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The following information is an abstract from a full article from a 1997 issue of Transplantation.
"Alpha 1-antitrypsin deficiency-associated panniculitis: resolution with intravenous alpha 1-antitrypsin administration and liver transplantation."
O'Riordan K, Blei A, Rao MS, Abecassis M. Department of Medicine,
Northwestern University Medical School, Chicago, IL 60611, USA.
Transplantation 1997 Feb 15;63(3):480-2 Abstract quote:
Panniculitis is a rare complication of alpha 1-antitrypsin (A1AT) deficiency that is characterized by acute inflammatory infiltrate and fat necrosis. Different treatment strategies are used to provide symptomatic relief. Here we describe two patients with homozygous A1AT deficiency who developed panniculitis and were successfully treated with A1AT replacement. The patient who received a liver transplant experienced complete resolution of the skin lesions. The patient who received A1AT intravenously showed complete response, but the skin lesions recurred when the levels of A1AT fell below 50 mg/100 ml. Panniculitis secondary to A1AT deficiency can be successfully treated with liver transplantation or intravenous infusion of A1AT.
This is an additional abstract from a Mayo clinic study on panniculitis.
Panniculitis associated with severe alpha 1-antitrypsin deficiency. Treatment and review of the literature.
Smith KC, Pittelkow MR, Su WP.
Department of Dermatology, Mayo Clinic, Rochester, MN 55905.
Arch Dermatol 1987 Dec;123(12):1655-61 Abstract quote :
Panniculitis associated with homozygous severe alpha 1-antitrypsin deficiency was documented in three women hospitalized for painful cutaneous and subcutaneous ulcerations (severe panniculitis with spontaneous ulceration and drainage of clear or serosanguineous fluid). None had a history of trauma or infection. One patient responded rapidly and completely to treatment with dapsone. One patient, who had more extensive disease, failed to respond to prednisone plus dapsone; infusions of alpha 1-proteinase inhibitor concentrate led to resolution of her panniculitis. One patient who had severe and extensive panniculitis and pleural effusions failed to respond to corticosteroids but did well when both dapsone and infusions of alpha 1-proteinase inhibitor concentrate were added to her treatment program.
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