Prognostic factors of paraneoplastic pemphigus.
OBJECTIVE
To identify the prognostic factors of overall survival in a series of patients with paraneoplastic pemphigus (PNP).


METHODS
Multicenter retrospective cohort study.


METHODS
Twenty-seven dermatology departments in France.


METHODS
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.


METHODS
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).


CONCLUSIONS
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.
Departments 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.
Sandy
S
Leger
Damien
D
Picard
Saskia
S
Ingen-Housz-Oro
Jean-Philippe
JP
Arnault
François
F
Aubin
Francis
F
Carsuzaa
Gilles
G
Chaumentin
Jacqueline
J
Chevrant-Breton
Olivier
O
Chosidow
Béatrice
B
Crickx
Michel
M
D'incan
Michel
M
Dandurand
Stéphane
S
Debarbieux
Emmanuel
E
Delaporte
Olivier
O
Dereure
Marie-Sylvie
MS
Doutre
Gérard
G
Guillet
Denis
D
Jullien
Ingrid
I
Kupfer
Jean-Philippe
JP
Lacour
Fabienne
F
Leonard
Catherine
C
Lok
Laurent
L
Machet
Ludovic
L
Martin
Carle
C
Paul
Jean-Michel
JM
Pignon
Caroline
C
Robert
Luc
L
Thomas
Pierre-Jean
PJ
Weiller
Vincent
V
Ferranti
Danièle
D
Gilbert
Philippe
P
Courville
Estelle
E
Houivet
Jacques
J
Benichou
Pascal
P
Joly
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use; D001323:Autoantibodies / Q000097:blood; D002352:Carrier Proteins / Q000276:immunology; D003598:Cytoskeletal Proteins / Q000276:immunology; D051180:Desmoplakins / Q000276:immunology; D000071821:Dystonin; D004892:Erythema Multiforme / Q000473:pathology*; D005260:Female; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D053208:Kaplan-Meier Estimate; D008297:Male; D008565:Membrane Proteins / Q000276:immunology; D008875:Middle Aged; D009092:Mucous Membrane / Q000473:pathology; D015999:Multivariate Analysis; D009369:Neoplasms / Q000150:complications*; D009419:Nerve Tissue Proteins / Q000276:immunology; D010257:Paraneoplastic Syndromes / Q000188:drug therapy / Q000276:immunology / Q000473:pathology*; D010392:Pemphigus / Q000188:drug therapy / Q000276:immunology / Q000473:pathology*; D051179:Plakins / Q000276:immunology; D011379:Prognosis; D016016:Proportional Hazards Models; D011498:Protein Precursors / Q000276:immunology; D012189:Retrospective Studies; D000069283:Rituximab; D012720:Severity of Illness Index
D000305:Adrenal Cortex Hormones; D058846:Antibodies, Monoclonal, Murine-Derived; D001323:Autoantibodies; D002352:Carrier Proteins; D003598:Cytoskeletal Proteins; C486268:DST protein, human; D051180:Desmoplakins; D000071821:Dystonin; D007155:Immunologic Factors; D007166:Immunosuppressive Agents; D008565:Membrane Proteins; D009419:Nerve Tissue Proteins; C491674:PPL protein, human; D051179:Plakins; D011498:Protein Precursors; C100754:envoplakin; D000069283:Rituximab