Clinical Observation Of Dermabrasion In The Treatment Of Familial Benign Chronic Pemphigus

Background Familial benign chronic pemphigus,also known as Hailey-Hailey disease(HHD),is an autosomal dominant hereditary skin disease caused by a mutation in the ATP2C1 gene on chromosome 3q21-24,which results in disturbed intracellular Calcium homeostasis and impaired formation of desmosomes,leading to adhesion disorders between epidermal keratinocytes.Histologically,acantholysis can be seen in HHD,characterized by "collapsed brick-like" structures.The clinical manifestations are repeated erythema,blisters,exudation,erosion,and secondary infections with malodor,lesions can be limited or generalized.Currently,the disease lacks effective treatment methods.Systemic application of antibiotics combined with glucocorticoid therapy has a definite effect,but the disease is easy to recur.Separate or combined application of short-term oral dapsone,cyclosporine,acitretin,tripterygium glycosides are alternatives.Topical treatment includes topical glucocorticosteroid ointment,antibiotic ointment,calcineurin inhibitor,which inhibit acantholysis and reduce inflammatory response.In addition,physical therapy also has good results,including electron beam radiation therapy,CO2 laser,dermabrasion and so on.Since the 1990 s,with the development of laser technology,pulsed laser and holmium laser grinding have shown better grinding results and relatively less side effects.As a long-established technology,dermabrasion is an effective method for the treatment of superficial skin diseases.Grinding the lesion to the superficial layer of the dermis causes local scarring,or causes epithelial regeneration of the appendage to eliminate local lesions,thus obtaining a more obvious therapeutic effect.The effects and adverse effects of dermabrasion are closely related to the depth of the grinding.Dermabrasion can be tried if the intractable skin lesions are limited to the epidermis or even the superficial dermis.Objective To evaluate the effect of different factors on the treatment of HHD in patients with HHD after grinding,and to evaluate the efficacy and safety of dermabrasion in the treatment of HHD.Methods Patients with familial benign chronic pemphigus were collected for dermabrasion.Histopathological biopsy was performed before,during and after surgery,and clinical photographs were compared.According to the division of the surgical site,calculate the effective rate and overall effective rate of different parts of the surgery,evaluate the efficacy,and record the adverse reactions.Results The surgical procedure of dermabrasion was applied to 4 patients,and a total of 47 areas were ground.in which 36 areas were recovered,7 areas were markedly improved,4 areas were improved,and no areas was ineffective.The total effective rate was 91.4%.Among them,the neck,axilla,breast,abdomen,forearm and other places have an effective rate of 100%,while the effective rates of the skin folds and easily-infected areas of the cubital fossa and groin areas are slightly lower,75% and 81.3%,respectively.During the Long-term follow-up,2 cases of hypertrophic scar were observed and the incidence of hypertrophic scar was 4.3%.Superficial scars were observed in the remaining surgical area.Compared to the normal skin,the epidermis of the area was rougher,flatter,and had softer texture,and chromatosis and/or depigmentation developed in the area.During the follow-up of 14-34 months,no recurrence was found in the surgical area,but in the untreated area.It was also confirmed by histopathological biopsy without a typical "collapsed brick wall" change.Conclusion 1.The total effective rate of dermabrasion for the treatment of familial benign chronic pemphigus was 91.4% with good efficacy and no recurrence.2.Dermabrasion for the treatment of familial benign chronic pemphigus is safe and has no significant side effect.