title: Koebnerization of Hailey-Hailey disease into a cutaneous drug eruption of acute generalized exanthematous pustulosis associated with systemic symptoms.
abstract: We describe a 65-year-old Caucasian female with well-controlled Hailey-Hailey disease (HHD) who developed acute generalized exanthematous pustulosis (AGEP) with severe systemic symptoms. Despite sparing of the patient's intertriginous skin, histopathologic evidence of HHD was observed in all biopsies, suggestive of a unique koebernization phenomenon of HHD to areas of cutaneous drug eruption. While internal organ involvement is less commonly reported in AGEP, there are an increasing number of patients with signs and symptoms suggestive of an AGEP/drug reaction with eosinophilia and systemic symptoms (DRESS) spectrum of cutaneous drug disorders. Early diagnosis of patients with AGEP and systemic symptoms is critical so that these patients may receive prompt and aggressive systemic therapy to decrease the risk of end organ damage and improve overall morbidity and mortality.
authors.affiliation: Department of Dermatology, Yale School of Medicine, New Haven, CT, 06520-8059, USA. mariam.totonchy@yale.edu.
authors.forename: Mariam B
authors.initials: MB
authors.lastname: Totonchy
authors.affiliation: Department of Dermatology, Yale School of Medicine, New Haven, CT, 06520-8059, USA.
authors.forename: Jennifer M
authors.initials: JM
authors.lastname: McNiff
authors.affiliation: Department of Dermatology, Yale School of Medicine, New Haven, CT, 06520-8059, USA. Christopher.bunick@yale.edu.
authors.forename: Christopher G
authors.initials: CG
authors.lastname: Bunick
mesh_terms: D056150:Acute Generalized Exanthematous Pustulosis / Q000175:diagnosis* / Q000188:drug therapy; D000368:Aged; D063926:Drug Hypersensitivity Syndrome / Q000175:diagnosis* / Q000188:drug therapy; D042241:Early Diagnosis; D005260:Female; D006801:Humans; D008776:Methylprednisolone Hemisuccinate / Q000008:administration & dosage / Q000627:therapeutic use; D016506:Pemphigus, Benign Familial / Q000150:complications* / Q000188:drug therapy; D011239:Prednisolone / Q000008:administration & dosage / Q000627:therapeutic use; D016896:Treatment Outcome; D014221:Triamcinolone / Q000008:administration & dosage / Q000627:therapeutic use; D014640:Vancomycin / Q000008:administration & dosage
chemical_list: D014221:Triamcinolone; D008776:Methylprednisolone Hemisuccinate; D014640:Vancomycin; D011239:Prednisolone
keywords: Hailey-Hailey disease; acute generalized exanthematous pustulosis; drug hypersensitivity; koebnerization; systemic drug reaction