Region Jnkpings ln Odontologiska Institutionen

Oral Status in thirty Swedish Individuals with Incontinentia Pigmenti

BERGENDAL B* , National Oral Disability Centre, The Institute for Postgraduate Dental Education, Jönköping, Sweden

HALLBERG G, Department of Paediatric Dentistry, County Council of Uppland, Uppsala, Sweden

Incontinentia Pigmenti (I.P.) is one out of several ectodermal dysplasia syndromes. The organs predominantly affected are eyes, skin and teeth. At the annual meeting of the patient organization for Incontinentia Pigmenti in 1998 in Sweden all diagnosis carriers were odontologically examined. They were 30 individuals, 29 women and one man, aged 1 - 56 years representing one to three generations of 17 families. Diagnosis had been set by a clinical geneticist in co-operation with ophthalmologist and dermatologist. Thirteen (43%) of the individuals had severe and sight threatening eye manifestations. Many of the mothers had got the diagnosis after giving birth to a child with obvious skin symptoms in the neonatal period. Some of these mothers had no or very discreet clinical dental symptoms.

All 30 individuals were clinically examined and an orthopanthomogram was taken of all but 4 individuals (3 children below the age of five and one child with severe mental retardation). Dental photographs, impressions for study casts and evaluation of stimulated whole saliva was also performed.
The dominating dental symptom was thooth agenesis which appeared in 22 of the individuals and varied from agenesia of one to eighteen teeth (mean 6). Atypical tooth form was found in the primary as well as the permanent dentition. Generally smaller tooth size than normal and parallel proximal surfaces was also a common finding. Salivary secretion rates were examined by sampling of stimulated whole saliva for five minutes. Thirteen of the 25 examined individuals had normal salivary secretion rates and in 12 individuals secretion rates were lower than normal (£ 0.7 ml/min). Some of the adult individuals had undergone extensive prosthodontic treatment e.g. bone augmentation preceding implant therapy. The high prevalance of impaired salivary function has not earlier been described in the literature and calls for special attention in the treatment planning and maintenance care for this group of patients. It is therefore of utmost importance that the diagnosis is set early and here the dental profession has a great responsibility since the dental conditions are often the only reason to seek care. Referral to ophthalmologist is mandatory to diminish the risk of impairment of sight or even blindness.

Professionals in the dental field are ideally placed to identify individuals with Incontinentia Pigmenti and should ensure that such patients are referred to the proper medical specialists.

Uppdaterad: 2013-04-03
Anna Thofelt, Avdelningen för odontologisk radiologi Jönköping, Folktandvården