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Alpha-1 Panniculitis

Panniculitis is an inflammation of the panniculus which is the fatty layer under the outer layer of the skin.

The condition typically appears as small, red, bumpy nodules on one or more areas of the body, but more commonly on the thighs, buttocks and areas subject to injury or pressure

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.

Diagnosis

A  skin biopsy is needed to confirm Panniculitis, generally performed under local anesthetic. The procedures involves the doctor cutting out a small wedge of skin and then sending it to a pathology lab for examination.

Treatment

Various therapies have been tried for panniculitis and panniculitis due to Alpha-1 which have included corticosteroids and antibiotics. In Panniculitis due to Alpha-1 intravenous infusions of donated alpha-1 protein known as augmentation therapy has been found to be the most successful.

 
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