title: Letter: Pemphigus herpetiformis of age of onset at 6 years.
abstract: BACKGROUND
  Pemphigus herpetiformis is a rare entity that combines the clinical features of dermatitis herpetiformis with the immunologic and histological features of pemphigus. It generally affects adults and pediatric cases are uncommon. We report a case of pemphigus herpetiformis in a 6-year-old girl treated successfully with dapsone.
  
  
  METHODS
  A 6-year-old girl presented with a pruritic, erythematous and vesiculous eruption. Histological examination showed acantholysis of the medial and superficial parts of the epidermis. Direct immunofluorescence study revealed cell surface immunoglobulin G and C3 deposits and staining on the dermo-epidermal junction. The child was diagnosed as having pemphigus herpetiformis.
  
  
  CONCLUSIONS
  Pemphigus herpetiformis produces clinically a pruritic and atypical eruption that resembles dermatitis herpetiformis, with standard histology and immunofluorescence patterns recalling those of pemphigus. Considered to have a good prognosis, it responds well to dapsone and/or to low dose oral corticosteroids. The age range of onset varies from 31 to 81 years according to studies, without a male or female predilection. This is a report of a 6-year-old girl presenting with pemphigus herpetiformis, successfully treated with dapsone for two years with no relapse.
authors.affiliation: Department of Dermatology, Hôtel Dieu de France, Beyrouth, Lebanon.
authors.forename: Roy
authors.initials: R
authors.lastname: Moutran
authors.forename: Ismael
authors.initials: I
authors.lastname: Maatouk
authors.forename: Farid
authors.initials: F
authors.lastname: Stephan
authors.forename: Eugenie
authors.initials: E
authors.lastname: Halaby
authors.forename: Gerard
authors.initials: G
authors.lastname: Abadjian
authors.forename: Roland
authors.initials: R
authors.lastname: Tomb
mesh_terms: D002648:Child; D003622:Dapsone / Q000627:therapeutic use; D003874:Dermatitis Herpetiformis / Q000175:diagnosis; D003879:Dermatologic Agents / Q000627:therapeutic use; D005260:Female; D006801:Humans; D010392:Pemphigus / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology; D011241:Prednisone / Q000627:therapeutic use; D016896:Treatment Outcome
chemical_list: D003879:Dermatologic Agents; D003622:Dapsone; D011241:Prednisone