 |
|
Familial Occurrence of  1-Antitrypsin Deficiency and Weber-Christian Disease
Samuel N. Breit, MB, BS, FRACP, FRCPA;
Peggy Clark, PhD;
J. Paul Robinson, MSc;
Elizabeth Luckhurst;
Roger L. Dawkins, MB, BS, BSc(Med), MD, MRCP, FRACP, FRCPA;
Ronald Penny, DSc, MD, BS, FRACP, FRCPA
Arch Dermatol. 1983;119(3):198-202.
Abstract
• Severe panniculitis of the Weber-Christian type occurred in two brothers, both with marked  1-antitrypsin (-AT) deficiency and phenotype PiZZ. Studies of inflammatory and immunologic function were undertaken in these two patients as well as in a third brother with severe 1-AT deficiency but without Weber-Christian disease. The findings of these investigations were suggestive of exaggerated immunologic and inflammatory function with enhanced lymphocyte responsiveness to phytohemagglutinin, enhanced activation of neutrophils and monocytes, and accelerated delayed hypersensitivity responses in all three subjects. This hyperreactivity may explain the apparent association of 1-AT deficiency with Weber-Christian disease.
(Arch Dermatol 1983;119:198-202)
Author Affiliations
From the Department of Immunology, St Vincent's Hospital and the Department of Medicine, University of South Wales, Sydney (Drs Breit and Penny, Mr Robinson, and Ms Luckhurst), The Institute of Clinical Pathology and Medical Research, Westmead (Dr Clark), and the Department of Clinical Immunology, Royal Perth Hospital, Perth (Dr Dawkins), Australia.
Footnotes
Accepted for publication May 17, 1982.
Reprint requests to Department of Immunology, St Vincent's Hospital, Victoria Street, Darlinghurst NSW 2010, Australia (Dr Breit).
 CiteULike  Connotea  Del.icio.us  Digg  Reddit  Technorati  Twitter
What's this?
THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES
American Thoracic Society/European Respiratory Society Statement: Standards for the Diagnosis and Management of Individuals with Alpha-1 Antitrypsin Deficiency
Am. J. Respir. Crit. Care Med. 2003;168:818-900.
FULL TEXT
Mucinous Nevus
Rongioletti and Rebora
Arch Dermatol 1996;132:1522-1523.
ABSTRACT
Panniculitis New Findings in the `Third Compartment'
Patterson
Arch Dermatol 1987;123:1615-1618.
ABSTRACT
Panniculitis Associated With Severe {alpha}1-Antitrypsin Deficiency: Treatment and Review of the Literature
Smith et al.
Arch Dermatol 1987;123:1655-1661.
ABSTRACT
Protease-Inhibitor Deficiencies in a Patient With Weber-Christian Panniculitis
Bleumink and Klokke
Arch Dermatol 1984;120:936-940.
ABSTRACT
|
