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  • Title: Protease-inhibitor deficiencies in a patient with Weber-Christian panniculitis.
    Author: Bleumink E, Klokke HA.
    Journal: Arch Dermatol; 1984 Jul; 120(7):936-40. PubMed ID: 6203490.
    Abstract:
    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 trauma. Histopathologic examination of the lesions showed a prominent vasculitis. Studies of serum complement and kallikrein levels and of the fibrinolysis-clotting system showed a decrease in the levels of C3, C4, and total hemolytic complement activity and deficiencies (less than 20% of the normal values) of alpha 1-antitrypsin (alpha 1-AT) and antichymotrypsin activity. Chemical analyses of the patient's alpha 1-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.
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