Palmoplantar keratoderma with scleroatrophy of the extremities (Q84896)

A rare autosomal dominant disorder characterised by atrophy and fibrosis of the skin of the limbs, nail hypoplasia and palmoplantar keratoderma. Aggressive squamous cell carcinoma of affected skin occurs in about 1 in 6 affected individuals, mostly in the third to fourth decade, with early metastasis formation. There is also an increased risk of bowel cancer.