PEDIATRICS Vol. 71 No. 1 January 1983, pp. 81-87
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Inherited Deficiency of the Third Component of Complement Associated with Recurrent Pyogenic Infections, Circulating Immune Complexes, and Vasculitis in a Dutch Family

John J. Roord MD1, Mohamed Daha PhD1, Wietse Kuis MD1, Henri A. Verbrugh MD1, Jan Verhoef MD1, Ben J. M. Zegers PhD1, and Jan W. Stoop MD1

1 From the University Children's Hospital Het Wilhelmina Kinderziekenhuis, Utrecht; University Hospital, Leiden; and University Hospital, Utrecht, The Netherlands

A family is described in which 3/11 children showed a homozygous deficiency of C3, and both parents and six other children had subnormal levels of C3. The three children with selective C3 deficiency suffered repeatedly from bacterial infections, whereas the parents and the other siblings were clinically healthy. During infectious episodes the patients showed a maculopapular skin rash, and at such times immune complexes were present in the serum. Biopsy specimens of the skin lesions showed the picture of leukocytoclastic vasculitis.

Key Words: complement deficiency • third component of complement • vasculitis • recurrent infections • circulating immune complexes

Submitted on January 13, 1982
Accepted on April 7, 1982




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