Three Cases Report Of Paraneoplastic Pemphigus And A Review Of The Literature

ObjectivesParaneoplastic pemphigus(PNP)is a autoimmune blistering disease which occurs in association with neoplasms.The clinical manifestations of PNP are different.so it is difficult to make an exact diagnosis.This article aims to analyze the characteristics of the disesase,clinical features.histopathology and immunofluorescence,associated neoplasms,treatment methods and prognosis to improve the overall undestanding of the disease for doctors and it's helpful to make an exact diagnosis and treatment at the beginning of the disease.MethodsThis study retrospective analyzed the clinical data of three patients who had detail data from five patients with paraneoplastic pemphigus in dermatological department of Qilu hospital from 2001 to 2017 to analysis characteristics,clinical manifestations,histopathology and immunofluorescence.diagnosis and treatment.associated tumors,prognosis and follow-up of PNP.We had a literature review from database of CNKI,wanfang and Pubmed at home and aboard,at the same time,analyzed 105 cases that from 69 literatures at home from 1993 to 2016 with detailed clinical data.ResultsThree cases of the patient's condition in our hospital1.General information:In our 3 patients,2 female and 1 male,male to female is 1:2.Ages range from 24 years old to 54 years old,the average age is 37.3 years old.The disease courses range from 40 days to 180 days,with an average of 80 days.2.Clinical manifestations:3 cases of patients had a involvement of oral,genital and conjunctiva.The characteristic manifestation is the multiple oral mucosa painful erosion and ulcers.1 case had a weight loss.3.Histopathology:3 patients were taken the involvement of the skin or mucosal to perform the histopathology examination.The results had the following characteristics:(1)necrotic keratinocytes were scattered or clustered distribution;(2)liquefaction degeneration of basal cell;(3)dermal superficial can see lymphocytes which were infiltrated with band.4.Immunofluorescence:2 patients were examined by direct immunofluorescence(DIF),and IgG and C3 were deposited in the spinous cells.Among 1 of the 2 patients,we can see the basement membrane had C3 deposition.1 patient in another hospital(serum in the bladder epithelium in rats as substrate)was examinated indirect immunofluorescence(IIF),the result showed that IgG was deposited in spinous intercellular,titers of 1:40.5.Associated tumors:2 cases of 3 patients with Castleman disease(CD);the other one with an unknown tumor.2 cases were located in the mediastinum,and the other was located in the pelvic.2 cases were performed histopathology examination after oprations.1 case was hyaline vascular type,and the other was plasma cell type.6.Auxiliary examinations:including imaging examinations and laboratory examinations.All 3 patients had imaging examinations,and found the underlying tumors.Laboratory tests indicated that patients had low serum albumin,the proportion of white balls upside down,but the tumor series were negative.Some patients showed abnormal blood routine,urine routine,stool routine,ESR,rheumatic series and humoral immunity series.7.Treatment:2 patients of 3 were treated with surgical resection of the tumor.1 patient was unable to perform surgery due to the special location of the tumor,and was treated with chemotherapy and radiotherapy in the local hospital.2 patients were treated with small dose of hormone after surgery.8.Prognosis:1 patient who didn't perform a surgery died of respiratory failure after one month;1 case had an operation 1 year ago,and still was treated with small dose of hormone,there was no new blisters in the body,but a little erosion and ulcers in oral mucosa;1 patient was lost.The clinical data of 105 patients of PNP in the literatures were analyzed as follows:1.General information:105 patients,male 48,female 57,the ratio of male to female is 1:1.19.The minimum age is 11 years old,the maximum age is 82 years old and the average age is 36.3 years old.The disease courses range from 15 days to 3 years,with an average of 170 days.2.Clinical manifestations:103 patients had a involvement of oral,genital or bilateral conjunctival with varying degrees.29 cases of patients had a fever;10 cases of patients had a hair loss with different degrees;17 patients had a involvement of finger(toe)nails;weight loss occurred in 28 patients.3.Histopathology:90 patients were performed hispathology examinations of the skin or nmucous membrane.There are characteristics as follows:(1)necrotic red stained keratinocytes;(2)the liquefaction degeneration of basal cells;(3)acantholysis;(4)the infiltration of dermal superficial or perivascular mononuclear cells.4.Immunofluorescence:direct immunofluorescence examinations were performed in 68 patients.There are 2 characteristics as follows:(1)IgG and(or)C3 were distributed in the epidermis cells;(2)IgG and(or)C3 in the basement membrane zone was linear deposited.Indirect immunofluorescence examinations were performed in 64 patients with serum(in rat bladder epithelium,monkey esophagus epithelium or human epidennis).The IgG and(or)C3 deposition in the intercellular membrane and(or)basement membrane zone was observed,with the titers of 1:20 to 1:2560.5.Associated tumors:61 patients of 105 cases with the most frequent tumor were Castleman disease.9 cases were lymphoma,8 cases were thymoma,7 cases were leukemia,4 cases were follicular dendritic cell sarcoma,10 cases were unknown,the other kinds were 10 cases.There were 2 patients with more than two kinds of tumors.30 cases of Castleman tumor were examined by histopathology,including hyaline vascular type in 27 cases,plasma cell type in 2 cases,mixed type in 1 case.54 cases of tumors were located in the retroperitoneum,and the tumor which was located in the mediastinum is 23 cases,and the other in the pelvic cavity was 5 case.6.Auxiliary examinations:including imaging examinations and laboratory examinations.The imaging examinations included CT,abdominal ultrasound,gynecological ultrasound and urinary ultrasound.Laboratory examinations can appear abnormal blood routine,urine routine,stool routine,ESR,C-reactive protein,liver function,blood glucose,blood lipid,serum electrolytes,rheumatic series,humoral immunity,lymphocyte subsets and tumor series.7.Treatment:mainly including treatments of primary tumors,autoimmune diseases and complications.Treatments of primary tumors includes surgery,radiotherapy and chemotherapy.Treatments for autoimmune diseases include corticosteroids,immunosuppressants,Human Immunoglobulin,or plasma.Most patients were treated with combination therapy.8.Prognosis and follow-up:56 patients survived,35 patients died,and the other 14 patients were lost.32 patients were treated with surgery.The majority of patients with benign tumors after treatment of primary tumors,whose rash can be basically or completely subsided,and patients with malignant tumors had a poor prognosis.In some patients,bronchiolitis obliterans(BO)may appeared before or after surgery.The most common cause of death was respiratory failure,followed by infection induced multiple organ failure.Conclusion1.Paraneoplastic pemphigus can occur at any age,especially 30 to 40 years old.The incidence of female is higher than male.2.The clinical manifestations are diverse.The characteristics of the disease are painful mucosal erosions and polymorphic cutaneous lesions.Oral mucosa and conjunctiva are the most frequently involved.In some patients,the oral mucosa erosions is the only symptom.3.Histopathology examinations have some characteristics of the presence of necrotic keratinocytes.acantholysis and the liquefaction degeneration of basal cells.Immunofluorescence shows that IgG and(or)C3 are deposited in the spinous cells and/or basement membranes.4.The most common tumor is lymphoid hyperplasia,and Castelman is the most common tumor in China.5.Treatment:combination therapy is the main treatment,which including the treatments of primary tumors.autoimmune diseases and the complications.6.Prognosis:the prognosis of the disease is poor.The mortality rate is high with malignant tumors,but if with benign neoplasms,the prognosis is better after the turmors are removed.7.The most common compliaction is infection.pain and bronchiolitis obliterans.The most common cause of death is respiratory failure.8.Early diagnosis and early treatment are helpful to prolong the lifetime of patients.