title: Incontinentia pigmenti and Behçet's disease: a case of impaired neutrophil chemotaxis.
abstract: 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.
authors.affiliation: Department of Dermatology, Tokyo Medical and Dental University, Japan.
authors.forename: M
authors.initials: M
authors.lastname: Endoh
authors.forename: H
authors.initials: H
authors.lastname: Yokozeki
authors.forename: R
authors.initials: R
authors.lastname: Maruyama
authors.forename: T
authors.initials: T
authors.lastname: Matsunaga
authors.forename: I
authors.initials: I
authors.lastname: Katayama
authors.forename: K
authors.initials: K
authors.lastname: Nishioka
mesh_terms: 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*
chemical_list: D015850:Interleukin-6