A Case Of Paraneoplastic Pemphigus With NHL Reported And Domestic And International Literature Analysis

Background and ObjectiveParaneoplastic Pemphigus(PNP)is a relatively rare clinical,autoimmune bullous disease involving the skin and mucous membranes.Often associated with malignant or benign tumors,most of the tumor originated in the lymphatic system,can involve multiple internal organs.In 1990,the first report of the disease abroad,that PNP is an independent disease.80% of PNP patients were associated with non-Hodgkin's lymphoma(NHL),chronic lymphocytic leukemia(CLL)and Castleman's disease.China in 1999 first reported the disease by Professor Zhu Xuejun,and made a series of studies.There have been reports of more than 200 cases of paraneoplastic pemphigus reported in China,accompanied by Castleman's disease,and the paraneoplastic pemphigus with NHL is rare and reported mostly.The etiology of this disease is not very clear,the incidence is low,higher mortality.There is no unified treatment program,mostly to chemotherapy,some cases can be used to remove some or all of the surgery,supplemented by chemotherapy or radiotherapy,the prognosis is poor.The aim of this study was to investigate the clinical features,histopathological and immunological characteristics of patients with non-Hodgkin's lymphoma of the paraneoplastic pemphigus,improve the diagnosis and treatment of the disease and reduce the misdiagnosis rate.Method1.Retrospectively analyzed the clinical data of a case of Paraneoplastic Pemphigus with non-Hodgkin's lymphoma treated by 2015 in our hospital.And search the CNKI,Wanfang database,Pubmed and other databases,enter the "paraneoplastic pemphigus","non-Hodgkin's lymphoma" and other key words,with NHL of paraneoplastic pemphigus related literature Systematic analysis,summed up its clinical characteristics and treatment.2.The data were analyzed by SPSS 22.0 statistical software package.The measurement data were expressed by((?)±s),and the two independent samples were used the t test to compare.The count data were calculated by Fisher exact test method,bilateral P test,When there is statistical significance P <0.05.Results1.Data in this case:(1)General information:the patient was a femal,aged 46,farmer.(2)Clinical manifestations:Conjunctival hyperemia,erosion of the oral mucosa and perineum,conscious pain,a large area of trunk and limbs erythema,moderate fever,temperature up to 38.5?.(3)Imaging examination: retroperitoneal soft tissue mass,consider the lymph node,the proposed puncture biopsy;Mesenteric area,bilateral iliac vascular multiple enlarged lymph nodes;Pelvic effusion;Chronic inflammation of lower lobe of left lung.(4)Skin biopsy: hyperkeratosis(trunk),epidermal water blister like structure,liquifaction degeneration of basal cells,basal layer visible colloide bodies,with infiltration of lymphocytes in the superficial dermis,and pigment incontinence.The lesion was an interface dermatitis with erythema multiforme.(5)Direct immunofluorescence: Ig G and C3 in the intercellular deposition of prickle cells.(6)Retroperitoneal lymph node biopsy and gene rearrangement Tip: non-Hodgkin's lymphoma(low-grade follicular lymphoma,diffuse type).(7)Minimally diagnosed with non-Hodgkin's lymphoma(low-grade follicular lymphoma)of paraneoplastic pemphigus.(8)Treatment:After definite diagnosis,she was transferred to the Department of Oncology and given one cycles of chemotherapy with CHOP regimen.2.A systematic literature analysis results: More than 700 related literatures were retrieved from 1990 to 2016,of which only 37 cases of parapharyngeal pemphigus with NHL were present.The incidence,clinical features,treatment and prognosis of 37 patients were analyzed comprehensively,The results are as follows.2.1 General conditions: All ages of disease onset at the age of 21 to 77,of which 40 to 49 years old and 60 to 79 years old is the onset of the two peak period,occurs in the elderly,the average age is(54.9±16.3)years old,male 13 cases,female 24 cases,male:female?1:1.8.2.2 Incidence: The incidence of domestic and foreign differences vary widely,mainly in Asia(62%)and Europe(24%)and other countries,especially Japan(11 cases),Beijing(3 cases),Hongkong China(2 cases),Shandong(2 cases),Hunan(1 case),Hubei(1 case),Taiwan(1 case)and other regions.2.3 Clinical manifestations: Of the 37 patients,36 cases(97%)had oral mucosal lesions,32 cases(87%)appeared pleomorphic rash,19 cases(51%)had lips erosion,scab,18 cases(49%)Occurred conjunctivitis,palmoplantar erythema,keratosis in 10 cases(27%),genital mucosal erosion in 8 cases(22%).2.4 Among the 37 patients,14 patients were diagnosed with impaired lung injury.Among them,5 cases were diagnosed with bronchiolitis and all died of respiratory failure,the mortality rate is 100%.5 patients were infected and all died,the mortality rate is 100%.2.5 Of the 37 patients,15 cases were misdiagnosed,and were misdiagnosed as erythema multiforme in 3 cases(20%),Steven-Jhon syndrome in 3 cases(20%),roliferative pemphigus in 2 cases(13%),the rest were misdiagnosed as pemphigus vulgaris,lichen planus,Behcet's disease,toxic epidermal necrolysis and lichen planus pemphigoid,blister eczema,cytomegalovirus infection and so on.2.6 The combination of rituximab,plasma exchange,intravenous immunoglobulin and other comprehensive treatment;combined with rituximab chemotherapy group compared with chemotherapy alone group,the former natural survival time was significantly higher In the latter,the difference was statistically significant(P=0.029).There was no statistically significant difference in mortality between the two groups(P=0.696).Conclusions1.With NHL of paraneoplastic pemphigoid occur in the elderly,women more than men.2.With NHL of paraneoplastic pemphigus in China is relatively rare,skin lesions diversified,easy to misdiagnosis.Persistent,severe and extensive mucosal damage is an important clue to the diagnosis of PNP.3.The mortality of patients with bronchiolitis obliterans is very high.4.Rituximab is effective in the treatment of this disease,which can prolong the survival time and improve the prognosis.