I Stolze1, H Hamm, G H Weyandt. 1. Department of Dermatology, University Hospital Würzburg, Würzburg, Germany.
Abstract
AIM: Hailey-Hailey disease (HHD) is a genodermatosis characterized by recurrent chronic weeping plaques at intertriginous sites. In severe cases, topical and systemic treatments are often insufficient. Extensive excisions are traumatic for patients and have a high morbidity. Superficial ablative procedures such as dermabrasion have become established, but are difficult to perform in the perianal region and scrotum. Therefore, alternative therapies are required. METHOD: Four patients with extensive anogenital HHD were treated by argon plasma coagulation (APC). This leads to a controlled coagulation of nearly constant depth. After dividing the affected areas into segments, a multilayered APC was performed in every second segment to the upper corium. The remaining segments were treated in the same way after healing of the initially treated parts. RESULTS: A disease-free period of up to 37 months was observed in all four patients. There was no case of reduced cutaneous sensitivity or extensive scarring. CONCLUSIONS: Argon plasma coagulation is effective and safe for perianal and scrotal HHD. The benefits include the accessibility to difficult anatomic sites and good control of ablation. The risk of anal stenosis is minimized by the segmental two-step procedure. The rapid re-epithelialization occurs from structures not affected by the disease and allows rapid mobilization of patients.
AIM: Hailey-Hailey disease (HHD) is a genodermatosis characterized by recurrent chronic weeping plaques at intertriginous sites. In severe cases, topical and systemic treatments are often insufficient. Extensive excisions are traumatic for patients and have a high morbidity. Superficial ablative procedures such as dermabrasion have become established, but are difficult to perform in the perianal region and scrotum. Therefore, alternative therapies are required. METHOD: Four patients with extensive anogenital HHD were treated by argon plasma coagulation (APC). This leads to a controlled coagulation of nearly constant depth. After dividing the affected areas into segments, a multilayered APC was performed in every second segment to the upper corium. The remaining segments were treated in the same way after healing of the initially treated parts. RESULTS: A disease-free period of up to 37 months was observed in all four patients. There was no case of reduced cutaneous sensitivity or extensive scarring. CONCLUSIONS:Argon plasma coagulation is effective and safe for perianal and scrotal HHD. The benefits include the accessibility to difficult anatomic sites and good control of ablation. The risk of anal stenosis is minimized by the segmental two-step procedure. The rapid re-epithelialization occurs from structures not affected by the disease and allows rapid mobilization of patients.