Hereditary sclerosing poikiloderma

J Korean Med Sci. 2012 Feb;27(2):225-7. doi: 10.3346/jkms.2012.27.2.225. Epub 2012 Jan 27.


Hereditary sclerosing poikiloderma (HSP) is a very rare disease. The clinical features are principally widespread poikiloderma and linear hyperkeratotic and sclerotic bands. We report an 18-yr-old male who presented reticular hyperpigmented lesions on the trunk and extremities since 2-yr-old. Also, linear sclerosing bands appeared on both antecubital and popliteal fossae after yr. Histopathologic finding showed dense sclerotic collagen fibers with telangiectasia in the upper dermis and fragmentations of damaged elastic fibers in the elastic stain, consistent with HSP. We report the first Korean case of HSP.

Keywords: Hereditary Sclerosing Poikiloderma; Korean.

Publication types

  • Case Reports

MeSH terms

  • Abnormalities, Multiple
  • Adolescent
  • Elastic Tissue / pathology
  • Fingers / abnormalities
  • Humans
  • Hyperpigmentation / pathology
  • Male
  • Micrognathism / pathology
  • Rothmund-Thomson Syndrome / diagnosis*
  • Rothmund-Thomson Syndrome / pathology
  • Sclerosis / pathology
  • Skin Diseases / diagnosis
  • Skin Diseases / pathology