Conditions / Skin

Rothmund-Thomson syndrome

info · Skin · ICD-10: Q82.8

A skin disease characterized by poikiloderma, congenital bone defects, and an increased risk of osteosarcoma in childhood and skin cancer later in life that has_material_basis_in homozygous or compound heterozygous mutation in the DNA helicase gene RECQL4 on c

A skin disease characterized by poikiloderma, congenital bone defects, and an increased risk of osteosarcoma in childhood and skin cancer later in life that has_material_basis_in homozygous or compound heterozygous mutation in the DNA helicase gene RECQL4 on chromosome 8q24.

Signs and symptoms

  • Epicanthus
  • Delayed eruption of teeth
  • Short stature
  • Hypertelorism
  • Microphthalmia
  • Hypoplasia of teeth
  • High palate
  • Poikiloderma
  • Prominent antihelix
  • Narrow palpebral fissure

Also known as: Congenital poikiloderma; RTS