RESUMO
The rare case of Incontinentia Pigmenti [IP] also known as Bloch-Sulzberger Syndrome was first introduced by Garrod [1906]. This genetically condition involves the skin followed by the teeth. It is also known as a sex linked dominant trait. Brownish gray spots are routinely seen on abdominal and limber skin. The aim of this report is to illustrate the clinical feature of IP cases with reference to earlier reports. An 8.5 year old girl was referred to the pedodontic department for her lack of teeth. Several missing teeth were noted in both jaws with only a number of teeth being present in mouth with change in form. Patient chief complaint was malfunction in mastication and esthetics. Present teeth in clinical exam were as follows: 51,52,61,62,72,73,83. Patient was available for a follow up appointment after 1.5 year. Both upper permanent central incisors were semi erupted at this stage along with newly erupted upper primary canines. Clinical consultation was obtained from departments of oral pathology, oral pediatrician medicine and medical genetics. The condition was agreed as Bloch-Selzberger Syndrome also named as Incontinentia Pigmenti. Based on the collected clinical evidences including the lack of several teeth Incontinentia Pigmenti was diagnosed in association to hyper pigmented spots on skin of abdominal sides. Preventive and intermediate dental treatments are encouraged in such cases