1. Pyoderma eczema and folliculitis with defective leucocyte and lymphocyte function: a new familial immunodeficiency disease responsive to a histamine-1 antagonist.
- Author
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Jung LK, Engelhard D, Kapoor N, Pih K, and Good RA
- Subjects
- Blepharitis drug therapy, Blepharitis genetics, Child, Dermatitis, Atopic drug therapy, Dermatitis, Atopic genetics, Eczema genetics, Female, Folliculitis genetics, Humans, Immunologic Deficiency Syndromes genetics, Male, Pedigree, Pyoderma genetics, Chlorpheniramine therapeutic use, Eczema drug therapy, Folliculitis drug therapy, Immunologic Deficiency Syndromes drug therapy, Leukocytes immunology, Lymphocytes immunology, Pyoderma drug therapy
- Abstract
A new familial immunodeficiency disease characterised by recurrent and persistent pyoderma, folliculitis, and atopic dermatitis is described in a father and son. It is accompanied by abnormalities of lymphocyte function (including defective proliferative responses to phytomitogens, and subnormal response in immunoglobulin production after stimulation of the lymphocytes by pokeweed mitogen) and defective leucocyte chemiluminescence responses, which were associated with defective ability for intracellular killing of microbial organisms. The abnormalities of lymphocyte and leucocyte function, as well as the clinical manifestations, responded dramatically to treatment with the histamine-1 antagonist, chlorpheniramine, suggesting that the underlying defect in this disease may relate to defective histamine metabolism or abnormal expression of histamine receptors on lymphocytes and leucocytes.
- Published
- 1983
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