The Unilateral Focal Dermal Hypoplasia - A Case Report and Literature Review

Introduction: Focal dermal hypoplasia (FDH), formerly referred to as Goltz syndrome, is a rare multisystem condition characterised by an atypical genodermatosis which involves tissues derived from both ectoderm and mesoderm. It also affects various other tissues and organs, with the greatest impact on the connective tissue of the bones, skin, eyes, and oral cavity. Case presentation: A 7 - year - old female patient presented with a history of FDH exhibited a diverse range of systemic and oral manifestations. Extra and Intraoral examination showed Cutaneous changes along with facial asymmetry and few other oral manifestations including increase in incidence of caries and radiographic interpretation shows congenitally missing permanent teeth. Conclusion: This paper will enlighten the distinctive general and oral observations which will help the pediatric dentist to understand this rare clinical anomaly, formulating plans, and providing preventive and rehabilitative management.