Incontinentia pigmenti. Evidence for both neutrophil and lymphocyte dysfunction

Arch Dermatol. 1978 Aug;114(8):1182-6. doi: 10.1001/archderm.114.8.1182.


A child with incontinentia pigmenti (Bloch-Sultzberger syndrome) had recurrent pneumococcal meningitis and pneumococcal bacteremia with associated subdural hematomas. Immunologic evaluation revealed defective neutrophil chemotaxis with normal neutrophil chemiluminescense. In addition, lymphocytes showed a depressed proliferative response to phytohemagglutinin stimulation. An immunologic defect may prove to be part of this syndrome.

Publication types

  • Case Reports

MeSH terms

  • Chemotaxis, Leukocyte
  • Female
  • Humans
  • Infant
  • Lectins
  • Lymphocytes / immunology*
  • Neutrophils / immunology*
  • Pigmentation Disorders / immunology*
  • Pigmentation Disorders / pathology
  • Skin Diseases / immunology*
  • Skin Diseases / pathology
  • Syndrome


  • Lectins