ABSTRACT
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 multiformelike 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 multiformelike 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 multiformelike 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 multiformelike 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.
Subject(s)
Erythema Multiforme/pathology , Neoplasms/complications , Paraneoplastic Syndromes/pathology , Pemphigus/pathology , Adrenal Cortex Hormones/therapeutic use , Adult , Aged , Aged, 80 and over , Antibodies, Monoclonal, Murine-Derived/therapeutic use , Autoantibodies/blood , Carrier Proteins/immunology , Cytoskeletal Proteins/immunology , Desmoplakins/immunology , Dystonin , Female , Humans , Immunologic Factors/therapeutic use , Immunosuppressive Agents/therapeutic use , Kaplan-Meier Estimate , Male , Membrane Proteins/immunology , Middle Aged , Mucous Membrane/pathology , Multivariate Analysis , Nerve Tissue Proteins/immunology , Paraneoplastic Syndromes/drug therapy , Paraneoplastic Syndromes/immunology , Pemphigus/drug therapy , Pemphigus/immunology , Plakins/immunology , Prognosis , Proportional Hazards Models , Protein Precursors/immunology , Retrospective Studies , Rituximab , Severity of Illness IndexABSTRACT
We report a case of Schistosoma mansoni bilharziasis in a HIV patient 38 y after leaving an endemic region. A viable S. mansoni egg on a liver biopsy sample was diagnostic. Despite severe concomitant immunodeficiency the effective treatment was a single dose of praziquantel. Worms' intravascular longevity and the role of immunodeficiency as a possible factor in eggs retention after such prolonged latent schistosomiasis are discussed.
