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Incontinentia pigmenti. Evidence for both neutrophil and lymphocyte dysfunction.

R T Jessen, D E Van Epps, J S Goodwin

    Archives of Dermatology
    |August 1, 1978
    PubMed
    Summary

    Incontinentia pigmenti patients may have immune system defects. This case highlights recurrent infections and impaired neutrophil and lymphocyte function in a child with this rare genetic disorder.

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    Area of Science:

    • Immunology
    • Pediatrics
    • Genetics

    Background:

    • Incontinentia pigmenti (IP), also known as Bloch-Sultzberger syndrome, is a rare X-linked dominant disorder affecting the skin, central nervous system, eyes, and teeth.
    • Recurrent infections are a known complication in some IP patients, but the underlying immunological mechanisms are not fully understood.

    Observation:

    • A pediatric patient diagnosed with incontinentia pigmenti presented with recurrent episodes of pneumococcal meningitis and bacteremia.
    • The patient also experienced associated subdural hematomas, indicating severe infectious complications.

    Findings:

    • Immunological evaluation revealed a significant defect in neutrophil chemotaxis, the process by which immune cells move towards infection sites.
    • Neutrophil chemiluminescence, a measure of immune cell activation, was normal.

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  • Lymphocyte proliferation response to phytohemagglutinin stimulation was depressed, suggesting impaired cellular immunity.

    • Implications:

    • These findings suggest that underlying immunodeficiency, specifically defective neutrophil function and impaired lymphocyte response, may be a significant component of incontinentia pigmenti.
    • Understanding these immunological defects is crucial for managing infectious complications in IP patients.
    • Further research is warranted to elucidate the precise immunological abnormalities associated with IP and to develop targeted therapeutic strategies.