Prognostic Factors of Paraneoplastic Pemphigus. - Université de Rennes Accéder directement au contenu
Article Dans Une Revue Archives of Dermatology -Chicago- Année : 2012

Prognostic Factors of Paraneoplastic Pemphigus.

Saskia Ingen-Housz-Oro
  • Fonction : Auteur
Jean-Philippe Arnault
  • Fonction : Auteur
Francis Carsuzaa
  • Fonction : Auteur
Gilles Chaumentin
  • Fonction : Auteur
Jacqueline Chevrant-Breton
  • Fonction : Auteur
Olivier Chosidow
  • Fonction : Auteur
Béatrice Crickx
  • Fonction : Auteur
Michel Dandurand
  • Fonction : Auteur
Stéphane Debarbieux
  • Fonction : Auteur
Emmanuel Delaporte
  • Fonction : Auteur
Olivier Dereure
Marie-Sylvie Doutre
  • Fonction : Auteur
Gérard Guillet
  • Fonction : Auteur
Ingrid Kupfer
  • Fonction : Auteur
Jean-Philippe Lacour
Fabienne Leonard
  • Fonction : Auteur
Catherine Lok
  • Fonction : Auteur
Laurent Machet
Ludovic Martin
Carle F Paul
Jean-Michel Pignon
  • Fonction : Auteur
Caroline Robert
Pierre-Jean Weiller
  • Fonction : Auteur
Vincent Ferranti
  • Fonction : Auteur
Philippe Courville
  • Fonction : Auteur
Estelle Houivet
  • Fonction : Auteur

Résumé

OBJECTIVE To identify the prognostic factors of overall survival in a series of patients with paraneoplastic pemphigus (PNP). DESIGN Multicenter retrospective cohort study. SETTING Twenty-seven dermatology departments in France. PATIENTS A total of 53 patients (31 men and 22 women; median age, 59 years; age range, 30-88 years) were diagnosed as having PNP between 1992 and 2010. MAIN OUTCOME MEASURES Overall Kaplan-Meier survival rates were estimated, and features associated with survival were assessed using univariate (log-rank test) and multivariate (Cox regression) analyses. RESULTS The study included 53 patients with PNP. Thirty-six patients (68%) died during the study. The 1-, 3-, and 5-year overall survival rates were 49%, 41%, and 38%, respectively. The main causes of death were infections (n = 21) and evolution of neoplasia (n = 6). In univariate analysis, the main detrimental prognostic factors identified were erythema multiforme-like skin lesions (P = .05) and histologic keratinocyte necrosis (P = .03). None of the 5 patients with Castleman disease died during the study. After adjustment for age and sex in multivariate analysis, erythema multiforme-like skin lesions remained predictive of fatal outcome, with a 2-fold increase in death rate (hazard ratio [HR], 2.3; 95% CI, 1.05-5.03; P = .04). The prognosis of patients with PNP was even poorer when erythema multiforme-like skin lesions were associated with severe skin or mucosal involvement at presentation (HR of death, 3.0; 95% CI, 1.01-8.92; P = .049). CONCLUSION Patients with PNP with erythema multiforme-like skin lesions and histologic keratinocyte necrosis, especially when associated with extensive lesions at presentation, are likely to have a more severe and rapid fatal outcome and should be managed very carefully.

Domaines

Cancer
Fichier non déposé

Dates et versions

hal-00727669 , version 1 (04-09-2012)

Identifiants

Citer

Sandy Leger, Damien Picard, Saskia Ingen-Housz-Oro, Jean-Philippe Arnault, François Aubin, et al.. Prognostic Factors of Paraneoplastic Pemphigus.. Archives of Dermatology -Chicago-, 2012, pp.1-8. ⟨10.1001/archdermatol.2012.1830⟩. ⟨hal-00727669⟩
1014 Consultations
0 Téléchargements

Altmetric

Partager

Gmail Facebook X LinkedIn More