| 词条 | Pyoderma gangrenosum |
| 释义 |
| name = Pyoderma gangrenosum | image = Crohnie Pyoderma gangrenosum.jpg | caption = Pyoderma gangrenosum on the leg of a person with Ulcerative Colitis. | pronounce = | field = | synonyms = | symptoms = | complications = | onset = 40s or 50s[1] | duration = | types = | causes = | risks = | diagnosis = | differential = | prevention = | treatment = Corticosteroids, ciclosporin, infliximab, canakinumab[2] | medication = | prognosis = | frequency = | deaths = |alt=}} Pyoderma gangrenosum is a condition that causes tissue to become necrotic, causing deep ulcers that usually occur on the legs. When they occur, they can lead to chronic wounds. Ulcers usually initially look like small bug bites or papules, and they progress to larger ulcers. Though the wounds rarely lead to death, they can cause pain and scarring. Treatments may include corticosteroids, ciclosporin, infliximab, or canakinumab.[1] The disease was identified in 1930. It affects approximately 1 person in 100,000 in the population. Though it can affect people of any age, it mostly affects people in their 40s and 50s.[2] TypesThere are two main types of pyoderma gangrenosum:[2]
Other variations are:[3]
CausesThough the cause is not well understood, the disease is thought to be due to immune system dysfunction, and particularly improper functioning of neutrophils. In support of an immune cause, a variety of immune mediators such as IL-8, IL-1β, IL-6, interferon (IFN)-γ, G-CSF, TNF, matrix metallopeptidase (MMP)-9, MMP-10, and Elafin have all been reported to be elevated in patients with pyoderma gangrenosum.[6] Also in support of an immune cause is the finding that at least half of all pyoderma gangrenosum patients suffer from immune-mediated diseases.[2] For instance, ulcerative colitis, rheumatoid arthritis, and multiple myeloma (MM) have all been associated with pyoderma gangrenosum. It can also be part of a syndromes such as PAPA syndrome. One hallmark of pyoderma gangrenosum is pathergy, which is the appearance of new lesions at sites of trauma, including surgical wounds.[7] AssociationsThe following are conditions commonly associated with pyoderma gangrenosum:[8]
DiagnosisDiagnosis of PG is challenging owing to its variable presentation, clinical overlap with other conditions, association with several systemic diseases, and absence of defining histopathologic or laboratory findings. Misdiagnosis and delayed diagnosis are common. It has been shown that up to 39% of patients who initially received a diagnosis of PG have an alternative diagnosis.[10] In light of this, validated diagnostic criteria have recently been developed for ulcerative pyoderma gangrenosum.[11] Diagnostic CriteriaIn addition to a biopsy demonstrating a neutrophilic infiltrate, patients must have at least 4 minor criteria to meet diagnostic criteria.[11] These criteria are based on histology, history, clinical examination and treatment.
TreatmentFirst-line therapy for disseminated or localized instances of pyoderma gangrenosum is systemic treatment by corticosteroids and ciclosporin. Topical application of clobetasol, mupirocin, and gentamicin alternated with tacrolimus can be effective. Pyoderma gangrenosum ulcers demonstrate pathergy, that is, a worsening in response to minor trauma or surgical debridement. Significant care should be taken with dressing changes to prevent potentially rapid wound growth. Many patients respond differently to different types of treatment, for example some benefit from a moist environment, so treatment should be carefully evaluated at each stage. Papules that begin as small "spouts" can be treated with Dakin's solution to prevent infection and wound clusters also benefit from this disinfectant. Wet to dry applications of Dakins can defeat spread of interior infection. Heavy drainage can be offset with Coban dressings. Grafting is not recommended due to tissue necrosis. If ineffective, alternative therapeutic procedures include systemic treatment with corticosteroids and mycophenolate mofetil; mycophenolate mofetil and ciclosporin; tacrolimus; thalidomide; infliximab; or plasmapheresis.[12] See also
References1. ^1 {{cite journal |last1=Partridge |first1=ACR |last2=Bai |first2=JW |last3=Rosen |first3=CF |last4=Walsh |first4=SR |last5=Gulliver |first5=WP |last6=Fleming |first6=P |title=Effectiveness of systemic treatments for pyoderma gangrenosum: a systematic review of observational studies and clinical trials. |journal=The British Journal of Dermatology |date=August 2018 |volume=179 |issue=2 |pages=290–295 |doi=10.1111/bjd.16485 |pmid=29478243}} 2. ^1 2 3 {{cite journal |first1=J Mark |last1=Jackson |first2=Jeffrey P |last2=Callen |editor1-first=Dirk M |editor1-last=Elston |date=April 23, 2012 |url=http://www.emedicine.com/DERM/topic367.htm |journal=eMedicine |title=Pyoderma Gangrenosum}} 3. ^{{cite journal |doi=10.1136/bmj.333.7560.181 |pmc=1513476 |title=Diagnosis and treatment of pyoderma gangrenosum |year=2006 |last1=Brooklyn |first1=T. |journal=BMJ |volume=333 |issue=7560 |pages=181–4 |pmid=16858047 |last2=Dunnill |first2=G |last3=Probert |first3=C}} 4. ^{{cite journal |doi=10.1046/j.1365-2230.2003.01418.x |title=Pustular pyoderma gangrenosum |year=2003 |last1=Shankar |first1=S. |last2=Sterling |first2=J. C. |last3=Rytina |first3=E. |journal=Clinical and Experimental Dermatology |volume=28 |issue=6 |pages=600–3 |pmid=14616824}} 5. ^{{cite journal |doi=10.1111/j.1365-4632.2005.02591.x |title=Vegetative pyoderma gangrenosum: A report of two new cases and a review of the literature |year=2005 |last1=Langan |first1=Sinead M. |last2=Powell |first2=Frank C. |journal=International Journal of Dermatology |volume=44 |issue=8 |pages=623–9 |pmid=16101860}} 6. ^{{cite journal |vauthors=Patel F, Fitzmaurice S, Duong C, He Y, Fergus J, Raychaudhuri SP, Garcia MS, Maverakis E | title = Effective strategies for the management of pyoderma gangrenosum: a comprehensive review. | journal = Acta Derm Venereol | volume = 95 | issue = 5 | pages = 525–31 | year = 2015 | pmid = 25387526 | doi = 10.2340/00015555-2008}} 7. ^{{cite journal |doi=10.1111/j.1468-3083.2008.02626.x |title=Seat belt pyoderma gangrenosum: Minor pressure as a causative factor |year=2008 |last1=Rashid |first1=RM |journal=Journal of the European Academy of Dermatology and Venereology |volume=22 |issue=10 |pages=1273–4 |pmid=18837131}} 8. ^{{cite journal|last=Brooklyn|first=Trevor|author2=Giles Dunnill |author3=Chris Probert |title=Diagnosis and treatment of pyoderma gangrenosum|journal=British Medical Journal|year=2006|volume=333|issue=7560|pages=181–184|doi=10.1136/bmj.333.7560.181|pmc=1513476|pmid=16858047}} 9. ^{{cite journal|last=Tendas|first=Andrea|author2=Niscola P|author3=Barbati R|author4=Abruzzese E|author5=Cuppelli L|author6=Giovannini M|author7=Scaramucci L|author8=Fratoni S|author9=Ales M|author10=Neri B|author11= Morino L|author12=Dentamaro T|author13=De Fabritiis P|title=Tattoo related pyoderma/ectyma gangrenous as presenting feature of relapsed acute myeloid leukaemia: an exceptionally rare observation.|journal=Injury|date=May 2011|volume=42|issue=5|pages=546–7|doi=10.1016/j.injury.2010.08.014|pmid=20883993}} 10. ^{{Cite journal|last=Weenig|first=Roger H.|last2=Davis|first2=Mark D. P.|last3=Dahl|first3=Patrick R.|last4=Su|first4=W. P. Daniel|date=2002-10-31|title=Skin ulcers misdiagnosed as pyoderma gangrenosum|journal=The New England Journal of Medicine|volume=347|issue=18|pages=1412–1418|doi=10.1056/NEJMoa013383|issn=1533-4406|pmid=12409543}} 11. ^1 {{Cite journal|last=Maverakis|first=Emanual|last2=Ma|first2=Chelsea|last3=Shinkai|first3=Kanade|last4=Fiorentino|first4=David|last5=Callen|first5=Jeffrey P.|last6=Wollina|first6=Uwe|last7=Marzano|first7=Angelo Valerio|last8=Wallach|first8=Daniel|last9=Kim|first9=Kyoungmi|title=Diagnostic Criteria of Ulcerative Pyoderma Gangrenosum|url=https://jamanetwork.com/journals/jamadermatology/fullarticle/2672268|journal=JAMA Dermatology|volume=154|issue=4|pages=461–466|language=en|doi=10.1001/jamadermatol.2017.5980|pmid=29450466|date=April 2018}} 12. ^{{cite journal |doi=10.1016/j.jaad.2004.10.006 |title=Treatment recommendations for pyoderma gangrenosum: An evidence-based review of the literature based on more than 350 patients |year=2005 |last1=Reichrath |first1=Jörg |last2=Bens |first2=Guido |last3=Bonowitz |first3=Anette |last4=Tilgen |first4=Wolfgang |journal=Journal of the American Academy of Dermatology |volume=53 |issue=2 |pages=273–83 |pmid=16021123}} External links{{Medical resources| DiseasesDB = 11064 | ICD10 = {{ICD10|L|88||l|80}} | ICD9 = {{ICD9|686.01}} | ICDO = | OMIM = | MedlinePlus = | eMedicineSubj = article | eMedicineTopic = 1123821 | MeshID = D017511 | Orphanet = 48104 }}{{Commons category|Pyoderma gangrenosum}}{{Reactive neutrophilic cutaneous conditions}}{{Cutaneous ketatosis, ulcer, atrophy, necrobiosis, and vasculitis}}{{Paraneoplastic syndromes}} 4 : Reactive neutrophilic cutaneous conditions|Rare diseases|Gangrene|Necrosis |
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