Epidermolysis Bullosa (EB) is heterogeneous group of rare heritable disorders characterised by blistering of the skin with minor trauma. Despite limited evidence to suggest patients with EB have more postoperative wound complications than the general population, we have noted reluctance among some surgeons to operate on these patients. 

We consequently designed this study to investigate postoperative wound healing in patients with EB. Patients recruited from the Australian National Diagnostic Laboratory Database for EB and the Australasian EB Registry were posted the ‘Surgical Wound and Scar Healing in EB’ questionnaire which contains twenty-two questions about a patients experience of having surgery.

Forty-six patients reported a total of 94 surgical procedures. The most frequent surgical procedure was the excision of a skin lesion, followed by musculoskeletal surgery, a caesarean section or an appendectomy. Five patients reported blistering at the surgical wound site after seven surgeries. Four patients reported four postoperative wound infections and one reported a postoperative wound dehiscence. Twenty patients had 34 postoperative wounds which they felt healed slower than someone without EB and 30 patients had 55 postoperative wounds which they felt healed at the same rate as someone without EB. After 67 surgeries, the postoperative scar was reported to heal [lat and after 18 it was reported to heal as a keloid scar.

It is unlikely that patients with localised EB simplex will develop blistering at postoperative wound site, but about a quarter of patients with generalised EB may develop blistering which is likely interfere with wound healing. Postoperative wound infections do not appear to occur any more frequently in patients with EB than in patients within the general population and wound dehiscence is uncommon in patients with EB. Postoperative wounds may heal at a similar or slower rate in patients with EB compared to those within the general population and patients with EB may have a propensity to develop keloid scars.

Despite the inherent limitations of a postal survey we feel clinicians should be more con[ident to refer patients with EB for surgery and surgeons reassured about postoperative wound healing and complications.

Surgical wound healing in patients with epidermolysis bullosa