| Objective:Paraneoplastic pemphigus(PNP)is a unique autoimmune blister disease in pemphigus.Clinically,it is relatively rare,the pathogenesis is not clear,there is no unified diagnostic criteria,and the efficacy of drugs is different,and the prognosis is poor.By reporting seven cases of PNP,and searching the reports of PNP at home and abroad in recent years for analysis,in order to improve the understanding of doctors on PNP,improve the level of diagnosis and treatment,and reduce the rate of misdiagnosis and missed diagnosis.Methods:The clinical data of seven cases of PNP were reported in detail,and keywords such as "paraneoplastic pemphigus" were searched in the databases of CNKI and Pub Med,to collect relatively complete medical history data for the diagnosis of PNP,and to explore the clinical features and treatment methods of PNP.Results:1.Case data Among the seven cases,4 cases were male(57.14%),3 cases were female(42.86%),and male: female≈1.33.Patients ranged in age from 41 to69,with an average age of(54.29±9.81)years.Four patients(57.14%)had significant weight loss.Oral mucosa(100%),lip redness(57.14%),eyes(57.14%),trunk(57.14%),limbs(42.86%),vulva(42.86%)were mostly involved,and most cases(85.71%)started from oral mucosa.Another case(case 1)also involved oral mucosa during the disease development.The skin of oral cavity mucous membrane shows for erosion,ulcer or vesicle more,accompany apparent ache.The dermis of the skin shows relatively diversiform,if erythema,papule,vesicle,erosion,ulcer to wait for a manifestation.The main histopathological features of the cases were necrosis of keratinocytes(100 %),superficial dermal inflammatory cell infiltration(100%)and release of the spinous layer(85.71%).In some cases,interface changes were also observed.Direct immunofluorescence and/or complement C3 spine can be found that Ig G network between cells and/or take deposits,basement membrane and mesh between complement C3 spine cell deposition respectively Ig G spines network between cells(case1),Ig G deposition(case6),mesh between complement C3 spine cell deposition and complement C3 basement membrane(case5),Ig G with linear sedimentary basement membrane zone(case2),Ig G linear deposition(case7),and Ig G,complement C3 negative(case3?case 4?case 7).Potential tumors were found in 6 cases(85.71%),and the accompanying tumor type could not be determined in case6 due to the failure of biopsy.All patients were treated with glucocorticoids.Surgical resection(57.14%)was the main treatment for the tumor.However,due to different tumor types,combined with radiotherapy or chemotherapy alone was chosen,and surgery was not performed in some cases due to unstable conditions(case 3).In both cases 3 and 5,there were latent thymomas,and in case 5,myasthenia appeared.The most common complication was pulmonary infection(57.14%),Three patients improved and were discharged and are still being followed up.One died and three were lost to follow-up.2.Literature system analysis2.1 General situation: Among the 193 patients with PNP,108 were male(56.00%),85 were female(44.00%),male:female≈1.27,male was more than female,and the age range was 7-88 years.The average age was(47.04±18.86)years,in which the age range of male cases was 13-83 years,the average age was(48.83±17.18)years,and the age range of female cases was 7-88 years,with the average age(44.77±20.68)years.Among the 53 patients with PNP in China,31 patients were male(58.49%)and 22 patients were female(41.51%).Male:female ≈1.41,age range 11 to 76 years,mean age(40.40 ± 16.17).The male cases ranged from 13 to 69 years old,with an average age of(41.00±15.28)years,the female cases ranged from 11 to 76 years old,with an average age of(39.55±17.68)years.Among the 140 foreign patients with PNP,77 were male(55.00%)and 63 were female(45.00%),with male:female ≈1.22.The 140 cases ranged in age from 7 to 88 years,with an average age of(49.56±19.24)years,in which the age range of male cases is 15-83 years,the average age is(51.99 ±16.98)years,the age range of female cases is 7-88 years,and the average age is(46.59 ± 21.46)years.The age frequency distribution of 193 cases is mainly concentrated in the age range of 30-70 years(71.50%),the age frequency distribution of domestic cases is mainly concentrated in the age range of 30-60years(64.15%),and the frequency distribution of the age frequency of foreign cases is mainly concentrated in the age range of 30-70 years Aged(71.43%).The age frequency distribution of the three is similar.2.2 Clinical manifestations: The main affected areas of mucosa in 193 patients with PNP are oral cavity,tongue,lips,eyes,nose,vulva,and perianal area,which are mostly characterized by erosion,ulcers,vesicle,scabies,lichen planus,and conjunctival hyperemia(Eyes),the skin is mainly the head,face,trunk and limbs,and most of them are polymorphic erythema,vesicle,bullae,lichen planus,erosion.2.3 Histopathology: The histopathological manifestations of 193 patients with PNP were mainly spinous layer loosening,interfacial dermatitis,and superficial dermal inflammatory cell infiltration.The direct immunofluorescence-positive result pattern mainly includes:(1)Ig G and/or complement C3 deposits are found between spinous cells,(2)Ig G and/or complement C3 deposits are found in the basement membrane area,and(3)the two are mixed.Indirect immunofluorescence methods mostly use mouse bladder or monkey esophagus as the substrate,and the positive result is Ig G deposition between spinous cells.IIF test results are rarely recorded in domestic cases,and the positive response in foreign cases is about 53.57%(n=75).2.4 Potential tumors: A total of 26 tumors were found in 193 patients with PNP,some of whom had multiple tumors,5 patients had suspicious tumors but no pathological examination,and 1 patient had no tumor.Castleman’s disease(50.82%)and thymoma(11.48%)were the most common underlying tumors in China.Non-hodgkin’s lymphoma(27.86%)and Castleman’s disease(23.57%)were the most common potential tumors in foreign patients.The common underlying tumors in the total cases were Castleman’s disease(31.84%),non-hodg kin’s lymphoma(20.90%),follicular dendritic cell sarcoma(9.45%),and chronic lymphocytic leukemia(7.96%).2.5 Treatment: Of the 193 cases,178(92.23%)included hormonal treatment in the treatment plan,and 15 cases did not mention the use of hormones.Seven cases were given high-dose glucocorticoids.For patients with PNP accompanied by Castleman disease,thymoma,follicular dendritic cell sarcoma,more surgery,hormone therapy,combined with human immunoglobulin,cyclophosphamide and other treatments Methods,most patients improved and were discharged.There were 55 cases based on surgery combined with hormone-based treatment,and the total effective rate was 70.91%,including 33 cases in the domestic group,24 cases improved and discharged,and the effective rate was 72.73%,22 cases in the foreign group,15 cases improved and discharged.The efficiency is 68.18%.For PNP associated with non-solid tumors,treatment with hormones combined with immunosup-pressants,radiotherapy and chemotherapy,etc,the prognosis is poor.2.6 Mortality and causes of death: of the 193 cases,80(41.45%)were recorded,of which 75 were recorded as causes of death,and 5 were not mentioned as causes of death.The major causes of death were respiratory failure(46.67%),secondary infection(25.33%),tumor progression(14.67%),and multiple organ failure(9.33%).Conclusion:1.PNP is more common in 30-70 years old,with a slightly higher incidence in men than in women.2.Important clinical clues for the diagnosis of PNP include: refractory stomatitis with lip red thick blood clots,pleomorphic skin lesions,Necrotic keratinocytes in the epidermis,IIF positive with mouse bladder epithelium as substrate,and potential tumors(especially lymphoplastic tumors).3.The most common tumor associated with PNP patients in China is Castleman’s disease.Chest and abdomen CT examinations of suspected PNP patients in China help to quickly screen for tumors,and early surgery may benefit from this type of PNP patients.4.Glucocorticoids combined with immunosuppressive agents,chemoradiotherapy,surgery,etc.can be used as common treatment options for PNP.For refractory PNP,try combined biologic treatment.5.The death rate of PNP combined with BO is extremely high,and the standardized use of human immunoglobulin can reduce the occurrence of BO after surgery.Azithromycin can be used as a treatment option for BO.6.The prognosis of PNP is poor.Common causes of death include respiratory failure and secondary infection.Early surgery and vigilance of secondary infection can improve the prognosis. |
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