hailey - hailey disease ( hhd ) , otherwise known as familial benign chronic pemphigus is a rare autosomal dominant , acantholytic disease with incomplete penetrance that is characterized by recurrent blisters , plaques , and erosions often accompanied by a burning or pruritic sensation . it is caused by a mutation in the atp2c1 gene on chromosome 3q21-q24 , which encodes a disrupted golgi associated ca atpase . this mutation induces abnormal intracellular ca signaling which promotes premature keratinocyte proliferation leading to inappropriate desmosomal protein production causing failed keratinocyte adhesion and acantholysis , typically at flexural regions and friction prone sites . we report a 64-year - old female with a 37-year history of severe hailey - hailey disease involving her whole body . the patient s hhd has been unresponsive to common therapies used in the management of hhd . in 2005 , a then 55-year - old caucasian female presented with clinical signs and symptoms of hhd , which had been active for 32 years . the patient initially developed symptoms of hailey - hailey disease in 1973 in her axillae and inframammary folds as well as her pubic region ( namely her labia ) . she also reported that hhd lesions have previously appeared on her arms , neck , back , abdomen , popliteal regions , and oral mucosa . the presence of white bands on her fingernails , a rare manifestation of the disease , has also been documented . in 2003 , a second biopsy was performed and confirmed that the inframammary lesions were in fact hhd lesions . most recently , the patient presented with erythematous and crusted erosions and erupted bullae on her pubic region and middle back ( figure 1 ) . since the patient s initial presentation and diagnosis , several different treatments have been administered for the management of the disease and its symptoms including : steroid and non - steroidal anti - inflammatory treatments ( systemic , topical and intra - lesional ) , cyclosporin , methotrexate , dapsone , botulinum toxin a , fraxelated co2 laser to affected regions , and a variety of oral and topical antimicrobial therapies with minimal relief . of the treatments given the patient s disease and symptoms were best controlled by oral doses of prednisone during times of exacerbated symptoms with doses of 30 - 50 mg per os . however , attempts to taper the prednisone to doses less than 10 mg were met with the recurrence of symptoms of the disease . recent studies have reported the use of narrowband uvb or alitretinoin as successful independent therapeutic options in the treatment of hhd . considering the persistent nature of the patient s disease a combination therapy of alitretinoin , an oral retinoid agent known as toctino ( 30 mg per os daily ) , and narrowband uvb therapy was started . the narrowband uvb therapeutic dose range was determined by the patient s skin type . for our patient , the narrowband uvb range began at 0.200 j / cm with an initial exposure time of 30 seconds . uvb therapy was administered twice weekly and was to be continued until a total of 30 treatments ( approximately 4 months ) had been reached . treatment was administered using the professional full body unit by ultralite enterprises ( ultralite enterprises , lawrenceville , ga , usa ) . based on the patient s positive response to the treatment , the uvb dose was increased by increments of 0.020 j / cm each visit until a dose of 0.294 j / cm was reached . the treatment doses administered throughout the patient s treatment were within a safe range as the minimum erythematous dose described by hamada et al . the course of treatment undertaken in this case is unique because existing literature describes the treatment of hhd with oral alitretinoin while the patient was tapering prednisone . the patient described in this report began the combination alitretinoin - narrowband uvb treatment within days of prednisone cessation . the patient reported that the lesions found on her pubic region and middle back improved within the first 2 - 3 weeks of starting this new combination alitretinoin and narrowband uvb treatment . upon follow - up 6 weeks after beginning this new treatment course , a marked increase in healing bullae and tissue on the patient s back were noted . residual rubor from uvb burns experienced during the fourth uvb session were also noted . only after the fourth uvb treatment did the patient present with rubor from uvb burns , which led to the cessation of the uvb treatment , but continued use of oral alitretinoin as a mono - therapy . healing hhd lesions could also be seen in the patient s pubic area at this time with no new lesions noted ( figure 2 ) . no other topical or systemic treatments were used throughout this treatment period . currently , a mono - therapy of oral alitretinoin has maintained remission of the disease for 14 weeks since the beginning of the initial combination alitretinoin and narrowband uvb treatment course . no other treatments or interventions have been used , and no new lesions have been experienced with the alitretinoin mono - therapy . patients usually experience a relapsing - remitting course of the disease . for many patients , a family history of hhd lesions favoring the axillae , chest , neck , genital areas , and other flexural regions are usually noted . secondary infection with candida and/or staphylococcus is often noted and considered to be a common complication of the disease . involvement of the vulva , conjunctiva , and mucosae are considered rare manifestation of the disease . there is currently no cure for hhd , however , treatment for managing the symptoms are available . in some cases , antibiotics , antifungal agents , as well as systemic , topical , and intralesional corticosteroids have proven effective for the management of hhd . furthermore , other agents such as cyclosporine , retinoids , botulinum toxin a , and dapsone have also proven to be effective in some cases , and ineffective in others . according to sardy and ruzicka , the patient , whose case is described , presented with a severe manifestation of hhd with recurrent bullae , erythematous patches , and erosions . the patient has experienced hhd lesions on many different areas of her body including her genital region , neck , back , and oral mucosa , to name a few . for this patient , hhd has been a part of her life for almost 40 years . recently , the successful remission of hhd with narrowband uvb has been discussed . in this patient this may be attributed to the oral alitretinoin , a retinoid agent that has been shown to induce photosensitivity . the case report by sardy and ruzicka showed successful treatment with alitretinoin in a patient with hhd . the treatment was accompanied by a continued course of oral prednisone , which was later tapered while treatment with oral alitretinoin persisted . conversely , in our patient s case , the combination daily oral alitretinoin ( 30 mg ) and narrowband uvb therapy described , which resulted in the successful remission of the patient s hhd , was started after the tapering and complete cessation of prednisone use . further , considerable and sustained clinical improvement of the patient s hhd lesions has been noted with the administration of the daily oral alitretinoin therapy alone . presently there has been no need for any treatment with further uvb , prednisone , or any other systemic or topical agents as the patient s disease appears to be in remission . for the first time in almost 40 years the patient has found relief and an effective treatment for her hhd . in conclusion , we suggest that conjunctive therapy of oral alitretinoin with narrowband uvb therapy be considered as a therapeutic option for the treatment of hhd to be followed by a mono - therapy of alitretinoin . the use of oral alitretinoin and narrowband uvb therapy should be explored further . in addition , the efficacy of oral alitretinoin as a mono - therapy for hhd should be explored .
hailey - hailey disease ( hhd ) is a chronic familial bullous disease characterized by recurrent blisters and erosions typically at friction - prone areas of the body accompanied by acantholysis upon histologic examination . 
 there are a number of therapies used in the management of hhd . 
 its symptoms have been effectively treated with antimicrobial therapies , corticosteroids and other agents such as cyclosporine and prednisone 
 . however , such treatments are not always effective . 
 therefore , there is a need for new treatments for the management of hhd . in this report 
 , a patient with long - standing hhd responsive only to high levels of prednisone is described . 
 after the successful tapering and cessation of oral prednisone the patient began a new combination therapy of complementary doses of oral alitretinoin , and narrowband uvb therapy , which yielded a favorable response within 2 - 3 weeks . 
 after 6 weeks , a mono - therapy of daily ( 30 mg ) oral alitretinoin was sufficient to maintain successful near - complete remission of the disease .