Turnpenny et al. (1995) described a four-generation Scottish family with a distinct form of ectodermal dysplasia that predominantly affects the teeth, skin, hair, and skeletal system. Among the manifestations observed in this family were late adolescent hypodontia or oligodontia and also some individuals presenting multiple natal teeth. Some women experienced flexural acanthosis nigricans during childhood and early adolescence. Despite producing sweat, some family members exhibited variable heat tolerance. Additionally, they had thin and slow-growing scalp hair, which often improved in quality during pregnancy. Body hair was scanty, and one subject showed hypoplasia of pilosebaceous units. Hair samples displayed abnormal cuticular appearances, indicating a potential primary keratin structure defect. Nails appeared normal, and there was variability in relative macrocephaly due to cranial vault hyperostosis. Furthermore, short stature (5–10th centile) was noted, potentially representing a separate familial trait(Turnpenny et al. 1995).