Paraneoplastic Keratoderma — SCE Dermatology MCQ

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HardSkin CancerParaneoplastic KeratodermaSCE Dermatology

A 48-year-old man has palmoplantar keratoderma that developed in adult life. He also has oesophageal cancer. What is this paraneoplastic association called?

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Correct answer: E — Tylosis (Howel-Evans syndrome)

Tylosis (Howel-Evans syndrome) is a rare autosomal dominant condition characterised by diffuse palmoplantar keratoderma associated with oesophageal carcinoma. The RHBDF2 (iRHOM2) gene on chromosome 17q25 is the causative mutation. Keratoderma typically presents in childhood/adolescence, with oesophageal cancer risk increasing from age 40 (lifetime risk approaching 95% in affected families). Endoscopic surveillance is recommended from age 20-30 in affected families. Bazex syndrome (acrokeratosis paraneoplastica) is a different paraneoplastic keratoderma affecting the acral sites (ears, nose, fingers, toes) associated with upper aerodigestive tract SCC.

Reference: BAD 2019; BJD Hereditary Keratoderma