Hailey-Hailey disease responding to thalidomide

Abstract

Familial benign chronic pemphigus or Hailey-Hailey disease (HHD) is a rare autosomal dominant disorder characterized by the development of recurrent blisters and erosions in the intertriginous areas. Various topical and systemic treatment options include corticosteroids, topical 5-fluorouracil, topical vitamin D analogs, topical zinc oxide, dapsone, psoralen plus ultraviolet A, systemic retinoids, cyclosporine, methotrexate, and photodynamic therapy. In recalcitrant cases, further options including, invasive methods such as grenz ray therapy, carbon dioxide laser abrasion, and erbium: YAG laser ablation, dermabrasion, electron beam therapy, botulinum toxin, and full-thickness excision of affected skin with repair by split-thickness grafting have been reported as useful in treatment of HHD. We describe a case of HHD who was treated with several treatment modalities including antibiotics, corticosteroids, and dapsone earlier and when presented to us had a severe recalcitrant disease. Thalidomide, as a modality of treatment has been successfully used in few cases earlier. Our patient responded well to thalidomide.

Darier and Hailey‐Hailey disease: update 2021

“…There are also individual case reports on successful treatment with: –Doxycycline (100 mg per day) [94] –Methotrexate (7.5–15 mg per week) [95–97] –Dapsone (initially 50–200 mg per day, with subsequent reduction to 50 mg) [98] –Etanercept (one positive case report; 75 mg per week) [99] –Azathioprine [100] –Ciclosporin (2.5 mg/kg BW per day) [101, 102] –Thalidomide (initially 100 mg 3 x per day, maintenance dose 50 mg per day) [103] –Terbinafine (250 mg per day) [104] …”

An elderly lady with multiple blisters all over the body: Hailey Hailey Disease

“…Topical medication such as vitamin D3 analogs, antibiotics, and corticosteroids are advised for mild cases.A number of systemic treatments such as doxycycline, methotrexate, cyclosporine, acitretin, thalidomide, alefacept, alitretinoin, afamelanotide, terbinafine, and naltrexone are reported effective in many patients producing long remissions. 16,17,18 In case of severe diseases or those which are recalcitrant to conventional therapy, invasive treatments have also been attempted, such as laser ablation, photodynamic therapy, electron beam radiotherapy, botulinum toxin injection, surgical excision, and grafting of the lesions have been reported beneficial for patients. 19,20,21,22 Some patients are advised to keep away fromtriggers like certain foods, stress, excessive heat, prolonged sweating and constant friction to avoid an outbreak.…”

Treatment of Severe Hailey-Hailey Disease With Apremilast

“…The more severe forms of HHD are difficult to manage with only topical treatments or laser. Systemic approaches with cyclosporine, 2-5 methotrexate, 6,7 or thalidomide 8 have been proposed and gave isolated success.…”

“…The more severe forms of HHD are difficult to manage with only topical treatments or laser. Systemic approaches with cyclosporine, methotrexate, or thalidomide have been proposed and gave isolated success. There are increasing reports of data showing that keratinocytes are able to produce large amounts of chemokines, including CXCL10 .…”

“…Although the primary event of HHD is acantholysis, the standard treatment relies on topical corticosteroids that reduce localized inflammation. Isolated successes have also been reported with cyclosporine, methotrexate, and thalidomide, suggesting that immunomodulatory drugs could be helpful for severe cases. Apremilast is a small molecule, approved for use in the treatment of psoriasis, that specifically inhibits the activity of cyclic adenosine monophosphate phosphodiesterase-4 decreasing T h 1 and T h 17 activation and CXCL10 release.…”