Introduction:
A coexistence of pyoderma gangrenosum (PG) and granulomatosis with polyangiitis (GPA) is observed extremely rarely. Some authors believe that skin ulcers like-PG may be a cutaneous manifestation of the GPA. They highlight the difficulties in assessing the histopathological picture: lack of clear diagnostic criteria for PG and recommendation to repeat biopsy in order to determine the proper diagnosis.

Aim:
This article presents the case of 41-year-old man with GPA preceded with PG.

Case study:
We present the case of 41-year-old male with GPA (with the occurrence of bleeding into the alveoli, bleeding from the lower gastrointestinal tract and renal involvement) preceded with PG. He also had pathergy test positive. The patient was treated with cyclophosphamide and corticosteroid with improvement.

Results and discussion:
The presence of c-anti-neutrophil cytoplasmic antibodies (c-ANCAs) levels, despite the classic image of PG with a characteristic histology, requires special observation of patients who may develop systemic GPA. Such patients should have frequent assessments of internal organ involvement. ANCAs control is recommended since their concentrations correlate with disease activity. They may affect the re-activation and chemotaxis of neutrophils.

Conclusions:
The presence of c-ANCAs levels, despite the classic image of PG with a characteristic histology, requires special observation of patients who may develop systemic GPA.