Wegener's granulomatosis and pyoderma gangrenosum--rare causes of facial ulcerations.
BACKGROUND
Pyoderma gangrenosum (PG) is caused by immune system dysfunction, and particularly improper functioning of neutrophils. At least half of all PG patients also suffer from autoimmunological diseases, one of which is Wegener granulomatosis (WG). The purpose of this article was to compare cases of patients with WG and PG in terms of their clinical course, histopathology, and applied treatment. In both, histopathological features are not fully distinct. Data from microbiological and immunological evaluation and clinical presentation are required to establish the diagnosis. We also present the case of a patient with WG and deep facial skin lesions not responding to standard treatment.


METHODS
Systematic review of the literature in PubMed using the search terms "Wegener granulomatosis AND Pyoderma gangrenosum" and case report.


RESULTS
The finding of 22 reports in the literature (PubMed) suggests that it is a rare phenomenon. This study revealed a similar rate of comorbidity of WG and PG in both genders and an increased incidence of both diseases after the age of 50. Among skin lesions there was a dominance of ulceration, most often deep and painful, covering a large area with the presence of advanced necrosis and destruction of the surrounding tissue. The most common location proved to be the cervical-cephalic area. The most popular treatment included steroids with cyclophosphamide.


CONCLUSIONS
The rarity of the coexistence of these two diseases results in a lack of effective therapy. In such cases sulfone derivatives are still effective and provide an alternative to standard immunosuppression methods. Hyperbaric therapy and plasmapheresis can also play an important complementary role.
Department of Nephrology, Transplantology and Internal Medicine, Pomeranian Medical University, Szczecin, Poland.
Karolina
K
Kędzierska
Department of Maxillofacial Surgery, Pomeranian Medical University, Szczecin, Poland.
Krzysztof
K
Sindrewicz
Department of Maxillofacial Surgery, Pomeranian Medical University, Szczecin, Poland.
Tomasz
T
Smektała
Department of Nephrology, Transplantology and Internal Medicine, Pomeranian Medical University, Szczecin, Poland.
Magda
M
Wiśniewska
Department of Pathomorphology, Pomeranian Medical University, Pomeranian Medical University, Szczecin, Poland.
Marek
M
Masiuk
Department of Maxillofacial Surgery, Pomeranian Medical University, Szczecin, Poland.
Ewelina
E
Staniszewska
Department of Maxillofacial Surgery, Pomeranian Medical University, Szczecin, Poland.
Katarzyna
K
Sporniak-Tutak
Department of Nephrology, Transplantology and Internal Medicine, Pomeranian Medical University, Szczecin, Poland.
Edyta
E
Gołembiewska
Department of Nephrology, Transplantology and Internal Medicine, Pomeranian Medical University, Szczecin, Poland.
Kazimierz
K
Ciechanowski
D005145:Face; D005260:Female; D014890:Granulomatosis with Polyangiitis / Q000276:immunology / Q000473:pathology* / Q000628:therapy; D006801:Humans; D009333:Neck; D009336:Necrosis / Q000276:immunology / Q000473:pathology* / Q000628:therapy; D009504:Neutrophils / Q000276:immunology; D017511:Pyoderma Gangrenosum / Q000276:immunology* / Q000473:pathology* / Q000628:therapy; D012883:Skin Ulcer / Q000276:immunology / Q000473:pathology* / Q000628:therapy; D055815:Young Adult