Hemophagocytic (...)

• systemic sclerosis
  28 November 2009

  scleroderma

• zymogen granules
  19 June 2017
• autoimmune lymphoproliferative syndrome
  6 April 2004

  ALPS, Canale-Smith syndrome

• Sweet syndrome
  29 July 2007

  Sweet disease, acute febrile neutrophilic dermatosis

• Buerger disease
  24 July 2007

  Buerger arteritis

• antiphospholipid syndrome
  25 October 2004

  Definition: The antiphospholipid syndrome is characterized by clinical evidence of arterial or venous thrombosis, thrombocytopaenia, recurrent fetal loss and repeated positivity of antiphospholipid autoantibodies.
  The designation ’antiphospholipid syndrome’ was proposed for the association of arterial and venous thrombosis, recurrent fetal loss, and immune thrombocytopenia with a spectrum of autoantibodies directed against cellular phospholipid components.
  Anticardiolipin antibodies may (...)

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