Features of coexisting granulomatosis with polyangiitis in a patient with pemphigus vulgaris: a case report.

Pemphigus vulgaris (PV) and granulomatosis with polyangiitis (Wegener's or GPA) are two rare autoimmune disorders. Both can involve the upper airways, and diagnosis can be difficult in the absence of extra-airway symptoms. We report the case of a patient with well-controlled PV but persistent upper respiratory tract symptoms. Further evaluation revealed perforation of the nasal septum and elevated serologies consistent with GPA. The patient improved with rituximab treatments. This case demonstrates that alternative concomitant diagnosis should be considered in patients with symptoms suggestive of recalcitrant PV and/or GPA, since these patients may require more aggressive initial treatment.