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1.
Arch Dermatol ; 148(10): 1165-72, 2012 Oct.
Article in English | MEDLINE | ID: mdl-22801794

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 Index
2.
J Am Acad Dermatol ; 50(2): 203-9, 2004 Feb.
Article in English | MEDLINE | ID: mdl-14726873

ABSTRACT

BACKGROUND: Rare osteoarticular complications occurring after erysipelas have been reported. We describe 9 patients in whom various osteoarticular complications developed during erysipelas. OBJECTIVE: We sought to analyze osteoarticular complications during erysipelas, paying special attention to clinical, bacteriologic, and radiologic data. METHODS: Data were retrospectively recorded from the files of patients seen in 3 dermatologic centers between 1998 and 2000. They included laboratory tests, bacteriologic cultures, radiologic investigations, and treatment modalities and outcome of both erysipelas and osteoarticular complications. RESULTS: We observed 9 patients (7 men and 2 women; mean age 49.6 years) who first presented with typical erysipelas of the lower limb and then osteoarticular complications developed during the course of their disease, always localized to a joint contiguous to the erysipelas plaque. These complications included: relatively benign complications, ie, bursitis (n = 5) or algodystrophy (n = 1), occurring after erysipelas with favorable course; and more severe complications, ie, osteitis (n = 1), arthritis (n = 1), and septic tendinitis (n = 1), occurring after erysipelas characterized by local cutaneous complications (abscess, necrosis). CONCLUSIONS: Osteoarticular complications of erysipelas can be divided into the 2 groups of nonseptic complications (mainly bursitis), which are characterized by a favorable outcome, and septic complications (osteitis, arthritis, tendinitis), which require specific, often prolonged treatment and, sometimes, operation. Their diagnosis is on the basis of clinical and radiologic findings rather than joint aspirations, which are usually not possible through infected skin tissue.


Subject(s)
Arthritis, Infectious/etiology , Bursitis/etiology , Erysipelas/complications , Osteitis/etiology , Tendinopathy/etiology , Adult , Aged , Arthritis, Infectious/diagnosis , Arthritis, Infectious/pathology , Bursitis/diagnosis , Bursitis/pathology , Diagnosis, Differential , Female , Humans , Male , Middle Aged , Osteitis/diagnosis , Osteitis/pathology , Retrospective Studies , Tendinopathy/diagnosis , Tendinopathy/pathology
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