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March 1983

Familial Occurrence of a1-Antitrypsin Deficiency and Weber-Christian Disease

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.

Arch Dermatol. 1983;119(3):198-202. doi:10.1001/archderm.1983.01650270016009

• Severe panniculitis of the Weber-Christian type occurred in two brothers, both with marked α1-antitrypsin (α-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 α1-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 α1-AT deficiency with Weber-Christian disease.

(Arch Dermatol 1983;119:198-202)

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