'Faculty of Medicine, Universiti Kebangsaan Malaysia'
Abstract
Introduction: Palmoplantar keratoderma (PPK) is characterised by excessive thickening of the palms and soles. Hereditary PPK is rare with prevalence in Asia estimated at 1 to 3 per 10,000. A careful assessment is needed due to this group of diseases' considerable clinical and genetic variation. Case report: We report a 3-year and 6-month-old girl who presented with skin thickening for two years. She has no extracutaneous symptoms. Her father and elder brother have similar
dermatological manifestations. On examination, the child appears well, with no syndromic features. Her skin over both palms and soles is dry, coarse, hyperkeratotic, with trans gradient area involving web spaces of feet. There is no
pseudo ainhum. The diagnosis of PPK is based on clinical findings. Laboratory investigations are not done. She was prescribed urea 10% LA cream BD and Vaseline
as emollient LA QID. The skin thickening subsequently reduced within months. Conclusion: Hereditary PPK is a challenging condition to diagnose because it is uncommon, with a variety of presentations and classifications. More importantly,
PPK can be associated with many other conditions and could be a feature of other syndromes, which the unknowing physician could miss. A thorough history and careful physical examination are important since patients with syndromic PPK will require a multidisciplinary approach as part of its management. The patient and her parents may be anxious about how the disease progresses. Ensuring life-long
compliance with the medication is crucial as recurrence is common after discontinuing treatment