N Dermatol Online. 2010; 1(2): 31
Conflicts of interest: None
BÖÖKS SYNDROME. OBSERVATION FOUR PATIENTS
Brzeziński Piotr1, Płowaś Marta1, Jagiełło-Wieczorek Ewelina2
16th Military Support Unit, Ustka, Poland
2Independent Public Central Clinical Hospital in Warsaw, Poland
Corresponding author: Dr. Piotr Brzezinski e-mail:brzezoo77@yahoo.com
How to cite this article: Brzeziński P, Płowaś M, Jagiełło-Wieczorek E. Zespół Booka. Obserwacja czterech pacjentów. Our Dermatol Online 2010; 1(2): 31.
In 1950 Böök JA described the ectodermal syndrome with impaired dentition (hypodontia of the premolar region), hyperhidrosis of the palms and soles, and premature whitening of the hair. In determining the team abbreviations letters: PHC. Sixty-three persons were submitted to thorough clinical and odontological examinations. The collected evidence indicates that this syndrome is caused by a simple autosomal dominant gene. All persons presented three full syndrome. The symptoms, however, occurred in varying degrees. The penetrance of this gene as concerns hypodontia and early graying is complete, with no difference observed between the sexes. In the observation of the authors is four patients from 22 to 23 years who have the features syndrome Books, namely, hyperhidrosis of the palms or soles and different tooth defects (wide spacing, retardation, abnormal structure). However are needed genetic testing and accurate diagnosis of dental.
REFERENCES:
1. Book JA.: Clinical and Genetical Studies of Hypodontia. I. Premolar Aplasia, Hyperhidrosis, and Canities Prematura;A New Hereditary Syndrome in Man. Am J Hum Genet. 1950; 2: 240-263.
2. Weyers H.: Aplasia of the premolars as a leading symptom of ectodermal dysplasia. Dtsch Zahnarztl Z. 1980; 35: 836-840.
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