Orphanet: Actinic prurigo

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Actinic prurigo

Disease definition

A rare, chronic, photodermatosis disease characterized by intensely pruritic, polymorphic, erythematous, excoriated and/or lichenified papules, macules, plaques and nodules, occurring on sun-exposed areas of the skin (particularly face, nose, lips, and ears), frequently associating cheilitis (especially of the lower lip) and conjuctivitis, which are present year-round or only in the spring/summer (depending on geographic location), observed mainly in Native Americans and Mestizos. Cheilitis may be the sole clinical presentation. Histologically, the presence of lymphoid follicles in mucosa is pathognomonic.


Classification level: Disorder
  • Synonym(s):
    • Familial polymorphous light eruption of American Indians
    • Hereditary polymorphous light eruption of American Indians
    • Hutchinson summer prurigo
    • Hydroa aestivale
  • Prevalence: Unknown
  • Inheritance: Multigenic/multifactorial or Not applicable 
  • Age of onset: Childhood
  • ICD-10: L56.4
  • OMIM: 174770
  • UMLS: C0406217
  • MeSH: -
  • GARD: -
  • MedDRA: -
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