Biomaterial Database

Incontinentia pigmenti: a case report. 2006

R Bentolila, and H Rivera, and M C Sanchez-Quevedo

Pedodontics Graduate Program, Faculty of Dentistry, Central University of Venezuela, Caracas, Venezuela.

Incontinentia pigmenti (IP) is a genodermatosis with an X-linked dominant mode of inheritance, characterized by ectodermal, mesodermal, neurological, ocular, and dental manifestations. The purpose of this case study was to report the oral and dental manifestations of an IP case in a Venezuelan pediatric patient. A 9 year-old Venezuelan girl was evaluated. She showed macular pigmented lesions in her face, trunk, back, legs, and fingers as well as abnormal hair distribution, alopecia on the vertex, and hypoplasia of eyebrows. During the dental examination, conical shaped-teeth and delayed dental eruption was evidenced. The microanalytical examination showed dentin without significant alterations in the mineralization except for hypermineralization in focal areas. In addition, a decrease in the enamel mineralization was observed.

UI	MeSH Term	Description	Entries
D007184	Incontinentia Pigmenti	A genodermatosis occurring mostly in females and characterized by skin changes in three phases - vesiculobullous, verrucous papillomatous, and macular melanodermic. Hyperpigmentation is bizarre and irregular. Sixty percent of patients have abnormalities of eyes, teeth, central nervous system, and skin appendages.	Bloch-Sulzberger Syndrome,Bloch-Siemens Syndrome,Bloch-Siemens-Sulzberger Syndrome,Bloch Sulzberger Syndrome,Syndrome, Bloch-Sulzberger
D011859	Radiography	Examination of any part of the body for diagnostic purposes by means of X-RAYS or GAMMA RAYS, recording the image on a sensitized surface (such as photographic film).	Radiology, Diagnostic X-Ray,Roentgenography,X-Ray, Diagnostic,Diagnostic X-Ray,Diagnostic X-Ray Radiology,X-Ray Radiology, Diagnostic,Diagnostic X Ray,Diagnostic X Ray Radiology,Diagnostic X-Rays,Radiology, Diagnostic X Ray,X Ray Radiology, Diagnostic,X Ray, Diagnostic,X-Rays, Diagnostic
D002648	Child	A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL.	Children
D003743	Dental Enamel	A hard thin translucent layer of calcified substance which envelops and protects the dentin of the crown of the tooth. It is the hardest substance in the body and is almost entirely composed of calcium salts. Under the microscope, it is composed of thin rods (enamel prisms) held together by cementing substance, and surrounded by an enamel sheath. (From Jablonski, Dictionary of Dentistry, 1992, p286)	Enamel,Enamel Cuticle,Dental Enamels,Enamel, Dental,Enamels, Dental,Cuticle, Enamel,Cuticles, Enamel,Enamel Cuticles,Enamels
D003804	Dentin	The hard portion of the tooth surrounding the pulp, covered by enamel on the crown and cementum on the root, which is harder and denser than bone but softer than enamel, and is thus readily abraded when left unprotected. (From Jablonski, Dictionary of Dentistry, 1992)	Dentine,Dentines,Dentins
D004577	Electron Probe Microanalysis	Identification and measurement of ELEMENTS and their location based on the fact that X-RAYS emitted by an element excited by an electron beam have a wavelength characteristic of that element and an intensity related to its concentration. It is performed with an electron microscope fitted with an x-ray spectrometer, in scanning or transmission mode.	Microscopy, Electron, X-Ray Microanalysis,Spectrometry, X-Ray Emission, Electron Microscopic,Spectrometry, X-Ray Emission, Electron Probe,X-Ray Emission Spectrometry, Electron Microscopic,X-Ray Emission Spectrometry, Electron Probe,X-Ray Microanalysis, Electron Microscopic,X-Ray Microanalysis, Electron Probe,Microanalysis, Electron Probe,Spectrometry, X Ray Emission, Electron Microscopic,Spectrometry, X Ray Emission, Electron Probe,X Ray Emission Spectrometry, Electron Microscopic,X Ray Emission Spectrometry, Electron Probe,X-Ray Microanalysis,Electron Probe Microanalyses,Microanalyses, Electron Probe,Microanalysis, X-Ray,Probe Microanalyses, Electron,Probe Microanalysis, Electron,X Ray Microanalysis,X Ray Microanalysis, Electron Microscopic,X Ray Microanalysis, Electron Probe
D005260	Female		Females
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D014071	Tooth Abnormalities	Congenital absence of or defects in structures of the teeth.	Odontome,Abnormalities, Teeth,Abnormalities, Tooth,Teeth Abnormalities,Abnormality, Teeth,Abnormality, Tooth,Odontomes,Teeth Abnormality,Tooth Abnormality
D014074	Tooth Calcification	The process whereby calcium salts are deposited in the dental enamel. The process is normal in the development of bones and teeth. (Boucher's Clinical Dental Terminology, 4th ed, p43)	Calcification, Tooth,Teeth Calcification,Calcification, Teeth,Calcifications, Teeth,Calcifications, Tooth,Teeth Calcifications,Tooth Calcifications