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Superficial radiotherapy tied to remission, better quality of life in severe Hailey-Hailey disease

JAMA Dermatology
Reuters Health - 28/01/2022 - In patients with severe, treatment-resistant Hailey-Hailey disease (HHD), a rare skin disorder, superficial radiotherapy is associated with successful remission and improvements in health-related quality of life (QoL), according to a new study.

"The study results suggest that superficial radiotherapy may be associated with remission in the treatment of Hailey-Hailey disease and potentially provides a long-term improvement of treated skin areas," Dr. Stine Regin Wiegell of the University of Copenhagen, Denmark, and colleagues write in JAMA Dermatology.

Painful erosions of the skin in sensitive areas, such as the groin and genitals, are a key manifestation of HHD, and "the disease has a great negative impact of the patient's QoL and decreases work ability and sex life," Dr. Wiegell explained in an email to Reuters Health.

Standard treatments for HHD, such as antibiotics, are frequently used to control flares, but do not induce long-term remission, she added. "Additionally, other systemic treatments such as isotretinoin and naltrexone are not very effective in controlling flares and are associated with side effects."

To explore the use of superficial radiotherapy (SR), which some research had suggested might be helpful, Dr. Wiegell and her colleagues analyzed data on 13 patients who received the treatment in Denmark between 2015 and 2021. Patients had a mean age of 52 years and a mean disease duration of 24 years.

For each HHD area, patients received one SR cycle with 16 Gy in eight fractions of 2 Gy every two to three days. Across the group, patients received one to six treatment cycles to between one and five separate body areas.

Of the 62 treated areas, 56 (90%) achieved complete remission with a median follow-up of 28 months. Nine patients (69%) experienced complete remission in all treated areas after the initial treatment cycle, while another three patients had complete remission following the second treatment cycle.

In a single patient who experienced partial remission in one of two treated skin areas, the patient chose to stop treatment due to disease improvement.

Severe inflammation occurred for up to one month after treatment in most patients, and this inflammation was followed by transient and slight hyperpigmentation. There were no subsequent skin infections or flares in successfully treated areas.

Prior to treatment, the mean Dermatology Life Quality Index (DLQI) score was 22, indicating that HHD had an "extremely large effect" on patients' life. After treatment, the DLQI score reduced to a mean of 3, indicating HHD now had a "small effect" on patients' life.

According to Dr. Wiegell, there is "always a concern" that SR may be associated with secondary skin cancer in the treated area. In the study, however, no carcinomas were reported during follow-up. Given that "the dose of SR in HHD is extremely low, we therefore consider the risk of secondary skin cancer very low," she added.

Based on the overall findings, Dr. Wiegell suggested that SR "should be first-line treatment for patients with severe treatment-refractory HHD."

Dr. Zachary Schwager, director of the Blistering Disease Clinic at Lahey Hospital & Medical Center in Burlington, Massachusetts, told Reuters Health by email that treatment "resulting in long-term remission could be a major game changer for patients suffering with this disease (by) sparing them ongoing physical and psychological distress."

Dr. Schwager, who was not involved in the new study, added that if further research supports the findings, SR "could be adopted as viable treatment option for patients with severe HHD or those failing conventional topical treatments."

SOURCE: https://bit.ly/3GWLGBS JAMA Dermatology, online January 12, 2022.

By Brandon May

© 2023 The Author(s). Published by Medicom Medical Publishers.
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