Pyoderma Gangrenosum (Pathergy Trap): Kukula Mofulumizitsa Zilonda Zowonongeka Zowonongeka ndi Chifukwa Chake Kuwonongeka Kwa Opaleshoni Kumayipitsa Necrosis¶

Pyoderma gangrenosum (PG) ndi osowa, osapatsirana, autoinflammatory neutrophilic dermatosis momwe kusakhazikika kwa chitetezo chamthupi kumayendetsa ma neutrophils okhwima, osabala a polymorphonuclear mu dermis, kusungunula kolajeni ndi minofu yocheperako mkati mwa maola angapo mpaka masiku. Ngakhale kuti dzina lake lonyenga la mbiri yakale linapangidwa mu 1930, pyoderma gangrenosum ndi ** osati matenda a bakiteriya ("pyoderma") kapena matenda enieni a ischemic vascular gangrene **. M'malo mwake, imayamba ngati papule yotupa, wosabala pustule, kapena hemorrhagic vesiculopustule, yomwe nthawi zambiri imayambika pamalo enieni a khungu laling'ono, kulumidwa ndi tizilombo, venipuncture, kapena opaleshoni, chinthu chodziwika bwino chomwe chimadziwika kuti **cutaneous pathergy ** (omwe alipo 50 peresenti ya odwala 30). Pakadutsa maola 24 mpaka 72, pustule yosabala imasweka pakati ndikumakulirakulira mwachangu (1 mpaka 2 centimita patsiku) kukhala yowawa kwambiri, purulent, necrotic crater yozungulira ndi pathognomonic boggy, tople, tople-blue-blue, malire ozunguliridwa ndi kuwala kowala kofiira. Pakati pa 50 ndi 70 peresenti ya odwala omwe ali ndi pyoderma gangrenosum ali ndi matenda oyambitsa matenda, nthawi zambiri Matenda Otupa (Ulcerative Colitis kapena Crohn Disease), Rheumatoid Arthritis kapena Seronegative Spondyloarthritis, Monoclonal Gammopathy (makamaka, Hematoid Igmopathy) (Acute Myeloid Leukemia kapena Myelodysplastic Syndrome). Chifukwa chilonda chomwe chikuchulukirachulukira komanso chiwopsezo, kupweteka kwambiri, komanso kuchuluka kwa maselo oyera amagazi a 20,000 mpaka 40,000/mcL kumatsanzira kwambiri necrotizing fasciitis (matenda a bakiteriya odya nyama) kuwononga. Mu pyoderma gangrenosum, **kugunda kulikonse kwa scalpel ya dokotalayo kumayambitsa mkuntho wophulika wa neutrophil m'mphepete mwa khungu lomwe langodulidwa kumene **, zomwe zimapangitsa kuti chilondacho chichuluke kuwirikiza kawiri kapena katatu usiku umodzi, kuwonetsa minyewa ndi fupa, kapena kupangitsa kudula mwendo kosafunikira. Ngati wodwala ayamba zilonda zomwe zikuchulukirachulukira ndi zofiirira zomwe zimachepa m'malire atavulala pang'ono kapena opaleshoni, imbani foni kwanuko. manambala angozi kapena funsani mwamsanga dermatology kuchipatala mwamsanga. Onaninso maupangiri a abale athu pa calciphylaxis, violaceous retiform plaques, ndi sodium thiosulfate, erysipelas vs necrotizing fasciitis vs stasis dermatitis triage,ndi Mkazi wamasiye wakuda latrodectism vs bulauni recluse loxoscelism necrotic ulcer triage.

Zomwe mukufunikira mwamsanga ndi kuyika chenjezo la "OSATI KUCHITA OPANDA OPANDA" pa tchati cha wodwalayo ndi kuvala zilonda , ** kusiyanitsa pyoderma gangrenosum ndi matenda enieni a necrotizing soft-tissue pogwiritsa ntchito kuyezetsa kwa bedi, banga la Gram, zikhalidwe za minofu, ndi PHACELSPERIFOD yotsimikizika elliptical kapena punch skin biopsy ya malire a violaceous (pokhapokha ngati pakufunika kuti asatengere matenda, vasculitis, kapena malignancy, komanso poyambitsa chitetezo chamthupi kuti chiwononge biopsy pathergy), ndi **kuyamba kuchitapo kanthu mwachangu anti-neutrophil immunosuppression , makamaka Corticosteroid2 Precodnisosneic mg/kg/tsiku kapena pulse IV Methylprednisolone 500 mpaka 1,000 mg/tsiku kwa masiku atatu), oral Cyclosporine (4 mpaka 5 mg/kg/tsiku), kapena **intravenous Infliximab (5 mg/kg, muyezo wa golide wa Crohn/Ulcerative PG ndi Colperitis)*-

Chitani izi tsopano¶

1. Fufuzani Urgent Hospital Emergency and Dermatology Evaluation ngati pustules pakhungu, zilonda zapakhungu, kapena zilonda zazing'ono zimalowa m'phanga lopweteka kwambiri lomwe lili ndi malire apepo-buluu, otambalala, opindika. Imbani 911 ku United States ndi Canada, 999 ku United Kingdom, 112 ku United Kingdom, 112 ku European Union, 112 manambala angozi.
2. Imitsani Kuwonongeka Kwakukulu Kulikonse Komwe Kwakonzedweratu Kapena Kudula Kwambiri Nthawi Yomwe (The Pathergy Trap): Ngati wodwala adziwa kapena akukayikira kwambiri pyoderma gangrenosum (kuphatikiza Postoperative Pyoderma Gangrenosum [PPG] kuwoneka patatha masiku 4 mpaka 14 pambuyo pochepetsa mabere / kumangidwanso, gawo lopangira opaleshoni, opaleshoni yamtima kupangidwa kwa stoma), ** kuwononga koopsa kwa opaleshoni ndi scalpel kapena curette kumatsutsana kwambiri **. Kudula maopaleshoni kumabweretsa mafunde akulu a IL-1beta-, IL-8-, ndi ma neutrophils oyendetsedwa ndi TNF-alpha kupita ku dermis yatsopano, kumayambitsa zilonda zam'mimba pachifuwa chonse, pamimba, kapena miyendo mkati mwa maola 24.
3. ** Chotsani Choonadi cha Necrotizing Fasciitis, Ecthyma Gangrenosum, ndi Matenda Afungal Ozama Popanda Kuyambitsa Kuchotsa Mosafunikira: **
4. Chifukwa Chimene Madokotala Ochita Opaleshoni Amapusitsidwa: Pyoderma gangrenosum imayambitsa purulent exudate yachikasu-yoyera (yopangidwa ndi mamiliyoni a ma neutrophils akufa), kutentha kwakukulu, ndi leukocytosis mpaka 35,000/mcL.
5. ** Momwe Mungawadziwikire Pambali pa Bedi: ** Mosiyana ndi necrotizing fasciitis (yomwe imafalikira mozama motsatira minofu ya subcutaneous fascia * patsogolo * ya kuwonongeka kwa khungu, imatulutsa mpweya wochepa kwambiri / crepitus, madzi onyansa a "madzi" amadzimadzi, ndi systemic septic shock ndi hypotension ndi lactic acidosis ), ** pyodermallydemia ndi chotupa chachikulu cha pyodermal fungo loipa kwambiri, mfuti zamfuti-ziwopsezo zakuwonongeka **, **ziro subcutaneous gas **, **ziro fungo loipa la anaerobic **, kutuluka kwapang'onopang'ono, ndi madontho a Gram opanda zikhalidwe ndi zikhalidwe zamagazi.
6. Chidziwitso cha PG pambuyo pa opaleshoni: Matenda a mabakiteriya otchedwa postoperative necrotizing bala nthawi zambiri amayamba mkati mwa 24 mpaka maola 72* atachitidwa opaleshoni (Gulu A Streptococcus kapena Clostridium) kapena amawoneka ngati chiphuphu chosinthasintha, pamene Postoperative Pyoderma Gangrenosumally 4 masiku ang'onoang'ono op-4 ma pustules osabala pamzere wa suture omwe amalumikizana ndikuchepetsa kung'ambika konse pomwe kuteteza areola yotuwa pa opaleshoni ya bere ndikulephera kusintha maantibayotiki ambiri amtundu wa IV.
7. Ikani Njira Zowunikira Zovomerezeka (The Delphi International Consensus Criteria ndi PARACELSUS Score) ndi Kupeza Biopsy Yoyang'aniridwa:
8. Pansi pa **2018 Delphi International Consensus **, kufufuza kumafuna ** 1 Major Criterion ** (biopsy of the ulcer edge kusonyeza ** dense sterile neutrophilic dermal infiltrate **) kuphatikizapo ** osachepera 4 a 8 Zing'onozing'ono ** (kupatulapo matenda pa histology / chikhalidwe; pathergy; chilonda chotupa / kutupa kwa masiku 4; matenda a nyamakazi; zotumphukira erythema, kufooketsa malire, ndi zilonda zambiri ndi chimodzi pa mwendo m'munsi mwa mwendo kapena "wophwanyika mapepala" makwinya * * pa malo ochiritsidwa;
9. Pansi pa PARACELSUS Score (pomwe mphambu ya >= 10 points ikuwonetsa > 90% kuthekera kwa PG), mfundo zazikulu zimaperekedwa ku **P ** rogressing matenda (3 pts), Akuwunika koyenera kuzindikiridwa kosiyana (3 pts), ndi Reddish-violaceous woundant (3 ptsmeli) Cmawonekedwe a zilonda zam'mimba (2 pts), Ekuwawa koopsa> 4/10 pa VAS (2 pts), ndi Localized pathergy phenomenon (2 pts).
10. Yambitsani High-Dose Systemic Corticosteroids kapena Cyclosporine Pompo Kuti Muyimitse Mkuntho wa Neutrophil:
11. Kupulumutsidwa Kwadongosolo Kwambiri Kwambiri (Umboni Woyeserera wa STOP GAP): Yang'anirani Oral Prednisolone/Prednisone pa 0.75 mpaka 1.5 mg/kg/tsiku (mpaka 60 mpaka 100 mg/tsiku), kapena pakukula, kukulirakulira mwachangu kwa multilocular or postoperative Mentrathy (500 ku 1,000 mg IV kamodzi tsiku lililonse kwa 3 mpaka 5 masiku otsatizana) **, zomwe zimamanga kukula kwa malire a centrifugal ndi kuchepetsa ululu wopweteka mkati mwa **48 kwa maola 72 **.
12. ** Njira Yopulumutsira Mzere Woyamba (Oral Cyclosporine):** Chizindikiro cha UK ** STOP GAP mayesero olamulidwa mwachisawawa ** adatsimikizira kuti Oral Cyclosporine pa 4 mpaka 5 mg/kg/tsiku (yogawika kawiri tsiku lililonse) imakhala ndi mphamvu yochiritsa ya zilonda zofananira komanso kuthamanga kwa oral prednisolosporine ndi odwala omwe ali ndi Cyclosporine yoyamba. matenda a shuga, osteoporosis aakulu, kunenepa kwambiri, kapena steroid psychosis (kupewa Cyclosporine mu kulephera kwaimpso kapena kuthamanga kwa magazi kosalamulirika).
13. Yambani Biologic TNF-Alpha Blockade (IV Infliximab 5 mg/kg) ya Inflammatory Bowel Disease-Associated, Peristomal, kapena Steroid-Refractory Pyoderma Gangrenosum: *Intravenous Infliximab (5 mg/kg pa Masabata 0, 2, ndi 8) yothandizidwa ndi biotherapy kuyesa koyendetsedwa ndi placebo kwa pyoderma gangrenosum, kupeza mayankho ofulumira azachipatala mu ** 69 peresenti ya odwala mkati mwa masabata a 2 ** ndikugwira ntchito ngati chithandizo chosatsutsika chosankha pamene PG imakhala ndi matenda a Crohn, Ulcerative Colitis, kapena peristomal ulceration.
14. Yesetsani Kwambiri Kusamalira Mabala a M'deralo Mogwirizana ndi Chinyezi komanso Kuponderezana Mofatsa: Osapaka ulusi wouma womwe umamatirira pa bedi la zilonda ndikung'amba dermis pochotsa (kuyambitsa kupweteka). Tetezani khungu lozungulira lozungulira ndi zinc oxide kapena cyanoacrylate barrier film, pakani mafuta amphamvu kwambiri atopical clobetasol propionate 0.05% kapena tacrolimus 0.1% mafuta onunkhira molunjika pamalire akuwotcha a violaceous overbarrier, valani zomatira zapakati ndi ** orginate hydrochloride, silikoni ya orginate, orginate hydrochloride kuvala **, ndikuyika ** bandeji yofatsa yokhala ndi mitundu ingapo ** kamodzi kupweteka kwakukulu kumalire kumayendetsedwa ndi systemic steroids.

Mtengo wachigamulo: wokayikira Pyoderma Gangrenosum vs Necrotizing Fasciitis vs Venous / Arterial Ulcer vs Martorell Ulcer¶

flowchart TD
    A["Wodwala Amapereka Chilonda Chokulirapo Mwachangu, Chowawa Kwambiri (1 mpaka 2 cm/tsiku) + Purulent Yellow Base + Boggy Violaceous/Gunmetal-Gray Undermined Overhanging Border (Nthawi zambiri Pambuyo Pakuvulala Kwakung'ono, Venipuncture, kapena Opaleshoni Yaposachedwa)"] --> B{"Khwerero 1: Kutuluka Mwamsanga kwa Matenda Opatsira Ofewa a Necrotizing (Necrotizing Fasciitis): Kodi Pali Gasi / Crepitus, Fungo Loipa la Anaerobic, Fascial Tracking Beyond Skin Margins, kapena Septic Shock?"}
    B -->|YES: True Bacterial Necrotizing Fasciitis / Wet Gangrene| C["Kufufuza Kwa Opaleshoni Yadzidzidzi & Kuwononga + IV Vancomycin + Piperacillin-Tazobactam/Meropenem + Clindamycin"]
    B -->|NO: Primary Dermal Ulceration with Violaceous Undermined Overhang + Negative Gram Stain + Palpable Pulses| D["Khwerero 2: Ikani PARACELSUS Score (>= 10 Points) & Screen for Systemic Associations: IBD (Ulcerative Colitis / Crohn), Rheumatoid Arthritis, IgA Gammopathy / AML / MDS, kapena Levamisole-Adulterated Cocaine Use"]
    D --> E["KUYAMBIRA KWACHITETEZO CHOFUNIKA: Ikani 'STRICT PATHERGY PRECAUTIONS - POPANDA KUTITSA OPANDA OPANDA OPANDA OPANDA, POPANDA DRY ADHERENT GAUZE' Sign at Bedside"]
    E --> F{"Khwerero 3: Chitani Mphepete mwa Mphepete mwa Biopsy (Phatikizanipo Violaceous Border + Subcutaneous Fat) kwa H & E Histopathology + Bacterial, Fungal & Mycobacterial Tissue Cultures"}
    F --> G{"Unikani Kuopsa kwa Matenda, Kuchuluka kwake & Zomwe Zimayambitsa Matenda Ophatikizana"}
    G -->|Severe, Rapidly Expanding, Multilocular, or Postoperative PG| H["ZOCHITIKA ZOTHANDIZA ZOTHANDIZA: 1. Oral Prednisone (1 mpaka 1.5 mg / kg / tsiku) OR Pulse IV Methylprednisolone (500 kwa 1,000 mg / tsiku x 3-5 masiku); OR 2. Oral Cyclosporine (4 mpaka 5 mg/kg/tsiku - STOP GAP Trial Equivalent)"]
    G -->|Coexisting Inflammatory Bowel Disease (Crohn / UC), Peristomal PG, or Steroid-Refractory Disease| I["BIOLOGIC RESCUE: Intravenous Infliximab (5 mg/kg pa Masabata 0, 2, 6) kapena Adalimumab + Systemic Corticosteroid Bridge"]
    G -->|Underlying Hematologic Malignancy (AML / MDS / IgA Myeloma) or Severe Sepsis Contraindicating Steroids| J["High-Dose Intravenous Immunoglobulin (IVIG 2 g/kg pamasiku 2-5) kapena Wolimbana ndi IL-1 (Anakinra / Canakinumab) + Thandizani Underlying Leukemia/Myeloma"]
    G -->|Small (< 3 cm), Early, Localized, or Mild Peristomal Ulcer| K["Clobetasol Yamphamvu Kwambiri 0.05% Mafuta kapena Tacrolimus Yapamwamba 0.1% Yogwiritsidwa Ntchito Kumalire A Violaceous + Osamala Intralesional Triamcinolone (40 mg/mL) + Zovala Zama Silicone Osatsatira"]

Pathophysiology: mkuntho wosabala wa neutrophil, pathergy wakhungu, ndi mitundu isanu yachipatala ya Pyoderma Gangrenosum.¶

Pyoderma gangrenosum imakhala kumapeto kwa zilonda zam'mimba za ** neutrophilic dermatoses spectrum ** (pamodzi ndi ** Sweet syndrome **, ** subcorneal pustular dermatosis **, ** hidradenitis suppurativa **, ndi ** erythema elevatum diutinum **).

HOW MINOR TRAUMA TRIGGERS EXPLOSIVE PATHERGIC ULCERATION IN PG
=======================================================================
[Minor Cutaneous Trauma: Needle Stick, Bug Bite, Scalpel Incision]
                               |
                               v
[Keratinocyte Damage Releases DAMPs (Damage-Associated Molecular
 Patterns: ATP, HMGB1, S100 Proteins, LL-37 Cathelicidin)]
                               |
                               v
[Hyper-Reactive NLRP3 Inflammasome Activation in Dermal Macrophages]
   * Massive local surge of Interleukin-1 beta (IL-1beta), IL-1alpha,
     Tumor Necrosis Factor-alpha (TNF-alpha), IL-8 (CXCL8), IL-17,
     and Granulocyte Colony-Stimulating Factor (G-CSF).
                               |
                               v
[STERILE NEUTROPHIL AVALANCHE INTO THE DERMIS]
   * Millions of mature neutrophils flood the dermis and release
     Matrix Metalloproteinases (MMP-9, MMP-2), Neutrophil Elastase,
     Myeloperoxidase, and Neutrophil Extracellular Traps (NETs).
                               |
                               v
[LIQUEFACTIVE DERMAL COLLAGEN MELTDOWN ("Undermining")]
   * Because neutrophils dissolve the deep reticular dermis faster
     than the overlying epidermis dies, the dermal floor caves in
     beneath the skin edge -> Creates the classic BOGGY, VIOLACEOUS,
     OVERHANGING ("UNDERMINED") BORDER + Cribriform "Tissue Paper" Scar.
=======================================================================

Zosiyanasiyana Zisanu Zachipatala za Pyoderma Gangrenosum¶

1. Classic Ulcerative Pyoderma Gangrenosum (85% of Cases): Nthawi zambiri amaphulika pambuyo pretibial m'munsi miyendo (70%), thunthu, kapena pamimba. Zimayamba ngati mphuno yofiyira kwambiri kapena wosabala wozunguliridwa ndi erythematous halo yomwe imalowa mwachangu pakati pa chilonda chakuya ndi purulent-kuwoneka wosabala maziko ndi boggy, bluish-purple (violaceous), wofooketsa malire otalikirana. Ikachiritsa pansi pa chitetezo chamthupi, re-epithelialization imakula kuchokera ku zitsitsi zatsitsi zomwe zasungidwa m'milatho, ndikusiya pathognomonic cribriform ("sieve-like" kapena "psed ndudu / tissue paper") atrophic scar.
2. Bullous (Atypical) Pyoderma Gangrenosum (Mbendera Yofiira ya Hematologic Malignancy): Imakhala ndi mafupa otupa, opweteka, ongoyang'ana pamwamba (matuza odzaza magazi) okhala ndi kutupa kotuwa kotuwa, makamaka kumapazi** kumaso, m'manja, m'mwamba, ndi kumtunda*. Critical Oncology Pearl: Oposa **70 peresenti ya odwala omwe ali ndi Bullous Pyoderma Gangrenosum ali ndi vuto loyambitsa magazi m'thupi *, makamaka Acute Myeloid Leukemia (AML), Myelodysplastic Syndrome (MDS), Myeloproliferative Multiple Neoplasms, ndi **Iyo kawirikawiri histologically ndi bullous Sweet syndrome.
3. Pustular Pyoderma Gangrenosum (Acute IBD Flare Marker): Imawonetsa ngati mbewu zowawa, zosaoneka 2 mpaka 5 millimeter wosabala ma pustules ozunguliridwa ndi erythematous halo pamiyendo ndi thunthu lapamwamba lomwe sali zonse kupita ku zibowo zakuya; zimachitika pafupifupi nthawi ya kuyaka, koopsa kwa Ulcerative Colitis kapena Crohn Disease ndipo imatheka pamene matumbo akuwotcha ndi corticosteroids kapena infliximab.
4. Vegetative (Zapamwamba Granulomatous) Pyoderma Gangrenosum: Chotupa chocheperako, chowawa kwambiri, chowoneka ngati njerewere, chowoneka bwino cha zilonda zam'mimba chomwe chilibe malire owopsa, omwe amapezeka pa thunthu mwa odwala opanda kuyankha, matenda obwera chifukwa cha matenda amtundu uliwonse, minocycline.
5. Peristomal Pyoderma Gangrenosum (PPG): Imapezeka nthawi yomweyo moyandikana ndi mimba ileostomy kapena colostomy stoma mwa odwala omwe ali ndi Inflammatory Bowel Disease (omwe amawerengera 15% ya milandu yonse ya PG), yoyambitsidwa ndi **kusasinthika kwamakina kuchokera ku Tight Stoma, kapena kupukuta kwa zida zomangira. enzyme **. Chifukwa chakuti chilonda chopweteka kwambiri chimapangitsa kuti chimbudzi chisatseke pakhungu, chimbudzi chimangotuluka mosalekeza, zomwe zimapangitsa kuti pakhale kupweteka kwambiri komanso kulephera kwa chipangizo chamagetsi.

Autoinflammatory Genetic Syndromes Yokhala ndi Pyoderma Gangrenosum (PAPA, PASH, ndi PAPASH)¶

Odwala ang'onoang'ono omwe ali ndi pyoderma gangrenosum yoopsa, yobwerezabwereza yomwe imatsagana ndi ziphuphu zowononga kapena hidradenitis suppurativa, madokotala amawonetsa monogenic autoinflammatory inflammasomopathies yoyendetsedwa ndi masinthidwe a PSTPIP1 (Proline-Serine-Threonine Phosphatase ** Mapuloteni Ogwirizana ndi Mapuloteni) IL-1beta: - PAPA Syndrome: Pyogenic sterile Arthritis + Pyoderma gangrenosum + kwambiri cystic Acne. - PASH Syndrome: Pyoderma gangrenosum + Acne + Suppurative Hidradenitis (hidradenitis suppurativa). - PAPASH / PASS Syndromes: Kuonjezera Psoriatic arthritis kapena Ankylosing Spondylitis ku triad. Ma syndromes amayankha modabwitsa ku kutsekereza kwa IL-1 (Anakinra kapena Canakinumab) kapena TNF-alpha inhibitors (Infliximab / Adalimumab).

Kuzindikira kwa mbali ndi mbali: Pyoderma Gangrenosum vs Necrotizing Fasciitis vs Martorell Hypertensive Ischemic Ulcer vs Brown Recluse Spider Bite vs Levamisole Vasculopathy¶

PARACELSUS Diagnostic Score ndi Mandatory Laboratory Workup for Suspected Pyoderma Gangrenosum¶

1. Momwe Mungawerengere Chigoli cha PARACELSUS Pamphepete mwa Bedi¶

Lofalitsidwa ndi Jockenhovel ndi anzake monga chida chodziwira matenda okhudzidwa kwambiri chosiyanitsa pyoderma gangrenosum kuchokera ku venous, arterial, and infectious mimics, chiwerengero chonse cha 10 mfundo kapena kupitilira apo chimatsimikizira kuthekera kwakukulu kwa Pyoderma Gangrenosum: - Zofunika Zazikulu (3 Mfundo Iliyonse): 1. **P ** matenda opitilira muyeso (chilonda chomwe chikuchulukirachulukira m'masabata 6). 2. Kuwunika ndi kuchotseratu mitundu yosiyana siyana (kulephera kwa venous/arterial, matenda, vasculitis, calciphylaxis). 3. Reddish-violaceous (wofiirira-buluu) malire a bala. - Zofunika Zing'onozing'ono (2 Mfundo Iliyonse): 4. Akongolera (kuwongolera mwachangu) ndi mankhwala ochepetsa thupi. 5. Cmawonekedwe odabwitsa, polycyclic, kapena serpiginous zilonda zam'mphepete. 6. E** kupweteka kwambiri (> 4 mwa 10 pa Visualnalogi Scale, nthawi zambiri 8 mpaka 10 mwa 10). 7. Localized pathergy phenomenon (chilonda choyambika pamalo ovulala, opaleshoni, kapena biopsy). - Zonjezera Zowonjezera (Mfundo 1 Iliyonse): 8. Skutupa kowonjezera pa histopathology (dense dermal neutrophilic infiltrate). 9. U m'mphepete mwa mabala. 10. **S ** matenda okhudzana ndi matenda (IBD, nyamakazi ya nyamakazi, IgA gammopathy, malignancy hematologic).

2. Kukonzekera Mwadongosolo Koyenera kwa Wodwala Aliyense Watsopano Wopezeka ndi Pyoderma Gangrenosum¶

Chifukwa ** 50 mpaka 70 peresenti ya odwala amakhala ndi matenda oyambitsa matenda ** (ndipo chifukwa PG ikhoza kukhala chizindikiro choyamba chachipatala cha zamatsenga ** Acute Myeloid Leukemia, Myelodysplastic Syndrome, IgA Multiple Myeloma, Crohn Disease, kapena GPA/ANCA Vasculitis *), perekani gulu lathunthu lodziwira matenda mukaloledwa: - Hematologic and Paraproteinemia Screen: Complete Blood Count (CBC) with manual peripheral blood smear (kuyang'ana myeloblasts, dysplastic neutrophils, or Auer rods), Serum Protein Electrophoresis (SPEP) yokhala ndi Immunofixation Electrophoresis (IFE), ndi Ife)Kuwala kwa Maselo, ndi Imaselo Aulere monoclonal gammopathy, paraprotein yapamwamba yolumikizidwa ndi PG). - Sikirini ya Matenda a M'mimba / Otupa: Mwatsatanetsatane mbiri ya matenda otsekula m'mimba, hematochezia, kuchepa thupi, kapena aphthae m'kamwa; Fecal Calprotectin ndi Ileo-colonoscopy yokhala ndi mucosal biopsies ngati pali zizindikiro za GI kapena kuchepa kwa magazi m'thupi mosadziwika bwino. - Vasculitis, Thrombophilia, and Drug-Induced PG Screen: c-ANCA / PR3 ndi p-ANCA / MPO (kuti aletse Granulomatosis with Polyangiitis [GPA] and Levamisole-adulterated cocaine vasculopathy, yomwe imayambitsa retiform purpura ya mphuno ya khutu/NE ANCA), Antiphospholipid antibodies (lupus anticoagulant, anticardiolipin, beta-2 glycoprotein I), Rheumatoid Factor / Anti-CCP, Hepatitis B, Hepatitis C, and HIV serologies, and Urine Toxicology for Cocaine/Levamisole. - Deep Edge Skin Biopsy Protocol (Zitsanzo Ziwiri): Pezani elliptical incisional biopsy kapena 4-to-6 mm punch biopsy kutengera malire amphamvu a erythematous-violaceous mpaka pansi pamafuta a subcutaneous: tumizani *Special 1 in formalin for H&E, PAS, ndi Fiteel-GMENTHE, Fiteel-Grocotte, Fiteel-GMS, Filteel-GMS madontho **, ndi kutumiza ** Chitsanzo 2 mu saline wosabala kwa aerobic, anaerobic, fungal (Blastomyces, Cryptococcus, Histoplasma, Sporothrix), ndi atypical mycobacterial (*M. marinum, M. ulcerans, M. fortuitum *) zikhalidwe za minofu **.

Ndondomeko Yothandizira Pachipatala Pang'onopang'ono: Systemic Immunosuppression, Biologics, ndi Peristom Care¶

1. Mzere Woyamba wa Systemic Immunosuppression (Prednisone vs. Cyclosporine)¶

• Systemic Corticosteroids: Yambani Oral Prednisone kapena Prednisolone pa 1.0 mpaka 1.5 mg/kg/tsiku (60 mpaka 100 mg kamodzi tsiku lililonse), kapena chifukwa cha kuphulika kwa postoperative kapena multi-ulcer matenda, perekani **Pulse IV Methylprednisolone (500 mg mpaka 3 mg tsiku * IV masiku * 500) Pamene kupitirira malire kumayima ndipo kuponderezedwa kocheperako kumachepa (mkati mwa masabata 1 mpaka 3), pitirizani pang'onopang'ono kwa miyezi 3 mpaka 6.
• Oral Cyclosporine (Calcineurin Inhibitor): Yambani **Oral Modified Cyclosporine (Neoral) pa 4 mpaka 5 mg / kg / tsiku logawidwa kawiri tsiku ndi tsiku **. Pachizindikiro ** STOP GAP mayesero olamulidwa mwachisawawa **, oral cyclosporine (4 mg/kg/tsiku) ndi oral prednisolone (0.75 mg/kg/tsiku) anasonyeza liwiro lofanana la machiritso pa masabata a 6 ndi kutsekedwa kofanana kwa miyezi 6 (47%), ndi prednisolone yonyamula matenda aakulu kwambiri ndi cyclibleosporinetension kumayambitsa cyclibleosporine. Yang'anirani kuthamanga kwa magazi, creatinine, potaziyamu, ndi magnesium pakadutsa milungu 1 mpaka 2 iliyonse.

2. Njira Zochiritsira Zomwe Zapangidwira Zamoyo ndi Steroid-Sparing¶

• TNF-Alpha Inhibitors (Infliximab ndi Adalimumab): IV Infliximab (5 mg/kg pa Masabata 0, 2, ndi 6, kenako masabata 6 mpaka 8 aliwonse) ndi golide wodziwika bwino wa biologic agent wa PG, makamaka ndi klzzwxh:0118 choyamba.Subcutaneous Adalimumab* (160 mg Sabata 0, 80 mg Sabata 2, kenako 40 mpaka 80 mg mlungu uliwonse) ndi njira yabwino yolumikizira.
• IVIG, Steroid-Sparing Agents, and Cytokine Blockers: Perekani IVIG (2 g/kg kupyola masiku 3 mpaka 5) pamene matenda oopsa kapena sepsis akutsutsana ndi steroids. Phatikizani ma steroids kapena biologics ndi Oral Dapsone (50 mpaka 200 mg/tsiku pambuyo pakuwunika kwa G6PD), Mycophenolate Mofetil, Azathioprine, kapena Colchicine. Pamilandu yotsutsa kapena **PAPA/PASH syndromes **, IL-1 antagonists (Anakinra, Canakinumab), IL-23 inhibitors (Ustekinumab), ndi JAK inhibitors (Upadacitinib) amapeza chikhululukiro chofulumira.

3. Kodi Kuchita Opaleshoni Pakhungu Kumaloledwa Liti ku Pyoderma Gangrenosum?¶

• Panthawi Yakutupa Kwambiri: KONZE. Maopaleshoni aliwonse, kuwononga chakuthwa, kapena kumetedwa pakhungu (komwe kumayambitsa zilonda zatsopano za PG pantchafu malo opereka ndalama) ndizoletsedwa.
• Panthawi ya Machiritso Abwino Kwambiri Pansi pa Immunosuppression Yonse: Pamene steroids, cyclosporine, kapena infliximab azimitsa malire a neutrophilic ndipo ululu wapita, *kuchotsa pang'onopang'ono, Negative Pressure Wound Therapy (NPWT), kapena bioengineered clover dermal substitutes.

Nambala zangozi zachigawo ndi ndondomeko za bungwe¶

• United States ndi Canada: Imbani 911 kuti muwonjezere msanga zilonda zapakhungu zotsatizana ndi kutentha thupi kwambiri, kuuma, kapena kupweteka kosalekeza. Madokotala amatsata American Academy of Dermatology (AAD) ndi Delphi International Consensus njira zopewera matenda ndi kupewa matenda.
• United Kingdom: Imbani 999 kapena pitani ku dipatimenti yazadzidzi ya NHS. Pansi pa British Association of Dermatologists (BAD) ndi UK Dermatology Clinical Trials Network (UK DCTN STOP GAP Trial) malangizo, magulu a chipatala amayambitsa oral prednisolone (\(0.75\text{mg/kg/day}\)) kapena cyclosporine (\(4\text{mg/kg/tsiku)\))
• European Union: Imbani 112 m'maiko onse omwe ali membala wa EU. Tsatirani European Academy of Dermatology and Venereology (EADV) ndi German Dermatologic Society (DDG) PARACELSUS Score malangizo.
• Australia, New Zealand, ndi Asia-Pacific: Imbani 000 ku Australia, 111 ku New Zealand (Australasian College of Dermatologists [ACD]), 119 ku Japan ndi South Korea (Japanese Dermatological Association [JDA] Pyoderma Gangrenosum Guidelines in 111 ku New Zealand (Australasian College of Dermatologists [ACD]), 119 ku Japan ndi South Korea (Japanese Dermatological Association [JDA] Pyoderma Gangrenosum Guidelines in 111 ku New Zealand (Australasian College of Dermatologists [ACD]), 119 ku Japan ndi South Korea (Japanese Dermatological Association [JDA] Pyoderma Gangrenosum Guidelines in9), zofunsira9, zofunsira9, zofunsira* Singapore, kapena 112 / 108 ku India.

Mafunso omwe amafunsidwa pafupipafupi¶

Chifukwa chiyani kuchotsedwa kwa opaleshoni kumapangitsa chilonda cha Pyoderma Gangrenosum kukhala chowopsa?¶

Mosiyana ndi necrotizing fasciitis (yomwe imayambitsidwa ndi mabakiteriya odya nyama omwe amayenera kudulidwa m'chipinda chopangira opaleshoni), Pyoderma Gangrenosum (PG) ndi matenda a autoimmune komanso autoimmune momwe maselo oyera amagazi (neutrophils) amaukira molakwika khungu nthawi iliyonse yavulala. Kuchita mokokomeza kotereku kuvulala kumatchedwa cutaneous pathergy. Dokotala akalakwitsa chilonda cha purulent PG chifukwa cha matenda a bakiteriya ndikudula malire a khungu lofiirira ndi scalpel, maselo owonongeka a khungu amatulutsa ma alarm (interleukin-1 beta ndi TNF-alpha) omwe amayitanitsa mamiliyoni a neutrophils atsopano kumphepete kumene kudulidwa. Mkati mwa maola 24 mpaka 48, ma neutrophils amenewo amagaya kolajeni wathanzi m’mphepete mwa opaleshoni, kuchititsa chilondacho kukula kuwirikiza kawiri kapena katatu.

Kodi madotolo angadziwe bwanji kuti Postoperative Pyoderma Gangrenosum kusiyanitsidwa ndi matenda opangira mabala a bakiteriya pambuyo pa opaleshoni ya bere kapena m'mimba?¶

Zizindikiro zinayi zakuchipatala zimalekanitsa Postoperative Pyoderma Gangrenosum (PPG) ndi matenda a chilonda cha bakiteriya: (1) Nthawi: PPG imayamba pafupifupi masiku 4 mpaka 14 pambuyo pa opaleshoni ngati tinthu tating'ono tating'ono tomwe timatulutsa timadzi timene timaphatikizira ndikung'amba bala, pomwe matenda oopsa a Gulu A Strep amakhudza mawola 72; (2) Maonekedwe a m'malire: PPG imapanga m'mphepete mwapakhungu, wofiirira, wamfuti, wopindika ("osatsikira"); (3) Kuteteza mawere pakuchita opaleshoni ya bere: pochepetsa mawere kapena kumanganso PPG, khungu la bere limasungunuka pamene nsonga ya nipple-areolar imasungidwa mwapadera; ndi (4) Kulephera kwa maantibayotiki: PPG imakulirakulirabe ngakhale kuti mankhwala opha tizilombo toyambitsa matenda ophatikizika m'mitsempha yamitundumitundu komanso kuchotsedwa kwa opaleshoni, komabe amasiya kukula mkati mwa maola 48 atayamba kumwa kwambiri corticosteroids.

Chifukwa chiyani Pyoderma Gangrenosum amatchedwa "Pyoderma" ngati palibe matenda a bakiteriya?¶

Pamene akatswiri a dermatologists Brunsting, Goeckerman, ndi O'Leary adatchula matendawa koyamba mu 1930, adakhulupirira molakwika kuti adayambitsa matenda a streptococcal kapena staphylococcal ("pyoderma" amatanthauza mafinya pakhungu) zomwe zinachititsa kuti minofu ife ("gangrenosum"). Mankhwala amakono pambuyo pake adatsimikizira kuti mafinya achikasu akuda omwe amaphimba maziko a chilonda chatsopano cha PG ali ndi mabakiteriya a zero; kwenikweni ndi msuzi wosabala wopangidwa ndi mamiliyoni ambiri a maselo oyera a magazi (neutrophils) omwe adasefukira dermis chifukwa cha chitetezo chamthupi chochuluka. Ngakhale kuti dzinali silinatchulidwe molakwika m’mbiri, lakhala m’mabuku a zachipatala kwa zaka pafupifupi 100.

Ndi matenda ati omwe amalumikizidwa kwambiri ndi Pyoderma Gangrenosum?¶

Pakati pa 50 ndi 70 peresenti ya odwala omwe adapezeka ndi Pyoderma Gangrenosum ali ndi matenda oyambitsa matenda. Mayanjano anayi omwe amapezeka kwambiri ndi awa: (1) Matenda Otupa (Ulcerative Colitis ndi Crohn Disease, omwe amawoneka mu 20% mpaka 30% ya milandu); (2) Matenda a Nyamakazi (Rheumatoid Arthritis ndi ankylosing spondylitis); (3) Matenda a khansa ya m'magazi ndi matenda a magazi (makamaka Acute Myeloid Leukemia [AML], Myelodysplastic Syndrome [MDS], ndi IgA Monoclonal Gammopathy, zomwe zimagwirizanitsidwa makamaka ndi mtundu wa bullous wa PG pamanja ndi nkhope); ndi (4) Genetic autoinflammatory syndromes monga PAPA ndi PASH syndrome.

Zomwe zimagwira bwino ntchito Pyoderma Gangrenosum: Prednisone kapena Cyclosporine?¶

Malinga ndi kafukufuku wodziwika bwino wa UK STOP GAP, oral Prednisolone (0.75 mg/kg/tsiku) ndi Cyclosporine yapakamwa (4 mg/kg/tsiku) amagwira ntchito mofanana: onse amasiya kukula kwa zilonda pa liwiro lofanana ndikupeza machiritso ofanana (pafupifupi 50% ochiritsidwa pakadutsa miyezi 6). Madokotala amasankha pakati pawo potengera momwe wodwalayo alili: Prednisone (kapena IV Methylprednisolone) imayamikiridwa kwa odwala omwe ali ndi matenda a impso kapena kuthamanga kwa magazi, pamene Cyclosporine imayamikiridwa ndi odwala matenda a shuga, kunenepa kwambiri, osteoporosis, kapena mbiri ya steroid-induced psychiatric zotsatira. Pamene PG imalumikizidwa ndi matenda a Crohn kapena Ulcerative Colitis, intravenous Infliximab (5 mg/kg) ndiye mankhwala opangidwa ndi golide.

Magwero ndi malangizo ovomerezeka¶

• JAMA Dermatology Delphi International Consensus (Maverakis E, Ma C, Shinkai K, et al., 2018): Diagnostic Criteria of Ulcerative Pyoderma Gangrenosum: A Delphi Consensus of International Experts.
• BMJ (STOP GAP Randomized Controlled Trial, Ormerod AD, Thomas KS, Craig FE, et al., UK Dermatology Clinical Trials Network): *Kuyerekeza ndi Njira ziwiri Zomwe zimagwiritsidwa ntchito kwambiri pa Pyoderma Gangrenosum: Zotsatira za STOP GAP Randomized Controlled Trial (Prednisolone)
• JAMA Dermatology (Jockenhovel F, et al.): The PARACELSUS Score: Novel Diagnostic Tool for Pyoderma Gangrenosum.
• British Association of Dermatologists (BAD) ndi American Academy of Dermatology (AAD): Clinical Guidelines on Neutrophilic Dermatoses, Postoperative Pyoderma Gangrenosum Recognition, and Pathergy Prevention.
• Gut (Brooklyn TN, Dunnill MG, Shetty A, et al.): Infliximab for the Treatment of Pyoderma Gangrenosum: A Randomised, Double-Blind, Placebo-Controlled Trial.

Maupangiri ogwirizana¶

• Calciphylaxis (calcific uremic arteriolopathy): zolembera za violaceous, black eschars, ndi sodium thiosulfate
• Erysipelas vs necrotizing fasciitis vs stasis dermatitis: ululu wopitilira muyeso, bullae, ndi LRINEC score triage
• Mkazi wamasiye wakuda latrodectism minofu kulimba vs bulauni recluse loxoscelism necrotic zilonda triage
• Necrotizing fasciitis (matenda odya nyama) ndi mbendera zofiira za cellulitis
• Nambala zadzidzidzi malinga ndi dziko