Superficial radiotherapy may induce complete remission in Hailey-Hailey disease

Superficial radiotherapy yielded complete remission in 90% of treated areas in a small cohort of individuals with Hailey-Hailey disease, according to recent findings.

“Hailey-Hailey disease (HHD) is a chronic genodermatosis with recurrent vesicles and erosions mainly in the intertriginous areas,” Hans Christian Wulf, MD, DMSc, PharmD, and Stine Regin Wiegell, MD, PhD, DMSc, of the department of dermatology at Bispebjerg University Hospital in Copenhagen, Denmark, wrote.

While standard interventions for HHD are targeted toward managing flares, long-term remission often does not result. Patients often report poor quality of life.

In the retrospective case series, the researchers described outcomes associated with superficial radiotherapy among 13 individuals — including eight women — with severe, treatment refractory HHD.

Eligible participants had HHD for a mean duration of 24 years (standard deviation [SD], 14). The mean age of the study population was 52 years (SD, 18). Treatment and follow-up were conducted between January 2015 and April 2021 at a single dermatology center in Denmark.

Superficial radiotherapy treatment consisted of 20 kV. Eight fractions of two gray each, for a total of 16 gray, comprised one cycle. Participants underwent as few as one and as many as six treatment cycles, with one to five body areas receiving treatment per cycle. Overall, clinicians treated 62 separate body areas.

Complete long-term remission served as the primary endpoint. The researchers defined this as the absence of relapse during 12 months of follow-up.

Results showed that 90% of the 62 treatment areas reached long-term remission after superficial radiotherapy. This outcome was achieved after a mean follow-up duration of 32 months (SD, 12).

Complete full remission in all treated areas after one cycle of superficial radiotherapy was reported by nine patients (69%). An additional three patients reported this outcome after two cycles.

One patient reached partial remission in one of two areas that underwent treatment. However, this improvement was sufficient enough that the patient chose to abstain from further intervention.

Safety data showed severe inflammation for as long as 1 month as a common response to superficial radiotherapy. Temporary hyperpigmentation of treated areas occurred after the inflammation subsided.

Regarding quality of life, the researchers reported an average Dermatology Life Quality Index score of 22 prior to intervention. This average score decreased to 3 after treatment.

“The results of this case series suggest that treatment with [superficial radiotherapy] was associated with remission in patients with severe HHD and may provide a long-term improvement of treated skin areas,” Wulf and Wiegell concluded.