Benign familial pemphigus: little benefit from superficial radiotherapy

Benign familial pemphigus (Hailey-Hailey disease) is a rare relapsing-remitting epidermal blistering disease palliated by a multitude of medical and surgical treatments. There are limited reports of benefit from low-penetration X-rays. We describe two resistant cases that appeared to respond initially to superficial radiotherapy, a 66-year-old man who had multiple courses to the groins (4 x 2.00 Gy/5 x 3.00 Gy) and to the axillae and low back (10 x 2.00 Gy) and a 53-year-old man treated twice to the groins and perineum (5 x 3.00 Gy/10 x 2.00 Gy). However, on longer follow up, benefit was found to be questionable. We conclude that while superficial radiotherapy may offer temporary relief, there is little evidence that it alters the natural history of this disease.