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

Arch Dermatol. 1987 Dec;123(12):1655-61.


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.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adipose Tissue / pathology
  • Adult
  • Aged
  • Biopsy
  • Blood Proteins / therapeutic use
  • Dapsone / therapeutic use
  • Drug Therapy, Combination
  • Female
  • Humans
  • Panniculitis, Nodular Nonsuppurative / drug therapy
  • Panniculitis, Nodular Nonsuppurative / pathology*
  • Phenotype
  • Prednisone / therapeutic use
  • Protease Inhibitors / therapeutic use
  • Recurrence
  • alpha 1-Antitrypsin / analysis
  • alpha 1-Antitrypsin Deficiency*


  • Blood Proteins
  • Protease Inhibitors
  • alpha 1-Antitrypsin
  • Dapsone
  • Prednisone