Wegener’s granulomatosis and pyoderma gangrenosum – rare causes of facial ulcerations
| Autor: | Edyta Gołembiewska, Kazimierz Ciechanowski, Magda Wiśniewska, Ewelina Staniszewska, Tomasz Smektała, Katarzyna Sporniak-Tutak, Krzysztof Sindrewicz, Marek Masiuk, Karolina Kędzierska |
|---|---|
| Jazyk: | angličtina |
| Rok vydání: | 2016 |
| Předmět: |
Microbiology (medical)
medicine.medical_specialty Cyclophosphamide Neutrophils medicine.medical_treatment lcsh:Medicine Necrosis Young Adult 030207 dermatology & venereal diseases 03 medical and health sciences 0302 clinical medicine Skin Ulcer medicine Humans dapsone Wegener Granulomatosis Hyperbaric Oxygenation business.industry Standard treatment lcsh:R Granulomatosis with Polyangiitis Immunosuppression Skin ulcer medicine.disease Comorbidity Dermatology Pyoderma Gangrenosum Infectious Diseases Face Female Plasmapheresis medicine.symptom Granulomatosis with polyangiitis business Neck 030217 neurology & neurosurgery Pyoderma gangrenosum medicine.drug |
| Zdroj: | Postępy Higieny i Medycyny Doświadczalnej, Vol 70, Pp 210-218 (2016) |
| ISSN: | 1732-2693 0032-5449 |
| Popis: | 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. Discussion 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. |
| Databáze: | OpenAIRE |
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