Causes
There are many causes including conditions that involve widespread inflammation of the body such as lupus (systemic lupus erythematosus) and rheumatoid arthritis. Alpha-1 Antitrypsin Deficiency is one of those causes however Panniculitis appears to be a very rare manifestation of alpha 1
The inflammation and development of Alpha-1 Panniculitis has similarities to the development of emphysema. In both the skin and the lungs of Alphas, there is a lack of the protein alpha-1 antitrypsin (AAT) which is produced by the liver. The job of the AAT is to protect the body from inflammation, in people with Alpha-1 their AAT is malformed and cannot be released by the liver leading to lung disease and in rare cases Panniculitis.
Panniculitis was first described by physicians in France in 1972 and fewer than 100 cases have been reported since then.
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