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.

ORPHA:330061

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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