Superficial Radiotherapy For Familial Benign Chronic Pemphigus

Background:Familial benign pemphigus is an autosomal dominant hereditary dermatosis that affects the action of desmosomes between keratinocytes in the epidermis.The clinical manifestations are erosion and blister,often occurring in the axillary,groin,or submammary folds.HHD tends to be chronic and recalcitrant to treatment.Therefore,many modalities of treatment have been studied.The treatment includes topical and systemic drugs,surgery with skin grafting,laser therapy,radiotherapy and so on.The chronic nature of the disease and multiple recurrences make the disease bothersome for patients and a treatment challenge for physicians.So it is very important to explore a treatment which is effective.Therefore,we study the effect of superficial radiotherapy on patient with HHD.Objective:The outpatient or hospitalized patients who were diagnosed as familial benign pemphigus by pathological examination in 2014-2017 year were selected as the research objects.After signing the informed consent,they received proper management to assess the curative effect of five patients with HHD in the treatment of superficial radiotherapy.Methods:SRT-100 superficial radiotherapy treatment system was applied to 5 HHD patients who were recalcitrant to traditional therapy in axillary or groin areas.According to the severity of the initial skin lesions and the tolerance to radiation of the patients,a treatment plan was developed.The total radiation dose was 9-12 Gy at the energy 50 KV.Results:(1)After 4-35 months of follow-up,three cases were completely improved and two cases were partly improved;(2)Compliance of patients and pigmentation are major restrictive factors of superficial radiotherapy.Conclusion:HHD is such a disease which has a chronic course and severe skin lesions and patients suffer a lot of pain.Superficial radiotherapy can achieve an impressive and curative effect among the several managements.In addition,the adverse effects of radiotherapy include transient erythematous reactions,pigmentation at the irradiated site,and the risk of skin cancer.In our study,only 1 patients had temporary erythematous response,and 4 patients had been pigmented and there was no case of skin cancer at the end of the study because of the low dose of radiotherapy.