Punctate Palmoplantar Keratoderma Type 1 (PPPK1) Associated with Pancreatic Intraductal Papillary Mucinous Neoplasm...

Punctate palmoplantar keratoderma type 1 (PPPK1), known as Buschke-Fischer-Brauer syndrome, is an autosomal dominant disorder characterized by palmoplantar hyperkeratotic papules. While an association has been proposed between PPPK1 and malignant tumors, it remains controversial and is supported only by limited case reports, including rare associations with pancreatic cancer. To date, no association has been reported with precancerous pancreatic tumors including intraductal papillary mucinous neoplasm (IPMN). We present a 67-year-old male presenting with long-standing palmoplantar hyperkeratotic papules. PPPK1 was diagnosed clinically based on the morphology of skin lesions, chronic progressive course, and positive family history of similar findings. Review of the patient's medical records revealed a previously diagnosed pancreatic IPMN. Examination revealed symmetrical hyperkeratotic papules coalescing into plaques over the soles, involving pressure-bearing areas and toes, and multiple discrete punctate hyperkeratotic papules on the palms. History and examination supported the diagnosis of PPPK1. The patient was previously diagnosed with pancreatic IPMN and was following at another tertiary hospital for abdominal imaging. Topical salicylic acid 10% in paraffin was started; however, the patient was lost to follow-up, and the treatment response could not be evaluated. This case highlights an unreported observational association between PPPK1 and pancreatic IPMN. Although a definite relationship cannot be confirmed, this finding adds to the limited literature on pancreatic neoplasms associated with PPPK1. Further studies are needed to confirm this observational association and to determine whether systemic evaluation is routinely required in these patients.