title: Stage IV cutaneous acute graft-versus-host disease. Clinical and histological study of 15 cases.
abstract: BACKGROUND
  The most severe form of cutaneous acute graft-versus-host disease (aGVHD), stage IV, is characterized by the appearance of vesicles and blisters.
  
  
  OBJECTIVE
  To describe the clinicopathological characteristics and evolution of stage IV cutaneous aGVHD presented in our hospital.
  
  
  METHODS
  Retrospective study. The following criteria for inclusion were applied: (i) patients subjected to allogeneic stem cell transplantation between 1st January 1984 and 31st of December 2006; (ii) development of vesicles and/or blisters; (iii) extracutaneous coincidental aGVHD manifestations; and (iv) presence of histopathological features consistent with aGVHD.
  
  
  RESULTS
  Fifteen cases (10 females and 5 males) were studied. The mean age was 38.1 years. The lesions appeared after a median interval of 19 days, always following a milder stage of GVHD. Two patterns of clinical evolution were found. Mucosal involvement was observed in nine patients. Nikolsky's sign was positive in eight patients. Nine of the patients had biopsies of the vesiculobullous stage which showed a subepidermal blister with epidermal necrosis and basal vacuolar degeneration. Only two patients survived.
  
  
  CONCLUSIONS
  Stage IV cutaneous aGVHD is a severe and unusual complication after haematopoietic stem cell transplantation. Prognosis is poor with a very high mortality rate, although the cause of death is varied and not strictly linked to the cutaneous disease.
authors.affiliation: Department of Dermatology, Hospital Universitario La Princesa, Madrid, Spain. rgoiriz@yahoo.es
authors.forename: R
authors.initials: R
authors.lastname: Goiriz
authors.forename: Pf
authors.initials: P
authors.lastname: Peñas
authors.forename: S
authors.initials: S
authors.lastname: Pérez-Gala
authors.forename: Y
authors.initials: Y
authors.lastname: Delgado-Jiménez
authors.forename: M
authors.initials: M
authors.lastname: Aragüés
authors.forename: A
authors.initials: A
authors.lastname: García-Diez
authors.forename: J
authors.initials: J
authors.lastname: Fraga
authors.forename: A
authors.initials: A
authors.lastname: Figuera
authors.forename: J
authors.initials: J
authors.lastname: Fernández-Herrera
mesh_terms: D000208:Acute Disease; D000328:Adult; D005260:Female; D006086:Graft vs Host Disease / Q000473:pathology*; D006801:Humans; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies