Acne fulminans in a young man with granulomatosis with polyangiitis (Wegener's granulomatosis): A chance association or marker of serious systemic disease?

A 19-year-old man with granulomatosis with polyangiitis (Wegener's disease) presented with hemorrhagic facial nodules mimicking severe inflammatory acne (acne fulminans) as one of the first symptoms of the disease. The lesions were earlier treated as nodulocystic acne with isotretinoin without any benefit. Complete resolution was seen with pulsed methylprednisolone and oral prednisolone and mycophenolate mofetil thereafter. He also developed acute onset of severe pustular eruption of the face and a destructive ulcer of the auricle on two separate occasions. Facial lesions mimicking severe inflammatory acne, not responsive to standard treatment, may be a marker for more severe systemic disease such as Wegener's disease/granulomatosis with polyangiitis.