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Related Experiment Videos

Darier's disease: an immunologic study.

J G Marks, D E Thor, R S Lowe

    Archives of Dermatology
    |September 1, 1978
    PubMed
    Summary
    This summary is machine-generated.

    Patients with Darier's disease exhibit normal humoral immunity but impaired cell-mediated immunity (CMI). This immune deficiency may explain frequent bacterial skin infections and Kaposi's varicelliform eruption in Darier's disease patients.

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    Area of Science:

    • Dermatology
    • Immunology
    • Genetics

    Background:

    • Darier's disease is a rare genetic skin disorder.
    • Patients often experience secondary bacterial skin infections and Kaposi's varicelliform eruption.
    • The underlying immune status in Darier's disease is not well understood.

    Purpose of the Study:

    • To investigate the humoral and cell-mediated immunity (CMI) in patients with Darier's disease.
    • To determine if immune deficiencies contribute to the susceptibility to infections in Darier's disease.

    Main Methods:

    • Evaluated humoral immunity through quantitative immunoglobulins, isohemagglutinins, direct skin immunofluorescence, and B-cell counts.
    • Assessed CMI using delayed type hypersensitivity skin tests, T-cell counts, lymphocyte transformation assays, macrophage inhibition factor (MIF) assays, and skin windows.

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  • Tested polymorphonuclear leukocyte chemotaxis and phagocytosis.

    • Main Results:

    • Humoral immunity was found to be normal in all four patients.
    • Cell-mediated immunity (CMI) was depressed, indicated by blunted lymphocyte blastogenesis, MIF, and skin window responses.
    • Polymorphonuclear leukocyte chemotaxis and phagocytosis were normal.

    Conclusions:

    • Patients with Darier's disease have normal humoral immunity.
    • A defect in cell-mediated immunity (CMI) is present in Darier's disease.
    • The impaired CMI likely contributes to the increased susceptibility to bacterial skin infections and Kaposi's varicelliform eruption observed in Darier's disease.