RESIDENT ROUNDS: PART III

Local therapy includes effective wound care with moisture-retentive dressings,¹¹ topical corticosteroids, 5-aminosalicylic acid, sodium cromoglycate, and nitrogen mustard, with or without intralesional corticosteroids, or a short course of systemic cyclosporine. For more advanced disease, systemic therapy is necessary and includes systemic corticosteroids, azathioprine, alkylating agents (cyclophosphamide, chlorambucil, melphalan), daunorubicin, cyclosporine, tacrolimus, thalidomide, sulfa drugs, and minocycline. Non-pharmacologic therapies for advanced disease include intravenous immunoglobulin (IVIG), plasmapharesis, and hyperbaric oxygen therapy. In recalcitrant or severe cases, systemic steroids are the initial mainstay treatment with consideration for combination cyclosporine.¹² Surgical treatment has been proposed with split and full-thickness skin grafting and negative pressure wound therapy with favorable results.¹³ However, many would argue surgery in a PG patient is ill-advised given the known risk of pathergy. If skin grafting is proposed, one must understand the risks of new ulcer formation at the donor site and enlargement of ulcers at the recipient site.Given the wide variety of aforementioned treatments, selecting a treatment regimen with confidence can certainly be challenging. Additionally, PG often portends an unpredictable course and delayed response to therapy which, heralds difficulty in assessing treatment successes and failures. Miller and colleagues have delineated a practical ranking of medication efficacies in a 2010 review.¹² Prednisone, infliximab, cyclosporine, mycophenolate mofetil, and adalimumab were ranked among the top five pharmacotherapies with regard to efficacy. In our opinion, the soundest recommendation is infliximab infusion at 5 mg/kg as the corresponding study was blinded and placebo-controlled.Over the past few decades, anti-TNF therapy has obtained FDA approval for autoimmune diseases such as IBD, RA, and psoriasis. While infliximab has been proven to be effective in a well-designed study, no such study exists for adalimumab or etanercept in the treatment of PG. Etanercept has been used with success in a small case series,¹⁴ while only case reports have documented the efficacy of adalimumab.In our patient, the TNF-antagonist was also beneficial for his underlying systemic disease. This is expected since adalimumab is FDA approved for CD. In cases of PG with underlying systemic illness, it is imperative to manage such patients in conjunction with gastroenterologists, rheumatologists, hematologists, and oncologists, as necessary. Tailoring therapy to target the underlying illness will often improve the cutaneous manifestation of PG. In the common case of idiopathic PG, the dermatologist carries sole responsibility in providing care, perhaps in conjunction with wound care clinics.Although there exists a dire need for clinical trials in such a refractory illness like PG, designing trials harbors difficulty, mainly due to the rarity of the PG. Additionally, it is difficult to define outcomes in a PG study since ulcers require extensive time spans to heal, even after inflammation has subsided, as demonstrated in some of our photos (Figure 2). Cessation of the primary inflammation of PG and healing of ulcerated wounds are two distinct processes that should be evaluated separately. Wound healing is further hindered by location on the lower extremities, venous insufficiency, diabetic circulation compromise, and old age.In summary, the treatment of PG is difficult due to lack of treatment recommendations. Infliximab, prednisone, and cyclosporine are all well-known to induce rapid remission. However, given the chronicity of PG, a maintenance regimen is necessary, as up to 50% of patients require maintenance therapy to ensure remission.¹⁵ Biological therapy is an excellent therapeutic modality for PG, boasting a favorable side effect profile compared to other immunosuppressive therapies. Adalimumab is inarguably more convenient for patient preference compared to receiving infliximab infusions. In our experience, many patients will benefit from adjuvant cyclosporine to induce rapid remission and for flare ups when a patient is controlled on adalimumab. In extremely resistant cases, like the one presented in this case report, multicombination therapy may be necessary to achieve desired outcomes.

The authors have no conflict of interest to declare.