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Acute generalized exanthematous pustulosis

Disease definition

A rare toxic dermatosis disease characterized by the rapid development of numerous, nonfollicular, sterile, pinhead-sized pustules on an edematous and erythematous base, predominantly occurring on the trunk, intertriginous and flexural areas, with rare, mostly oral, mucosal involvement. Acute onset of fever (>38°C), peripheral blood leukocytosis, and mild eosinophilia are accompanying features. Systemic involvement, with hepatic, renal or pulmonary dysfunction, occasionally occurs. Histologically reveals characteristic spongiform, subcorneal and/or intraepidermal, pustules, as well as marked edema of the papillary dermis, a perivascularly accentuated, neutrophil-rich inflammatory infiltrate, and intrapustular or intradermal eosinophils.

ORPHA:293173

Classification level: Disorder
  • Synonym(s):
    • AGEP
    • Pustular drug eruption
    • Toxic pustuloderma
  • Prevalence: Unknown
  • Inheritance: Multigenic/multifactorial or Not applicable 
  • Age of onset: All ages
  • ICD-10: -
  • OMIM: -
  • UMLS: C0853331  C0877055
  • MeSH: -
  • GARD: -
  • MedDRA: 10048799

Additional information

Further information on this disease

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