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XERODERMA PIGMENTOSUM

  • Xeroderma pigmentosum (XP) was first described in 1874 by Hebra and Kaposi.
  • In 1882, Kaposi coined the term xeroderma pigmentosum for the condition, referring to its characteristic dry, pigmented skin.
  • XP is a rare disorder transmitted in an autosomal recessive manner. It is characterized by photosensitivity, pigmentary changes, premature skin aging, and malignant tumor development.
  • These manifestations are due to a cellular hypersensitivity to ultraviolet (UV) radiation resulting from a defect in DNA repair.

 

  • The skin is healthy at birth. Typically, the first stage makes its appearance after the age of 6 months. This stage is characterized by diffuse erythema, scaling, and frecklelike areas of increased pigmentation . These findings, as would be expected from the pathophysiologic basis for the disease, are seen over light-exposed areas, appearing initially on the face. With progression of the disease, the skin changes appear on the lower legs, the neck, and even the trunk in extreme cases. While these features tend to diminish during the winter months with decreased sun exposure, as time passes, these findings become permanent.

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