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Buckley's syndrome

A Rebora, E Nunzi, M Pezzuolo

    The British Journal of Dermatology
    |November 1, 1978
    PubMed
    Summary
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    This study identifies a rare immune disorder in an infant with eczema and infections, characterized by impaired neutrophil function. Treatment with Levamisole showed improvement in both laboratory defects and clinical symptoms.

    Area of Science:

    • Immunology
    • Pediatrics
    • Genetics

    Background:

    • Recurrent infections and chronic eczema in infants can indicate underlying primary immunodeficiency.
    • Elevated immunoglobulin E (IgE) and impaired neutrophil chemotaxis are key indicators of specific immune dysfunctions.

    Observation:

    • A case study of an infant girl presenting with chronic eczema, recurrent infections, elevated IgE, and impaired neutrophil chemotaxis.
    • The patient's clinical presentation aligns with the characteristics of patients described by Buckley, Wray & Belmaker (1972).

    Findings:

    • A similar neutrophil chemotaxis defect was identified in the patient's mother, suggesting a potential hereditary component.
    • In vitro testing demonstrated that Levamisole could correct the impaired neutrophil chemotaxis.

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  • Clinical improvement was observed in the patient following Levamisole treatment.

    • Implications:

    • This case highlights a potentially heritable immune disorder affecting neutrophil function.
    • Levamisole shows promise as a therapeutic agent for this specific immune defect, improving both laboratory parameters and clinical outcomes.
    • Further research into the genetic basis and broader therapeutic applications of Levamisole in similar pediatric immune deficiencies is warranted.