Clinical Analyses Of Familial Chronic Benign Pemphigus In 37 Cases

Background and ObjecktiveFamilial chronic benign pemphigus,also known as Hailey–Hailey disease(HHD),is an autosomal dominant dermatosis characterized by acantholysis of the skin.The pathogenetic gene is related to the mutation of gene ATP2C1 which located in 3q21-22.The disease is characterized by repeated blisters and bullae,which are prone to burst and form erosion.Most commonly involved sites are the neck,axillae,and groins.Sometimes lesions can also appear on scalp.Mucosa involvement is rarely seen.Besides,Nikolsky's sign is often negative.The disease is usually without systemic symptoms.Histopathology shows the formation of a fissure in the basal layer and partial or complete acantholysis in the epidermis,which appears as the collapsed brick wall and direct immunofluorescence is negative.The disease is rare in clinical practice,but sometimes due to the lack of typical clinical manifestations,lack of experience in the clinicians,improper treatment and other factors,which often lead to FCBP in clinical missed diagnosis and misdiagnosis.In this study,the literature was reviewed,and through clinical analysis of the skin manifestations,histopathology,immunopathology,causes of the misdiagnosis,treatment and prognosis of familial chronic benign pemphigus,this study aims to enrich the clinicians' understanding of familial chronic benign pemphigus,and reduce the missed diagnosis and misdiagnosis of this disease,so that the patients can be treated in a regular and timely manner.Materials and MethodsFrom January 2008 to January 2018,the total of 37 patients with familial chronic benign pemphigus in First Affiliated Hospital of Zhengzhou University were collected.All the patients met the diagnostic criteria of familial chronic benign pemphigus.The data of general data,family history,skin manifestations,pathological examination,causes of the misdiagnosis and missed diagnosis,treatment and prognosis of the patients were collected.Some of the measurement data were statistically analyzed using SPSS22.0 statistical software,and P < 0.05 was considered statistically significant.Results 1.GenderAmong the 37 patients with familial chronic benign pemphigus,19 were male and 18 were female,male are more than female.The ratio of male to female was 1.06:1.There was no significant difference in the distribution of male and female sex ratios(P=0.107).2.AgeAmong the 37 patients with familial chronic benign pemphigus,the youngest was 24 years old,the oldest was 82 years old,mean age 47.83 years old,the onset age was 17 to 45 years old(mean 31.81 years),the course of disease was 2 to 50 years(mean 14.94 years).3.Family history25 cases with family history,7 cases with sporadic cases and 5 cases with unknown family history.4.Inducing or aggravating factorsExercise,sweating,scratching and so on could make the severity of rash aggravated in 31 patients in varying degrees.The severity of the disease was related to season in 23 patients.5.Skin lesions5.1 the site of the disease: All 37 cases had varying degrees of axillary and groin involvement,followed by the neck,elbow fossa,axillary fossa,under breast fold,umbilicus,vulva,anus,scrotum,rare in the back,scalp so on.Some nails were affected,two patients in this group;generalized patients were rare,one patient in this group.5.2 Subjective symptoms: All 37 cases had different degrees of pruritus,the pain was obvious in 10 cases.5.3 Form of skin lesion:All 37 cases showed erosions,scabs,and moist proliferative surface,partial erythemas,fissures and hyperpigmentations.Nikolsky's sign was negative in 35 cases and positive in 2 cases.6.Concomitant disease4 patients with hypertension and / or diabetic,2 patients with tinea cruris,no visceral involvement was found in all 37 cases.7.Histopathology and immunopathology examinationSkin lesions were taken from 37 patients for pathological examination.All 37 cases showed different degree of epidermal acantholysis release,and villus existed.29 cases were accompanied with hyperkeratosis and / or parakeratosis.Keratinized cells were found in 8 cases.One or more inflammatory cells were infiltrated in the superficial dermal vessels and around the adnexa in 15 cases.In 12 cases,the capillaries in the papillary dermis were obviously dilated and proliferated.No cutaneous appendages involvement was found in all of the 37 cases.37 cases were examined by immunofluorescence,and the results were all negative.8.MisdiagnosisAmong the 37 cases,28 of them were misdiagnosed,and there were many misdiagnoses.The conditions were as follows: misdiagnosed as eczema 25 times,misdiagnosed as intertrigo 15 times,misdiagnosed as femoral hernia 6 times,misdiagnosed as pemphigus vegetans 1 times,misdiagnosed as pemphigus vulgaris once,misdiagnosed as keratosis follicularis once,misdiagnosed as inverse psoriasis once,misdiagnosed as neurodermatitis once,and misdiagnosed as external Paget disease of the breast once.9.TreatmentAll the 37 patients were treated with systemic therapy.At the acute stage of admission,21 patients were given methylprednisolone sodium succinate injection 30 ~ 40 mg / day,combined with antibiotics.The other patients with mild symptoms were given antibiotics.The patients who with fungal infection were treated with Spirenno capsule;with hypertrophic rash and/or keratinized papules were treated orally with Avera capsule 10 ~ 30 mg / day.Patients with significant itching were given oral antihistamine drugs.The choice of topical drugs varies according to the condition of skin rash.All patients,depending on the state of illness and treatment response,continued to consolidate the treatment for 2 to 4 weeks.10.PrognosisThe average hospitalization time of 37 patients was 8 days.28 cases were cured and discharged,and the rest were all improved and discharged.Conclusion1.Familial chronic benign pemphigus is an autosomal dominant hereditary disease.The performance of different patients may be different,and they are easily misdiagnosed and missed,so clinicians should raise their awareness.2.The course of familial chronic benign pemphigus is prolonged and easy to relapse,so individualized treatment should be used according to the clinical manifestation of the patients.