A rare presentation of psychotic depression with suicidality in a case of papillon-lefèvre syndrome

Abstract

Papillon-Lefèvre syndrome (PLS) is an autosomal recessive disorder that presents with palmoplantar hyperkeratosis and childhood-onset progressive loss of all dentition. Mental retardation is the only neurodevelopmental disorder reported with this condition till date. We report the first ever case in the literature of PLS presenting with psychotic depression and suicidal intention. A 40-year-old, never married, unemployed woman presented for psychiatric consultation and was given an International Classification of Diseases version 10 diagnosis of severe depression with psychotic symptoms. Physical examination warranted dermatological and dental evaluation before electroconvulsive therapy (ECT) could be administered. She was diagnosed with PLS and pseudoainhum by the skin and dental specialists. Karyotyping study was normal, and histopathology of the palmar tissue showed hyperkeratinization. She was treated with ECT, duloxetine and olanzapine, and she achieved full remission of her depression. She was prescribed oral retinoids and emollients for the skin disorder, and there was a good improvement. The dental prosthesis was fixed, and she was able to eat and feel better than before. Early diagnosis of this condition and rehabilitation would be important in improving wellbeing.