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Pyoderma gangrenosum in a patient with autoimmune haemolytic anaemia and complement deficiency

✍ Scribed by E.A. Coors; P. Von Den Driesch

Publisher
John Wiley and Sons
Year
2000
Tongue
English
Weight
231 KB
Volume
143
Category
Article
ISSN
0007-0963

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✦ Synopsis

About 50% of cases of pyoderma gangrenosum (PG) are associated with a variety of systemic diseases. We describe a patient with severe PG on both legs who suffered from an autoimmune haemolytic anaemia in association with a congenital deficiency of complement factors C2 and C4. To the best of our knowledge, this constellation has not been previously reported.

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Congenital deficiency of beta 2 integrin leucocyte adhesion molecules is a rare immunodeficiency and is often fatal. Neutrophils are unable to bind to ligands on the endothelium, and so cannot leave the circulation during inflammation or infection. When leucocyte adhesion deficiency (LAD) is caused