L. Anderson, W. Cranwell, L. Scardamaglia, V. Morgan, G. Varigos
Granulomatosis with polyangiitis (GPA), formerly known as Wegener granulomatosis, is a multisystem autoimmune disease primarily affecting the respiratory tract and kidneys. Skin involvement, including papules, vesicles, subcutaneous nodules, and most often palpable purpura, occurs in up to 50% of cases. A rare cutaneous manifestation is ulceration resembling pyoderma gangrenosum, often with non-specific histology. Although oral cyclophosphamide in combination with high-dose glucocorticoids has long been standard treatment, effective treatment for refractory cases remains uncertain due to the rare co-existence of GPA and PG.
We present a case of a 35-year-old Caucasian male with chronic relapsing cephalic PG-like lesions associated with antineutrophil cytoplasmic antibody (cANCA) positive GPA. Repeat skin biopsies showed no features consistent with cutaneous GPA or PG, with nonspecific acute and chronic inflammation. Despite longstanding inactive GPA, the PG-like ulcers failed to respond to various immunosuppressive agents including prednisolone, cyclophosphamide, azathioprine and cyclosporine. The patient denied manipulating or picking at the ulcers. The patient was treated as an inpatient with oral antibiotics for secondary infection, a weaning course of high-dose prednisolone, and mycophenolate mofetil (MMF) up to 1.5 mg BD. The ulcers had significantly regressed three months later.
The co-existence of GPA and PG is rare, particularly under the age of 50 and in a cervical-cephalic distribution. Thus, there is a paucity of research regarding effective management options for chronic non-healing ulcers in this context. Our case highlights the successful use of high-dose systemic glucocorticoids and MMF for treatment of refractory cephalic PG-like lesions in a patient with GPA.