title: Incontinentia pigmenti.
abstract: Incontinentia pigmenti (IP) or Bloch-Sulzberger syndrome is a rare X-linked dominant genodermatosis related to the NF kappa B essential modulator (NEMO) gene with approximately 800 cases reported worldwide. It usually occurs in females characterized by cutaneous, skeletal, neurological, ocular and dental abnormalities as well as an increased risk of childhood malignancies. Herein, we report a case of IP in a 14-year-old girl emphasizing early diagnosis and adding to the current literature on the subject.
authors.affiliation: Department of Pathology, Trauma Research Center, Baqiyatallah University of Medical Sciences, and Attending Surgeon, Azad University of Medical Sciences, Tehran, Iran.
authors.forename: Mohammad Hosein Kalantar
authors.initials: MH
authors.lastname: Motamedi
authors.forename: Ali
authors.initials: A
authors.lastname: Lotfi
authors.forename: Taghi
authors.initials: T
authors.lastname: Azizi
authors.forename: Mohammad
authors.initials: M
authors.lastname: Moshref
authors.forename: Sareh
authors.initials: S
authors.lastname: Farhadi
mesh_terms: D002648:Child; D005260:Female; D006651:Histocytochemistry; D006801:Humans; D007184:Incontinentia Pigmenti / Q000175:diagnosis* / Q000473:pathology*; D008437:Maxilla / Q000473:pathology; D008853:Microscopy; D009264:Nails, Malformed / Q000473:pathology; D012867:Skin / Q000473:pathology; D050277:Toe Phalanges / Q000473:pathology; D014070:Tooth / Q000473:pathology