Incontinentia pigmenti and Behçet's disease: a case of impaired neutrophil chemotaxis.
We describe a patient with incontinentia pigmenti who developed the clinical picture of intestinal Behçet's disease. Diagnosis of Behçet's disease was base on a recurrent genital and oral ulcer and a positive HLA-B51. Impaired neutrophil chemotactic activity and an elevated plasma IL-6 level were found. Chromosomal study revealed no abnormality in the patient or her family. We discuss the possibility that there are common immunological abnormalities in the two syndromes.
Endoh
M
M
Department of Dermatology, Tokyo Medical and Dental University, Japan.
Yokozeki
H
H
Maruyama
R
R
Matsunaga
T
T
Katayama
I
I
Nishioka
K
K
D000328:Adult; D001528:Behcet Syndrome / Q000150:complications* / Q000175:diagnosis / Q000276:immunology; D002634:Chemotaxis, Leukocyte; D004797:Enzyme-Linked Immunosorbent Assay; D005260:Female; D006801:Humans; D007184:Incontinentia Pigmenti / Q000150:complications* / Q000175:diagnosis / Q000276:immunology; D015850:Interleukin-6 / Q000097:blood*; D009504:Neutrophils / Q000502:physiology*
D015850:Interleukin-6