Granulomatosis with polyangiitis presenting with facial nerve paralysis and strawberry gingivitis in a child

Abstract

Granulomatosis with Polyangiitis (GPA), formally termed Wegener's Granulomatosis, is characterized by necrotizing granulomatous inflammation affecting the upper and lower respiratory tract and the kidneys. We report a case of a 10-year-old female who presented with a complete right facial paralysis preceded by a 6-week history of intermittent fever, headaches, and a right-sided otorrhea. She was admitted for urgent placement of bilateral tympanostomy tubes and intravenous antibiotics. Despite maximal medical management, she had recurrent fever and was noted to have intermittent epistaxis and gum bleeding with erythematous gingival lesions resembling strawberries in appearance. Gingival biopsy combined with other clinical features confirmed the diagnosis of GPA. Strawberry gingivitis, a reddish-purple exophytic gingival swelling, is a rare presenting feature of GPA; however, when present, it is almost pathognomonic. Early recognition of this clinical feature is critical in establishing a timely diagnosis.

Keywords

• Granulomatosis with polyangiitis
• Wegener's granulomatosis
• Strawberry gingivitis
• Facial nerve paralysis