Incontinentia pigmenti. Evidence for both neutrophil and lymphocyte dysfunction.
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.
D002634:Chemotaxis, Leukocyte; D005260:Female; D006801:Humans; D007223:Infant; D037102:Lectins; D008214:Lymphocytes / Q000276:immunology*; D009504:Neutrophils / Q000276:immunology*; D010859:Pigmentation Disorders / Q000276:immunology* / Q000473:pathology; D012871:Skin Diseases / Q000276:immunology* / Q000473:pathology; D013577:Syndrome
D037102:Lectins