• 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.
(Arch Dermatol 114:1182-1186, 1978)
Jessen RT, Van Epps DE, Goodwin JS, Bowerman J. Incontinentia PigmentiEvidence for Both Neutrophil and Lymphocyte Dysfunction. Arch Dermatol. 1978;114(8):1182-1186. doi:10.1001/archderm.1978.01640200036009