Panniculitis Associated With Severe a1-Antitrypsin Deficiency
- 1 December 1987
- journal article
- review article
- Published by American Medical Association (AMA) in Archives of Dermatology
- Vol. 123 (12), 1655-1661
- https://doi.org/10.1001/archderm.1987.01660360083017
Abstract
• Panniculitis associated with homozygous severe α1-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 α1-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 α1-proteinase inhibitor concentrate were added to her treatment program. (Arch Dermatol 1987;123:1655-1661)Keywords
This publication has 32 references indexed in Scilit:
- Risk of Cirrhosis and Primary Liver Cancer in Alpha1-Antitrypsin DeficiencyNew England Journal of Medicine, 1986
- Relationship between Weber-Christian panniculitis and the ZZ phenotype of alpha1-antitrypsinArchiv für dermatologische Forschung, 1985
- The Role of α1 Protease Inhibitor (α1 Antitrypsin) in the Regulation of Immunologic and Inflammatory ReactionsAustralian and New Zealand Journal of Medicine, 1980
- Report of nomenclature meeting for α1-antitrypsinHuman Genetics, 1980
- alpha1-Antitrypsin deficiency associated with persistent cutaneous vasculitis. Occurrence in a child with liver diseaseArchives of Dermatology, 1978
- Liver Disease in Alpha1-Antitrypsin Deficiency Detected by Screening of 200,000 InfantsNew England Journal of Medicine, 1976
- Liver, Lung and Malignant Disease in Heterozygous (Pi MZ) α1‐antitrypsin DeficiencyActa Medica Scandinavica, 1975
- Alpha1-antitrypsin and its deficiency.Published by Elsevier BV ,1974
- Isolation of Elastase-Like and Chymotrypsin-Like Neutral Proteases from Human GranulocytesHoppe-Seyler´s Zeitschrift Für Physiologische Chemie, 1974
- Neutrophil Proteases in InflammationAnnual Review of Medicine, 1972