Panniculitis associated with severe alpha 1-antitrypsin deficiency. Treatment and review of the literature

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Abstract
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.