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Yeast opsonization defect and immunoglobulin deficiency in severe infantile dermatitis (Leiner's disease)

D I Evans, A Holzel, H MacFarlane

    Archives of Disease in Childhood
    |September 1, 1977
    PubMed

    Insights

    Leiner's disease involves extensive dermatitis and failure to thrive, often linked to a complement component 5 (C5) defect. This study suggests C5 deficiency alone may not cause Leiner's disease, requiring hypogammaglobulinaemia for its full expression.

    Area of Science:

    • Immunology
    • Complement System
    • Pediatric Dermatology

    Background:

    • Leiner's disease is a rare infant disorder characterized by severe dermatitis, diarrhea, and failure to thrive.
    • A defect in the fifth component of complement (C5) has been previously implicated as the primary cause.
    • This study investigates the role of C5 deficiency in the context of other immune deficiencies.

    Observation:

    • Two brothers presented with extensive dermatitis and dysgammaglobulinaemia.
    • The younger brother was diagnosed with a C5 defect and exhibited symptoms consistent with Leiner's disease.
    • Despite normal development, the younger brother died suddenly from pertussis.

    Findings:

    • The C5 defect was confirmed in the younger brother.
    • The findings suggest that C5 deficiency alone may not be sufficient to cause the full clinical presentation of Leiner's disease.
    • Hypogammaglobulinaemia or other lymphoid deficiencies appear necessary for the expression of the disease.

    Implications:

    • Revises the understanding of Leiner's disease pathogenesis, highlighting the necessity of combined immune defects.
    • Suggests that patients with C5 deficiency may require additional immune support, particularly against infections.
    • Underscores the importance of comprehensive immune evaluation in infants presenting with severe dermatitis and failure to thrive.

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