Panniculitis associated with severe alpha 1-antitrypsin deficiency. Treatment and review of the literature.
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.
Smith
K C
KC
Department of Dermatology, Mayo Clinic, Rochester, MN 55905.
Pittelkow
M R
MR
Su
W P
WP
D000273:Adipose Tissue / Q000473:pathology; D000328:Adult; D000368:Aged; D001706:Biopsy; D001798:Blood Proteins / Q000627:therapeutic use; D003622:Dapsone / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D005260:Female; D006801:Humans; D010201:Panniculitis, Nodular Nonsuppurative / Q000188:drug therapy / Q000473:pathology*; D010641:Phenotype; D011241:Prednisone / Q000627:therapeutic use; D011480:Protease Inhibitors / Q000627:therapeutic use; D012008:Recurrence; D000515:alpha 1-Antitrypsin / Q000032:analysis; D019896:alpha 1-Antitrypsin Deficiency
D001798:Blood Proteins; D011480:Protease Inhibitors; D000515:alpha 1-Antitrypsin; D003622:Dapsone; D011241:Prednisone