Case series of hailey-Hailey disease treated with apremilast

Hailey-Hailey disease (HHD) is a rare autosomal-dominant blistering condition caused by heterozygous mutations in the ATP2C1 gene on chromosome 3q21-q24 which encodes ATPase.The typical skin lesions of HHD usually occur in friction or intertriginous areas, tend to form erythematous plaques with wormeaten erosions and painful fissures or moist vegetations.Treatment includes general measures like weight management to minimize friction and comfortable clothing to prevent heat, moisture and friction.Topical steroids, antibiotics, vitamin D3 derivative and tacrolimus helps to control the disease flares in mild cases.Systemic treatment includes antibiotics, steroids, cyclosporine, dapsone, methotrexate and thalidomide in severe cases.Treatment of this disease till date is far from satisfactory.Here we report 3 cases (two females and one male) presented with macerated, patch with fissures in both axilla and groin.Characteristic "dilapidated brick wall appearance" was observed histologically.The patients were prescribed various therapeutic regimens over the period of one year, the response was unsatisfactory, so they were started on apremilast (phosphodiesterase-4 inhibitor), initially with induction dose and later with maintenance dose of 30mg every 12h, with a very good response in 2 female patients and one lost with the follow-up.We suggest that apremilast may be a low risk therapeutic option for severe cases of HHD.Accumulation of a greater number of similar cases and further research will be required.