Accepted for publication July 20, 1983.
Reprints not available.
From the Department of Dermatology, State University of Groningen (the Netherlands).
• The diagnosis of Weber-Christian disease was made by clinical and histopathologic findings in a 25-year-old woman who had recurrent nodules on the legs and arms. The patient's history also disclosed multiple episodes of swelling from trauma. Histopathologic examination of the lesions showed a prominent vasculitis. Studies of serum complement and kallikrein levels and of the fibrinolysisclotting system showed a decrease in the levels of C3, C4, and total hemolytic complement activity and deficiencies (<20% of the normal values) of α1-antitrypsin (α1-AT) and antichymotrypsin activity. Chemical analyses of the patient's α1-AT indicated a PiZZ genotype. Intermediate values of both inhibitor levels were detected in six family members. It is assumed that protease-inhibitor deficiencies predispose the development of panniculitis and vasculitis on trauma.
(Arch Dermatol 1984;120:936-940)
Country-Specific Mortality and Growth Failure in Infancy and Yound Children and Association With Material Stature
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