We report a rare genetic condition characterized by gingival hyperkeratosis with skin changes affecting the hands and feet. The patient presented with asymptomatic oral lesions and the diagnosis was reached by taking a full clinical history and biopsy tests. Palmoplantar keratosis can be a feature of a range of different syndromes and can be associated with periodontal destruction, benign oral keratosis or cancer of the oesophagus. It is important for dentists to be aware that clues to diagnosis can be gained from examination of the hands and feet, in addition to an oral examination, particularly in recognizing oral genetic disorders.
Clinical relevance: To inform clinicians of both the intra-oral and extra-oral features of focal palmoplantar and gingival hyperkeratosis.