Gleich's syndrome or episodic angioedema with eosinophilia is a rare disease in which the body swells up episodically (angioedema), associated with raised antibodies of the IgM type and increased numbers of eosinophil granulocytes, a type of white blood cells, in the blood (eosinophilia). Angioedema ( BE: angiooedema) also known by its Eponym Quincke's edema, is the rapid swelling ( Edema) of the Dermis Immunoglobulin M, or IgM for short is a basic Antibody that is present on B cells It is the primary antibody against A and B Eosinophil granulocytes, usually called eosinophils (or less commonly acidophils) are White blood cells that are one of the Immune System components Eosinophilia is the state of having a high concentration of eosinophils ( Eosinophil granulocytes in the Blood. It was first described in 1984. [1]
Its cause is unknown, but it is unrelated to capillary leak syndrome (which may cause similar swelling episodes) and eosinophilia-myalgia syndrome (which features eosinophilia but alternative symptoms). Capillary leak syndrome (sometimes systemic capillary leak syndrome or Clarkson syndrome) is a rare Medical condition where the number and size of the Eosinophilia-myalgia syndrome (EMS is an incurable and sometimes fatal flu-like neurological condition that is believed to have been caused by ingestion of poorly produced L-tryptophan Moreover, it is not a form of hypereosinophilic syndrome as there is no evidence that it leads to organ damage. The hypereosinophilic syndrome (HS is a disease process characterized by a persistently elevated eosinophil count (≥ 1500 eosinophils/mm³ in the blood for at least six months without Some studies have shown that edema attacks are associated with degranulation (release of enzymes and mediators from eosinophils), and others have demonstrated antibodies against endothelium (cells lining blood vessels) in the condition. Antibodies (also known as immunoglobulins, abbreviated Ig) are Gamma globulin Proteins that are found in Blood or other Bodily The endothelium is the thin layer of cells that line the interior surface of Blood vessels forming an interface between circulating Blood in the [2]
Gleich syndrome has a good prognosis. Attack severity may improve with steroid treatment. Glucocorticoids (GC are a class of Steroid hormones characterised by an ability to bind with the glucocorticoid receptor ( GR) and trigger similar effects [1][2]
References
- ^ a b Gleich GJ, Schroeter AL, Marcoux JP, Sachs MI, O'Connell EJ, Kohler PF (1984). "Episodic angioedema associated with eosinophilia". N. Engl. J. Med. 310 (25): 1621–6. PMID 6727934.
- ^ a b Emonet S, Kaya G, Hauser C (2000). "Gleich's syndrome". Ann Dermatol Venereol 127 (6-7): 616–8. PMID 10930860.