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July 1984

Protease-Inhibitor Deficiencies in a Patient With Weber-Christian Panniculitis

Author Affiliations

From the Department of Dermatology, State University of Groningen (the Netherlands).

Arch Dermatol. 1984;120(7):936-940. doi:10.1001/archderm.1984.01650430122023

• 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)

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