PG is a rare form of neutrophilic dermatosis that presents as an inflammatory and ulcerative disorder of the skin. Because no diagnostic criteria have been established for clinical use, PG is a diagnosis of exclusion. In their review, Su et al11 proposed major and minor criteria for the diagnosis of PG: the major criteria are rapid progression of a painful, necrolytic cutaneous ulcer and exclusion of other cutaneous disease; the minor criteria are pathergy, systemic disease-associated PG (ie, inflammatory bowel disease, arthritis, IgA gammopathy, or malignancy), and/or rapid response to systemic glucocorticoid treatment. To be considered a definite case of PG, the condition should meet the 2 major criteria and at least 2 minor criteria. von den Driesch2 independently used inclusion criteria for the diagnosis of PG patients that are almost identical to Su et al.11
Based on Oka et al’s5 study and Ahronowitz et al’s12 review, neutrophil dysfunction, genetic factors, and dysregulation of the immune system might contribute to PG. The condition requires multiple treatment modalities to reduce inflammation and optimize wound healing, in addition to treating any underlying diseases. Prednisone and cyclosporine have been mainstays of the systemic treatment for PG, although several clinical trials12 support the use of biological therapies such as tumor necrosis factor-α inhibitors for refractory cases of PG. According to Prystowsky et al’s13 summary of the management of 22 cases of PG over 4 years and Chow et al’s14 review of current PG treatment, experience suggests patients should receive 0.5–1 mg/kg per day of oral prednisone or its equivalent. The glucocorticoid treatments should be tapered off and discontinued within 4–10 weeks.
Bennett et al15 performed a retrospective analysis of the medical records of 86 patients with PG who were evaluated and treated over 12 years at 2 university-based dermatology departments. Although clinical signs of improvement might be evident within a few days of initial treatment, the 86 PG patients reviewed required a mean 11.5 ± 11.1 months of treatment to achieve complete healing.
Results of Dodiuk-Gad et al’s16 review included an evidence-based, strategic approach to the general risk management of systemic glucocorticoid use. Although the potent anti-inflammatory and immunosuppressive effects of systemic glucocorticoids have led to their wide use in the treatment of dermatologic diseases, long-term therapy of weeks or months with systemic glucocorticoids is associated with significant adverse effects, such as osteoporosis, myopathy, peptic ulcer disease, hyperglycemia, hypertension, and edema. For Ms. J, given her medical history, continued use of prednisone could have led to poor control of her diabetes and hypertension, and her peptic ulcer disease could have recurred.
Wound treatment with HBOT is well established. According to a Cochrane database systematic review,7 HBOT significantly improved the chance of healing foot ulcers in persons with diabetes mellitus. Dauwe et al9 conducted a systematic review of HBOT in the treatment of complicated acute wounds, flaps, and grafts: a total of 8 studies (4 prospective, randomized, controlled trials; 3 prospective, nonrandomized, controlled trials; and 1 retrospective, controlled trial) addressed the use of HBOT for wounds in humans. The results showed HBOT can augment healing in complicated acute wounds, such as skin graft survival, healing of burn injuries, and crush injuries.
For Ms. J, hypertension, diabetes mellitus, and a history of peptic ulcer precluded long-term steroid use. Using adjuvant HBOT, the wounds heal rapidly in 2 weeks, allowing clinicians to decrease steroid dosage and duration. More studies are needed to increase the evidence base for this treatment approach.