Cutaneous Alternariosis in a Patient with Pulmonary Granulomatosis with Polyangiitis (GPA): Is It a Marker of Immunosuppression or Immune Dysregulation?

Granulomatosis with polyangiitis (GPA) is a rare multisystem disorder of autoimmune pathology involving small- and medium-vessel vasculitis of respiratory and renal system. Immune dysregulation due to GPA illness and immunosuppression after use of immunomodulators predisposes to opportunistic secondary infections. We report a 43-year-old male who presented with acute respiratory distress and acute hypoxic respiratory failure, skin lesions and multifocal nodules with radiological bronchus sign on high-resolution computed tomography thorax imaging and negative workup for sepsis screen on microbiological workup. He was diagnosed as GPA after positive anti C-antineutrophil cytoplasmic antibody test. Skin lesions needle aspirate for potassium hydroxide mount showed fungal aetiology. Punch biopsy for histopathology and culture with fungal polymerase chain reaction analysis finally confirmed the diagnosis of cutaneous alternariosis. Combination regimen of methylprednisolone, azathioprine and itraconazole was used for the management of GPA with cutaneous alternariosis. We observed remission of GPA and documented complete resolution of lung and skin lesions. We recommend a high index of suspicion during management of GPA with concurrent fungal infection for successful treatment outcome.