Bk syndrome

Synonym(s)

Definition

Syndrome consisting of premature graying of the hair, tooth development abnormalities, and palmoplantar hyperhidrosis

Pathogenesis

Autosomal dominant inheritance; unknown genetic defect

Clinical manifestation

Graying of the hair before age 14 years, mostly in the scalp hair; bicuspid aplasia; hyperhidrosis of the palms and soles

Differential diagnosis

Ectodermal dysplasia; essential hyperhidro-sis

Therapy

None

References

Bôôk JA (1950) Clinical and genetical studies of hypodontia. Premolar aplasia, hyperhidrosis, and canities prematura: A new hereditary syndrome in man. American Journal of Human Genetics 2:240-263

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