title: Pemphigus herpetiformis: a case series and review of the literature.
abstract: BACKGROUND
  Pemphigus herpetiformis (PH) is a rare subtype of pemphigus that presents challenges in diagnosis.
  
  
  OBJECTIVE
  To review the presentation, diagnosis, and management of PH.
  
  
  METHODS
  We reviewed the charts of all patients diagnosed and treated for PH in an immunobullous referral center between September 2007 and June 2013.
  
  
  RESULTS
  Eight patients were identified with a diagnosis of PH. All presented initially with pruritus. Clinical disease was manifest as either urticated erythematous plaques or a vesiculobullous eruption. Histological evaluation demonstrated eosinophilic spongiosis in all patients with acantholysis in half of cases (n = 4/8). Peripheral eosinophilia was noted in three of eight (37.5%) patients. In all cases, direct immunofluorescence showed intercellular deposition of immunoglobulin G in the epidermis. All patients required high-dose corticosteroid initially. All patients treated with dapsone or sulfasalazine (n = 4) achieved at least partial control. Other effective treatments included intravenous immunoglobulin (n = 2), azathioprine (n = 2), and leflunomide (n = 1). Rituximab was ineffective in two patients.
  
  
  CONCLUSIONS
  The clinical and histological features of PH develop over time and with treatment, making distinction between pemphigus subtypes challenging and delay in diagnosis common. Diagnosis of PH requires a high index of suspicion and is made on clinical grounds (urticated erythema) in the context of compatible histology and immunofluorescence findings. Treatment may be challenging, although efficacy of sulfonamide derivatives appears to offer a therapeutic effect.
authors.affiliation: Department of Medicine, University of Toronto, Toronto, Ontario, Canada.
authors.forename: Philip M
authors.initials: PM
authors.lastname: Laws
authors.affiliation: Department of Medicine, University of Toronto, Toronto, Ontario, Canada.
authors.forename: Kara
authors.initials: K
authors.lastname: Heelan
authors.affiliation: Department of Medicine, University of Toronto, Toronto, Ontario, Canada.
authors.forename: Faisal
authors.initials: F
authors.lastname: Al-Mohammedi
authors.affiliation: Department of Medicine, University of Toronto, Toronto, Ontario, Canada.
authors.forename: Scott
authors.initials: S
authors.lastname: Walsh
authors.affiliation: Department of Medicine, University of Toronto, Toronto, Ontario, Canada.
authors.forename: Neil H
authors.initials: NH
authors.lastname: Shear
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D017677:Age Distribution; D000368:Aged; D001707:Biopsy, Needle; D003874:Dermatitis Herpetiformis / Q000175:diagnosis / Q000188:drug therapy* / Q000453:epidemiology / Q000473:pathology*; D003937:Diagnosis, Differential; D005260:Female; D005455:Fluorescent Antibody Technique; D006801:Humans; D007150:Immunohistochemistry; D015994:Incidence; D008297:Male; D008875:Middle Aged; D010392:Pemphigus / Q000175:diagnosis / Q000188:drug therapy* / Q000453:epidemiology / Q000473:pathology*; D011379:Prognosis; D035583:Rare Diseases; D012189:Retrospective Studies; D018570:Risk Assessment; D012494:Sampling Studies; D012720:Severity of Illness Index; D017678:Sex Distribution; D016896:Treatment Outcome
chemical_list: D000305:Adrenal Cortex Hormones