We describe a 19-year-old girl with Kindler syndrome. She has suffered from bullae on pressure areas of the skin since birth. These healed with atrophic scars and caused marked atrophy of the skin of the palms and soles and wrinkled and parchment-like skin of the dorsum of the hands and feet. Since infancy the patient has also suffered from severe photosensitivity on exposed areas and developed poikiloderma on the skin of her face, neck, chest, and upper back. Since age 17 years the patient has been free of bullae but moderate photosensitivity exists. Oral examination showed limitation of mouth opening, ankyloglossia, overjet malocclusions, and atrophy of buccal mucosa with widespread white macules. Results of laboratory tests were within normal limits. The proposita's parents (family 1) are Jews of Kurdish origin and first cousins. She is the only one affected among five siblings. Family 1 is related to a second family (family 2) through a common great-grandfather. The parents of family 2 are first cousins and they have three affected children. An autosomal recessive mode of inheritance of Kindler syndrome is suggested in these two related families.
SUMMARY
Forty‐five patients aged 3 to 22 who have handicapping conditions were examined to determine their oral hygiene leveis and periodontal treatment needs. This study shows that oral hygiene leveis for males and the older population were higher. Periodontal treatment needs were highly correlated to oral hygiene, and were much higher for males and older age groups.
Various aspects were discussed and it was concluded that primary prevention is an immediate, although a partial solution. However, new aids and antibacterial solutions should be further investigated in this population to simplify plaque removal.
Our data indicate a dense distribution of dental needs preceding the ablative conditioning regimen for HSCT. These facts accentuate the vital need for cooperation between hospital dentists and treating physicians.
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