Some conflicting reports have appeared on the effectiveness of using RTX for the granulomatous versus vasculitic manifestations of GPA. Aires et al13 showed failure of improvement in most patients with granulomatous manifestations of GPA in a study of 8 patients. A retrospective study including 59 patients who were treated with RTX also showed that complete remission was more common in patients with primarily vasculitic manifestations, not granulomatous manifestations.14 However, some case series that included patients with refractory ophthalmic GPA, a primarily granulomatous manifestation, have found success using RTX.15,16 More studies are needed to determine if there truly is a difference and whether this difference has an effect on when to use RTX. The skin lesions our patient demonstrated were due to the vasculitic component of the disease, and consequently, the rapid and complete response we observed would be consistent with the premise that the therapy works best for vasculitis.
Most of the trials assessing the efficacy of RTX utilize a tool such as the Wegener granulomatosis-specific Birmingham Vasculitis Activity Score.17 This measure of treatment response does include a skin item, but it is part of the composite response score. Consequently, a specific statement regarding skin improvement cannot be made. Additionally, little is reported pertaining to the treatment of skin-related findings in GPA. One report did specifically address the treatment of dermatologic manifestations of GPA utilizing systemic tacrolimus with oral prednisone successfully in 1 patient with GPA and a history of recurrent lower extremity nodules and ulcers.18 The efficacy of RTX in limited GPA was good in a small study of 8 patients. However, the study had only 1 patient with purpura and 1 patient with a subcutaneous nodule.19 Several other case series and studies have included patients with various cutaneous findings associated with GPA.5-7,9,11 However, they did not comment specifically on skin response to treatment, and the focus appeared to be on other organ system involvement. One case series did report improvement of lower extremity gangrene with RTX therapy for ANCA-associated vasculitis.8 Our report demonstrates a case of severe skin disease that responded well to RTX. It is common to have various skin findings in GPA, and our patient presented with notable skin disease. Although skin findings may not be the more life-threatening manifestations of the disease, they can be quite debilitating, as shown in our case report.
Our patient with notable leg ulcerations required hospitalization due to GPA and received RTX in addition to corticosteroids for treatment. We observed a rapid and dramatic improvement in the skin findings, which seemed to exceed expectations from steroids alone. The other manifestations of the disease including lung nodules also improved. Although cyclophosphamide and corticosteroids have been quite successful in induction of remission, cyclophosphamide is not without serious adverse effects. There also are some patients who have contraindications to cyclophosphamide or do not see successful results. In our brief review of the literature, RTX, a B cell–depleting antibody, has shown to have success in treating refractory and severe GPA. There is little reported specifically about treating the skin manifestations of GPA. A few studies and case reports mention skin findings but do not comment on the success of RTX in treating them. Although the severity of other organ involvement in GPA may take precedence, the skin findings can be quite debilitating, as in our patient. Patients with GPA and notable skin findings may benefit from RTX, and it would be beneficial to include these results in future studies using RTX to treat GPA.