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Updated: May 20, 2026
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Published on: June 17, 2025
Prognostic factors of paraneoplastic pemphigus
Sandy Leger1, Damien Picard, Saskia Ingen-Housz-Oro
1Departments of Dermatology, Institut National de la Santé et de la Recherche Médicale (INSERM) U905, Institute for Research and Innovation in Biomedicine, Rouen University Hospital, University of Normandy, Rouen, France.
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.
