Department of Dermatology, Medical University of Lodz, Poland.
Acta Derm Venereol. 2010 Mar;90(2):179-82. doi: 10.2340/00015555-0791.
Hailey-Hailey disease, or familial benign chronic pemphigus, is an autosomal dominant genodermatosis. Disease symptoms may contribute to an adverse impact on quality of life and functional limitation and disability. As Hailey-Hailey disease is chronic and frequently recalcitrant to treatment, multiple therapeutic approaches, including surgical options, have been attempted. We describe here three cases of recalcitrant Hailey-Hailey disease that showed long-term improvement with radiotherapy. Axillary lesions were treated with electron beam at an anti-inflammatory dose (energy 6-8 MeV). Patients received 20 Gy in 10 fractions to 90% isodose, at each axilla. No disease recurrence was observed during a 38 months follow-up of the treated sites. The effect of radiotherapy was thus considered to be locally beneficial, but without any positive influence on the general course of the disease.
Hailey-Hailey 病,亦称家族性良性慢性天疱疮,是一种常染色体显性遗传皮肤病。疾病症状可能会对生活质量产生不利影响,并导致功能受限和残疾。由于 Hailey-Hailey 病为慢性疾病且常对抗治疗有抵抗,因此已尝试多种治疗方法,包括手术选择。我们在此描述了三例难治性 Hailey-Hailey 病患者,他们接受放射治疗后获得了长期改善。腋部病变采用电子束进行抗炎剂量(能量 6-8 MeV)治疗。患者在每个腋窝处接受 20 Gy 的 10 个分次照射,达到 90%等剂量线。在治疗部位的 38 个月随访期间,未观察到疾病复发。因此,放射治疗的效果被认为是局部有益的,但对疾病的总体进程没有任何积极影响。