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Alpha‐1 anti‐trypsin deficiency and Henoch‐Schönlein purpura associated with anti‐neutrophil cytoplasmic and anti‐endothelial cell antibodies of immunoglobulin‐A isotype
Author(s) -
Patterson Cynthia C.,
Jr Patrick Ross,
PopeHarman Amy L.,
Knight Deborah A.,
Magro Cynthia M.
Publication year - 2005
Publication title -
journal of cutaneous pathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.597
H-Index - 75
eISSN - 1600-0560
pISSN - 0303-6987
DOI - 10.1111/j.0303-6987.2005.00304.x
Subject(s) - medicine , immunology , antibody , henoch schonlein purpura , vasculitis , purpura (gastropod) , autoantibody , anti neutrophil cytoplasmic antibody , polyarteritis nodosa , pathology , disease , biology , ecology
Alpha‐1 anti‐trypsin (A1AT) deficiency is an inherited enzyme deficiency that manifests with fatal lung and liver complications. In addition to pulmonary and hepatic involvement, the disease has also been linked to an increased incidence of vasculitic syndromes and autoimmune diseases, including Wegener's granulomatosis, microscopic polyarteritis nodosa and Henoch‐Schönlein purpura (HSP). HSP, a systemic, small‐vessel vasculitis syndrome, is characterized by a non‐thrombocytopaenic purpuric rash, arthralgia, abdominal pain and nephritis. Both A1AT deficiency and HSP have been associated with anti‐neutrophil cytoplasmic antibodies (ANCA) and anti‐endothelial cell antibodies (AECA). We report a case of a 40‐year‐old man with severe A1AT deficiency, who developed HSP associated with AECA, ANCA and anti‐phospholipid antibodies of the immunoglobulin‐A isotype.