Pyoderma Gangrenosum (Pathergy Trap): Na-agbasa ngwa ngwa ime ihe ike na-adịghị mma ọnya na ihe kpatara mbibi ịwa ahụ ji abawanye Necrosis¶

Pyoderma gangrenosum (PG) bụ ihe a na-adịghị ahụkebe, nke na-adịghị efe efe, autoinflammatory ** neutrophilic dermatosis ** nke nrịbama na-adịghị mma na-ebute oke ọnụọgụ nke tozuru oke, polymorphonuclear neutrophils na-adịghị mma n'ime dermis, na-agbaze collagen na anụ ahụ subcutaneous n'ime awa ruo ụbọchị. N'agbanyeghị aha akụkọ ihe mere eme na-eduhie eduhie e chepụtara na 1930, pyoderma gangrenosum abụghị ọrịa nje ("pyoderma") ma ọ bụ ezigbo ischemic vascular gangrene **. Kama nke ahụ, ọ na-amalite dị ka papule na-egbuke egbuke, pustule na-adịghị mma, ma ọ bụ hemorrhagic vesiculopustule, na-ebutekarị na saịtị nke obere mmerụ ahụ, ahụhụ ahụhụ, venipuncture, ma ọ bụ ịwa ahụ, ihe ngosi mara mma nke a maara dị ka ** pathergy pathergy ** (ndị ọrịa dị na pasent 30). N'ime awa 24 ruo 72, pustule na-adịghị mma na-akụda etiti ma gbasaa centrifugally na ọsọ dị egwu (1 ruo 2 centimeters kwa ụbọchị) n'ime ihe na-egbu mgbu na-egbu mgbu, purulent, necrotic crater ** na-agbanye site na pathognomonic ** boggy, gunmetal-gray na-acha anụnụ anụnụ na-acha anụnụ anụnụ, na-acha anụnụ anụnụ. gbara ya gburugburu na-egbuke egbuke erythematous halo. N'etiti 50 na 70 pasent nke ndị ọrịa nwere pyoderma gangrenosum nwere ọrịa na-akpata ọrịa usoro, nke na-abụkarị ** Ọrịa bowel na-egbu egbu (Ulcerative Colitis ma ọ bụ Ọrịa Crohn) *, ** Rheumatoid Arthritis ma ọ bụ Seronegative Spondyloarthritis , ** Monoclonal Gammopathy (ọkpụkpọ anụ ahụ) Malignancy (Acute Myeloid Leukemia ma ọ bụ Myelodysplastic Syndrome). N'ihi na ọnya na-agbasa ngwa ngwa, ọnya purulent na-esonyere ahụ ọkụ, oke mgbu, na ọnụ ọgụgụ ọbara ọcha nke 20,000 ruo 40,000 sel / mcL na-eṅomi ** necrotizing fasciitis (ọrịa nje na-eri anụ ahụ) ma ọ bụ ọnya ọnya na-esote, ndị dọkịta na-awa ndị na-adịghị aghọta pyoderma gangrenosum na-agba ọsọ ngwa ngwa maka onye ọrịa na-arụ ọrụ ugboro ugboro. Na pyoderma gangrenosum, ** ọrịa strok ọ bụla nke onye dọkịta na-awa ahụ na-ebute oké ifufe neutrophil na-agbawa agbawa n'akụkụ akụkụ akpụkpọ ahụ ka ebipụrụ ọhụrụ **, na-eme ka ọnya ahụ gbasaa okpukpu abụọ ma ọ bụ okpukpu atọ n'abali, na-ekpughe akwara na ọkpụkpụ, ma ọ bụ na-eduga na mbepụ ụkwụ na-adịghị mkpa. Ọ bụrụ na onye ọrịa amalite ọnya ọnya na-agbasa ngwa ngwa nke nwere oke na-acha odo odo mgbe obere mmerụ ahụ ma ọ bụ ịwa ahụ gasịrị, kpọọ mpaghara gị. nọmba mberede ma ọ bụ chọọ nyocha dermatology ụlọ ọgwụ ngwa ngwa ozugbo. Nyochaakwa ntuziaka ụmụnna anyị na calciphylaxis, violaceous retiform plaques, na sodium thiosulfate, erysipelas vs necrotizing fasciitis vs stasis dermatitis triage, na Nwanyị di ya nwụrụ latrodectism vs brown recluse loxoscelism necrotic ọnyá afọ triage.

Ihe kacha mkpa maka ihe mberede gị ozugbo bụ ** idobe ịdọ aka ná ntị ozugbo "AKWỤKWỤKWỤKWỤKWỤKWỤKWỤKWỌ" ịdọ aka ná ntị na chaatị onye ọrịa na mgbakwasa ọnya ***, ** pyoderma gangrenosum dị iche iche sitere na ọrịa anụ ahụ dị nro nke necrotizing n'ezie na-eji nyocha ụlọ ọgwụ n'akụkụ akwa, ntụpọ Gram, ọkpụkpụ anụ ahụ, na ihe ziri ezi ** PARACELSUS ma ọ bụ Delphic diagnostic . Punch akpụkpọ biopsy nke oke violaceous na-arụ ọrụ (naanị ma ọ bụrụ na ọ dị mkpa ka ewepu ọrịa, vasculitis, ma ọ bụ malignancy, na nke ọma mgbe ị na-amalite usoro immunosuppression na-adịghị mma biopsy pathergy) , na ** malite ngwa ngwa na-eme ihe na-eme ihe na-eme ihe na-eme ihe na-eme ihe na-eme ihe na-eme ihe na-eme ihe na-eme ihe na-eme ihe na-eme ihe na-egbochi neutrophil immunosuppression , kpọmkwem ** elu-dose systemic (1 mg / ụbọchị Corticod). pulse IV Methylprednisolone 500 ruo 1,000 mg / ụbọchị maka ụbọchị 3), ** ọnụ Cyclosporine (4 ruo 5 mg / kg / day), ma ọ bụ ** Infliximab intravenous (5 mg / kg, ọkọlọtọ ọla edo maka Crohn/Ulcerative Colitis jikọtara ya na peristomal).PG

Mee nke a ugbu a¶

1. **Chọọ ihe mberede ụlọ ọgwụ ngwa ngwa na nyocha gbasara akpụkpọ ahụ ma ọ bụrụ na pustule akpụkpọ ahụ, mbepụ ịwa ahụ, ma ọ bụ obere ọnya na-abanye ngwa ngwa n'ime olulu na-egbu mgbu nke nwere oke odo odo-acha anụnụ anụnụ, bogi, oke oke. nọmba mberede.
2. ** Kwụsị nbibi ịwa ahụ dị nkọ ma ọ bụ nnukwu ọpụpụ ozugbo (Ọnyà Pathergy):** Ọ bụrụ na onye ọrịa amarala ma ọ bụ na-enyo enyo nke pyoderma gangrenosum (gụnyere ** Pyoderma Gangrenosum [PPG] na-aga n'ihu [PPG]** na-apụta 4 ruo 14 ụbọchị mgbe mbelata ara / nwughari, ngalaba cesarean, akụkụ akwara, ma ọ bụ akụkụ nke akụkụ ahụ nke akụkụ ahụ, akụkụ nke akụkụ ahụ nke akụkụ ahụ. A na-emegidekwa nbibi site na peeled ma ọ bụ curette. Ịwa ahụ na-ewebata nnukwu ebili mmiri nke IL-1beta-, IL-8-, na TNF-alpha na-ebute neutrophils na dermis ọhụrụ, na-eme ka ọnya centrifugal dị egwu gafee n'ime obi, afọ, ma ọ bụ akụkụ n'ime awa 24.
3. ** Kwụsị Ezi Necrotizing Fasciitis, Ecthyma Gangrenosum, na Ọrịa Fungal dị omimi na-enweghị ịkpalite mpụ na-enweghị isi na-enweghị isi:**
4. Ihe mere e ji aghọgbu ndị dọkịta na-awa ahụ: Pyoderma gangrenosum na-ebute purulent exudate na-acha odo odo na-acha ọcha (nke ọtụtụ nde neutrophils nwụrụ anwụ mejupụtara), nnukwu ahụ ọkụ, na leukocytosis ruru 35,000/mcL.
5. ** Otu esi agwa ha iche n'akụkụ akwa: ** N'adịghị ka fasciitis necrotizing (nke na-agbasa miri n'akụkụ muscle subcutaneous fascia * n'ihu * nke mgbawa akpụkpọ ahụ a na-ahụ anya, na-emepụta gas / crepitus subcutaneous, mmiri mmiri "mmiri mmiri" na-adịghị mma, na usoro septic ujo na hypotension na lactic acidosis), ** pyoderma nwere nnukwu ọnya na-egbuke egbuke, nke na-eme ka ọ bụrụ ọnyá na-egbuke egbuke. gunmetal-violaceous mebie overhang **, gas subcutaneous efu ***, isi ísì anaerobic efu na-adịghị mma **, mkpọ anụ ahụ na-apụ apụ, na ** ntụpọ Gram na-adịghị mma na ọdịbendị ọbara.
6. ** Ihe ngosi PG nke na-arụ ọrụ: ** Postoperative bacterial necrotizing ọnya ọrịa na-amalitekarị n'ime awa 24 ruo 72 ** nke ịwa ahụ (Group A * Streptococcus * ma ọ bụ * Clostridium ) ma ọ bụ dị ka ihe na-agbanwe agbanwe, ebe ** Pyoderma Gangrenosum (PPG) na-aga n'ihu dị ka obere oge (PPG) 4 ga-apụta obere oge. pustules na-adịghị mma n'akụkụ ahịrị suture nke na-ejikọta ma na-ewepụ ebe ahụ dum ebe ** na-echekwa areola ahụ na-acha odo odo na ịwa ahụ ara* na-adịghị emeziwanye na ọgwụ nje nje IV sara mbara.
7. Tinye Frameworks Diagnostic Akwadoro (The Delphi International Consensus Criteria and the PARACELSUS Score) wee nweta biopsy ezubere iche:
8. N'okpuru ** 2018 Delphi International Consensus ***, nchoputa chọrọ ** 1 Isi Criterion ** (biopsy nke ọnya ọnya na-egosi ** dense sterile neutrophilic dermal infiltrate **) gbakwunyere ** ma ọ dịkarịa ala 4 nke 8 Minor Criteria ** (mwepu nke ọrịa ogbu na nkwonkwo na histology / omenala; pathergy; akụkọ ihe mere eme nke ọnya afọ 4; erythema dị n'akụkụ, na-emebi oke, na nro dị iche iche nke nwere opekata mpe otu n'ụkwụ azụ azụ;
9. N'okpuru PARACELSUS Score (ebe akara nke >= 10 isi na-egosi> 90% puru omume nke PG), a na-enye isi ihe maka P ọrịa na-efe efe (3 pts), ** A *** nyocha nke nyocha dị iche iche dị mkpa (3 pts), na ** R *** eddish-violaceous ókè (3 pts) ọnya na-eme ihe ike (3 pts). pts), ** C *** ụdị ọnya ọnya dị egwu (2 pts), ** E *** oke mgbu> 4/10 na VAS (2 pts), na ** L *** ocalized pathergy phenomenon (2 pts).
10. ** Bido Corticosteroids sistemu dị elu ma ọ bụ Cyclosporine ozugbo iji kwụsị oke ifufe Neutrophil:**
11. Nzọpụta usoro nke mbụ (Nkwụsị nnwale GAP): Onye nchịkwa ** Oral Prednisolone/Prednisone na 0.75 ruo 1.5 mg/kg/ụbọchị (ruo 60 ruo 100 mg/ụbọchị), ma ọ bụ maka fulminant, na-agbasa ngwa ngwa multilocular ma ọ bụ postoperative PG. (500 ruo 1,000 mg IV otu ugboro kwa ụbọchị maka 3 ruo 5 ụbọchị sochirinụ), nke na-ejidekarị mgbasawanye nke centrifugal ma belata mgbu na-egbu mgbu n'ime awa 48 ruo 72 **.
12. ** Nhọrọ nke mbu nke mbu (Oral Cyclosporine): ** Ihe akara UK ** Kwụsị GAP nnwale a na-achịkwaghị nke ọma ** gosipụtara na ** Oral Cyclosporine na 4 ruo 5 mg / kg / ụbọchị ** (nke kewara ugboro abụọ kwa ụbọchị) nwere otu ọnya na-agwọ ọnya yana ọsọ na ọnụ prednisolone, na-eme ka ndị ọrịa nwere nnukwu ọrịa shuga na-ahọrọ. osteoporosis, oke ibu, ma ọ bụ steroid psychosis (zere Cyclosporine na ụkọ akụrụ ma ọ bụ ọbara mgbali elu na-achịkwaghị achịkwa).
13. ** Malite Biologic TNF-Alpha Blockade (IV Infliximab 5 mg/kg) maka Ọrịa Bowel na-efe efe-Associated, Perristomal, ma ọ bụ Steroid-Refractory Pyoderma Gangrenosum:** ** Intravenous Infliximab (5 mg / kg na Weeks 0, 2, na 6) na-akwado usoro ọgwụgwọ ọ bụla site na izu 0, 2, na 6, mgbe ahụ, a na-akwado usoro ọgwụgwọ ọ bụla. Nnwale a na-achịkwa placebo maka pyoderma gangrenosum, na-enweta nzaghachi ọgwụgwọ ngwa ngwa na ** 69 pasent nke ndị ọrịa n'ime izu 2 ** ma na-eje ozi dị ka ọgwụgwọ na-enweghị mgbagha nke nhọrọ mgbe PG na-ejikọta ya na ọrịa Crohn na-arụ ọrụ, Ulcerative Colitis, ma ọ bụ ọnyá peristomal.
14. Mee ahụ nke ọma, nlekọta ọnya dị n'ime ime obodo na mkpakọ dị nro: Ejila akwa gauze kpọrọ nkụ nke na-arapara n'àkwà ọnya wee dọkasịa viable dermis ma e wepụ ya (na-akpalite pathergy). Chebe akpụkpọ ahụ gbara ya gburugburu na ihe nkiri ihe mgbochi cyanoacrylate, tinye ** super-potent clobetasol propionate 0.05% mmanu ma ọ bụ tacrolimus 0.1% mmanu ** ozugbo na oke violaceous na-ekpo ọkụ, yi akwa akwa etiti na ** non-adherent, silicone, hydrochloride na silicone. tinye ** bandeeji mkpakọ multi-layer dị nro ** ozugbo oke mgbu nke oke oke na-achịkwa site na sistemu sistemu.

Osisi mkpebi: a na-enyo enyo Pyoderma Gangrenosum vs Necrotizing Fasciitis vs Venous/Arterial Ulcer vs Martorell Ulcer¶

flowchart TD
    A["Onye ọrịa na-enye ya na ọnya na-agbasa ngwa ngwa, na-egbu mgbu na-egbu mgbu (1 ruo 2 cm / ụbọchị) + Ebe odo odo na-acha odo odo + Boggy Violaceous/ Gunmetal-Gray na-emebi oke oke (Ọtụtụ mgbe mgbe obere mmerụ ahụ, Venipuncture, ma ọ bụ ịwa ahụ na-adịbeghị anya)"] --> B{"Kwụpụ 1: Mwepụta Ngwa ngwa nke Ezi Necrotizing Soft-Tissue Infection (Necrotizing Fasciitis): Enwere Gas/Crepitus Subcutaneous, Ọtọ Anaerobic Na-adịghị mma, Nleba Anya Na-agafe Oke akụkụ anụ ahụ, ma ọ bụ Septic Shock?"}
    B -->|YES: True Bacterial Necrotizing Fasciitis / Wet Gangrene| C["Nchọgharị ịwa ahụ na ime ụlọ ọrụ mberede + IV Vancomycin + Piperacillin-Tazobactam/Meropenem + Clindamycin"]
    B -->|NO: Primary Dermal Ulceration with Violaceous Undermined Overhang + Negative Gram Stain + Palpable Pulses| D["Kwụpụ 2: Tinye akara PARACELSUS (>= 10 Points) & ihuenyo maka Njikọ Sistemu: IBD (Ulcerative Colitis / Crohn), Rheumatoid Arthritis, IgA Gammopathy / AML / MDS, ma ọ bụ Levamisole-Adulterated Cocaine"]
    D --> E["Iwu nchekwa amanyere iwu: Ebe 'Mkpachapụ anya PATHERGY siri ike - Ọ BỤGHỊ ịwa ahụ dị nkọ, Ọ BỤGHỊ MKWU NDỊ NA-AKWU NDỊ MMADỤ' Bịnye aka na Bedside"]
    E --> F{"Kwụpụ 3: Mee Ezubere Biopsy Ezubere (Gụnye oke ike + abụba subcutaneous) maka H&E Histopathology + Bacterial, Fungal & Mycobacterial Tissue Cultures"}
    F --> G{"Nyochaa Ọdịmma Ọrịa, Ọdịmma & Ọrịa Na-akpata Sistemu"}
    G -->|Severe, Rapidly Expanding, Multilocular, or Postoperative PG| H["Nzọpụta usoro nke mbụ: 1. Oral Prednisone (1 ruo 1.5 mg / kg / ụbọchị) OR Pulse IV Methylprednisolone (500 ruo 1,000 mg / ụbọchị x 3-5 ụbọchị); MA ọ bụ 2. Oral Cyclosporine (4 ruo 5 mg/kg/ụbọchị - Kwụsị GAP Trial Equivalent)"]
    G -->|Coexisting Inflammatory Bowel Disease (Crohn / UC), Peristomal PG, or Steroid-Refractory Disease| I["Nzọpụta dị ndụ: Infliximab na-amị amị (5 mg/kg na izu 0, 2, 6) ma ọ bụ Adalimumab + Systemic Corticosteroid Bridge"]
    G -->|Underlying Hematologic Malignancy (AML / MDS / IgA Myeloma) or Severe Sepsis Contraindicating Steroids| J["Immunoglobulin dị elu nke dị elu (IVIG 2 g/kg karịa ụbọchị 2-5) ma ọ bụ IL-1 Antagonist (Anakinra / Canakinumab) + Na-agwọ ọrịa leukemia/Myeloma na-akpata"]
    G -->|Small (< 3 cm), Early, Localized, or Mild Peristomal Ulcer| K["Ihe kachasi ike n'elu Clobetasol 0.05% Ude ma ọ bụ Topical Tacrolimus 0.1% etinyere na oke ime ihe ike + Triamcinolone Intralesional Akpachara anya (40 mg/mL) + Uwe silikone na-anaghị agbaso"]

Pathophysiology: oké ifufe neutrophil na-adịghị mma, ọrịa akpụkpọ anụ, na ụdị ọgwụgwọ ise nke Pyoderma Gangrenosum.¶

Pyoderma gangrenosum na-anọdụ na njedebe ọnya ọnya siri ike nke ** neutrophilic dermatoses spectrum ** (n'akụkụ Sweet syndrome, ** subcorneal pustular dermatosis**, hidradenitis suppurativa, na 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.
=======================================================================

Ụdị ụlọ ọgwụ ise dị iche iche nke Pyoderma Gangrenosum¶

1. Classic Ulcerative Pyoderma Gangrenosum (85% nke ikpe): Ọtụtụ mgbe na-agbawa na ** ihu pretibial ala ụkwụ (70%) , ogwe, ma ọ bụ afọ. Na-amalite dị ka nodule na-acha ọbara ọbara dị nro ma ọ bụ pustule na-adịghị mma nke ihe na-acha uhie uhie gbara ya gburugburu nke na-abanye ngwa ngwa n'ime ọnya miri emi nwere ** purulent-apụtaghị ìhè isi yana ** boggy, bluish-purple (violaceous), mebie oke oke. Mgbe ọ na-agwọ n'okpuru immunosuppression, re-epithelialization na-etolite site na ntutu follicles echekwabara na àkwà mmiri, na-ahapụ pathognomonic ** cribriform ("sieve-like" ma ọ bụ "siga / anụ ahụ e gwepịara egwepịa") atrophic scar*.
2. Pyoderma Gangrenosum (Hematologic Malignancy Red Flag):** Ọ na-enye ya na ** nnukwu, na-egbu mgbu, nke na-ahụ maka hemorrhagic bullae (blisters jupụtara n'ọbara) nwere halo na-acha anụnụ anụnụ na-acha anụnụ anụnụ, nke kachasị na ** ihu, aka azụ, na elu. ** Ihe karịrị pasent 70 nke ndị ọrịa nwere Bullous Pyoderma Gangrenosum nwere ọrịa hematologic na-akpata ọrịa , kpọmkwem ** Acute Myeloid Leukemia (AML), ** Myelodysplastic Syndrome (MDS), ** Myeloproliferative Neoplasms, ma ọ bụ ọtụtụ ugboro na Myelogla. akụkọ ihe mere eme na ** bullous Sweet syndrome **.
3. ** Pyoderma Gangrenosum (Acute IBD Flare Marker):** Na-enye dị ka ihe ọkụkụ nke na-egbu mgbu, nke pụrụ iche ** 2 ruo 5 millimeter pustules na-adịghị mma nke gbara ya gburugburu erythematous halo ** na ogwe aka na ogwe elu nke na-adịghị * ọ bụla na-aga n'ihu na craters miri emi; Ọ fọrọ nke nta ka ọ bụrụ naanị n'oge ** nnukwu ọkụ ọkụ nke Ulcerative Colitis ma ọ bụ Ọrịa Crohn** ma na-edozi mgbe ejiri corticosteroids ma ọ bụ infliximab gwọọ ọnya afọ.
4. Pyoderma Gangrenosum (Pyoderma Gangrenosum): Akwụsịghị nwayọ, na-egbu mgbu, verrucous (wart-like), plaque ulcerative plaque nwere traktị sinus nke na-enweghị oke ike mebie nke ukwuu, nke na-emekarị na ogwe ahụ na ndị ọrịa * na-enweghị * na-akpata ọrịa sistemu ma ọ bụ na-anabata ya nke ọma.
5. ** Peristomal Pyoderma Gangrenosum (PPG):** Na-eme ozugbo n'akụkụ ** abdominal ileostomy ma ọ bụ colostomy stoma ** n'ime ndị ọrịa nwere Ọrịa Bowel Inflammatory (na-agụta 15% nke ikpe PG niile), kpalitere site na ** ọrịa na-adịghị ala ala sitere na Tight Stoma Appliance Wafers, ma ọ bụ ihe mgbaze enzyme. N'ihi na ọnya miri emi na-egbu mgbu na-egbochi wafer ostomy ka ọ ghara ịbanye n'ahụ akpụkpọ ahụ, stool na-agbapụ na-aga n'ihu na olulu mmiri ahụ, na-eme ka usoro mgbu na ọdịda ngwa.

Ọrịa mkpụrụ ndụ ihe nketa nke na-egosipụta Pyoderma Gangrenosum (PAPA, PASH, na PAPASH)¶

N'ime ndị ọrịa na-eto eto na-egosipụta nnukwu pyoderma gangrenosum na-aga n'ihu yana ihe otutu na-ebibi ihe ma ọ bụ hidradenitis suppurativa, ndị dọkịta na-enyocha maka monogenic autoinflammatory inflammasomopathies nke mmụgharị na PSTPIP1 (* Proline-Serine-Threonine Phosphatase Interacting Protein 1*) - ** Ọrịa PAPA: *** P *** yogenic sterile Arthritis + ** P *** yoderma gangrenosum + cystic siri ike ** A *** cne. - ** Ọrịa PASH: *** P *** yoderma gangrenosum + ** A *** cne + ** S *** uppurative ** H ** idradenitis (hidradenitis suppurativa). - ** Ọrịa PAPASH/PASS:** Na-agbakwunye Psoriatic ogbu na nkwonkwo ma ọ bụ A nkylosing Spondylitis na triad. Ọrịa ndị a na-anabata nke ọma na ** ezubere iche IL-1 mgbochi (Anakinra ma ọ bụ Canakinumab)* ma ọ bụ ** TNF-alpha inhibitors (Infliximab / Adalimumab)**.

Nchọpụta ọdịiche dị n'akụkụ n'akụkụ: Pyoderma Gangrenosum vs Necrotizing Fasciitis vs Martorell Hypertensive Ischemic Ulcer vs Brown Recluse Spider Bite vs Levamisole Vasculopathy¶

Akara nyocha nke PARAACELSUS yana ọrụ ụlọ nyocha amanyere amanyere maka Pyoderma Gangrenosum a na-enyo enyo.¶

1. Otu esi agbakọ akara PARACELSUS n'akụkụ akwa¶

Jockenhovel na ndị ọrụ ibe biputere ya dị ka ngwá ọrụ nyocha nke mmetụta dị elu iji mata ọdịiche nke pyoderma gangrenosum site na venous, arterial, na nje na-efe efe, ** ngụkọta akara nke 10 isi ma ọ bụ karịa na-akwado nnukwu ihe gbasara Pyoderma Gangrenosum : - ** Isi criteria (Isi 3 nke ọ bụla): 1. P *** ọrịa na-efe efe (ọnya na-agbasa ngwa ngwa na-etolite na <6 izu). 2. ** A *** nyocha na mwepu nke nyocha dị iche iche dị mkpa (ọrịa venous / akwara, ọrịa, vasculitis, calciphylaxis). 3. ** R *** eddish-violaceous (acha odo odo-acha anụnụ anụnụ) oke ọnya. - ** Obere criteria (Isi 2 nke ọ bụla): 4. A *** melioration (mmelite ngwa ngwa) site na ọgwụ mgbochi mgbochi. 5. **C *** ihe jọgburu onwe ya, polycyclic, ma ọ bụ ọnya serpiginous nwere akụkụ mebiri emebi. 6. ** E *** oke mgbu (> 4 n'ime 10 na Anya Analog Scale, mgbe mgbe 8 ruo 10 n'ime 10). 7. ** L *** ocalized pathergy phenomen (ọnya na-ebute na saịtị nke auma, ịwa ahụ, ma ọ bụ biopsy). - ụnyere mgbakwunye (1 Point ọ bụla):* 8. ** S *** mbufụt na-egbuke egbuke na histopathology (oke dermal neutrophilic infiltrate). 9. U akụkụ ọnya mebiri emebi. 10. ** S *** ọrịa ystemic metụtara (IBD, ogbu na nkwonkwo, IgA gammopathy, hematologic malignancy).

2. Arụmọrụ sistemu amanyere iwu n'ime onye ọrịa ọ bụla achọpụtara ọhụrụ nwere Pyoderma Gangrenosum.¶

N'ihi na ** 50 ruo 70 pasent nke ndị ọrịa na-ebute ọrịa na-akpata ọrịa na-akpata *** (na n'ihi na PG nwere ike ịbụ akara ngosi mbụ nke mgbaasị ** Acute Myeloid Leukemia, Myelodysplastic Syndrome, IgA Multiple Myeloma, Crohn Disease, ma ọ bụ GPA / ANCA Vasculitis ), nye iwu nyocha a zuru ezu: panel panel na nnabata. - ** Ihuenyo hematologic na Paraproteinemia: ** Ọnụ ọgụgụ ọbara zuru oke (CBC) na akwụkwọ ntuziaka mpụta ọbara smear (na-achọ myeloblasts, dysplastic neutrophils, ma ọ bụ Auer rods), ** Serum Protein Electrophoresis (SPEP) na Immunofixation Electrophoresis (IFE) (IFE) ihe ** IgA monoclonal gammopathy , paraprotein kpochapụwo jikọtara ya na PG). - ** Ihuenyo ọrịa afọ eriri afọ / na-ere ọkụ: Akụkọ gbasara afọ ọsịsa, hematochezia, ọnwụ arọ, ma ọ bụ aphthae ọnụ; ** Fecal Calprotectin *** na ** Ileo-colonoscopy nwere biopsies mucosal ** ma ọ bụrụ na akara ngosi GI ọ bụla ma ọ bụ anaemia enweghị ụkọ ígwè enweghị nkọwa. - ** Vasculitis, Thrombophilia, na Drug-Induced PG Screen: ** c-ANCA / PR3 na p-ANCA / MPO ** (iji wepụ ** Granulomatosis na Polyangiitis [GPA] na ** Levamisole-adlterated cocaine vasculopathy , nke na-akpata retiform purpura nke ntị-na-enwe ọnya afọ na MPO. ANCA), ** Antiphospholipid antibodies (lupus anticoagulant, anticardiolipin, beta-2 glycoprotein I), Rheumatoid Factor / Anti-CCP, ** ịba ọcha n'anya B, ịba ọcha n'anya C, na HIV serologies**, na ** Urine Toxicology maka Cocaine/Levamisole. - ** Usoro nyocha anụ ahụ miri emi (ntụpọ abụọ):** Nweta biopsy incisional dị warara ma ọ bụ 4-na-6 mm punch biopsy na-agbasa akụkụ ** erythematous-violaceous na-arụ ọrụ n'ime abụba subcutaneous **: zipu ** ụdị 1 na formalin maka H&E, PAS na ọlaọcha (Gronamine). Fite / Ziehl-Neelsen stains *, na zipu ** Ụdị 2 na saline na-adịghị mma maka ikuku, anaerobic, fungal ( Blastomyces, Cryptococcus, Histoplasma, Sporothrix ), na mycobacterial atypical ( M. marinum, M. ulcerans, M. fortuitum **) anụ ahụ.

Usoro ọgwụgwọ ụlọ ọgwụ nzọụkwụ-site-nzọụkwụ: Usoro mgbochi mgbochi ọrịa, bayoloji, na nlekọta Perristomal¶

1. Mgbochi sistemu sistemu nke mbụ (Prednisone vs. Cyclosporine)¶

• ** Corticosteroids sistemu: ** Malite ** Oral Prednisone ma ọ bụ Prednisolone na 1.0 ruo 1.5 mg / kg / ụbọchị (60 ruo 100 mg otu ugboro kwa ụbọchị)**, ma ọ bụ maka mgbawa postoperative ma ọ bụ ọrịa ọnya ọnya, na-enye ** Pulse IV Methylprednisolone (500 ruo 1,000 mg kwa ụbọchị). Ozugbo ịga n'ihu n'ókè akwụsị akwụsị na nfefe a na-emebi emebi na-agbadata (n'ime izu 1 ruo 3), jiri nwayọ nwayọ karịa ọnwa 3 ruo 6.
• ** Oral Cyclosporine (Calcineurin Inhibitor):** Malite ** Oral Modified Cyclosporine (* Neoral*) na 4 ruo 5 mg / kg / ụbọchị kewara ugboro abụọ kwa ụbọchị **. Na akara ngosi ** Kwụsị GAP nnwale a na-achịkwa nke ọma **, cyclosporine oral (4 mg / kg / day) na oral prednisolone (0.75 mg / kg / day) gosipụtara ọsọ ọsọ ọgwụgwọ na izu 6 na ọnụ ọgụgụ mmechi ahụ na ọnwa 6 (47%), na prednisolone na-ebu ọrịa siri ike karị na cyclosporine na-akpata nrụgharị. Nyochaa ọbara mgbali elu, creatinine, potassium, na magnesium kwa izu 1 ruo 2.

2. Ezubere iche maka usoro ndu ndu na Steroid-Sparing Therapies¶

• ** TNF-Alpha Inhibitors (Infliximab na Adalimumab): *** ** IV Infliximab (5 mg / kg na izu 0, 2, na 6, mgbe ahụ kwa izu 6 ruo 8)** bụ onye na-ahụ maka usoro ihe ọmụmụ ọla edo maka PG, ọkachasị na *Crohn Disease, Ulcerative Colitis, ma ọ bụ TB PG. ** Adalimumab subcutaneous *** (160 mg Izu 0, 80 mg Izu 2, wee 40 ruo 80 mg kwa izu) bụ nhọrọ dị mma nke subcutaneous.
• IVIG, Steroid-Sparing Agents, and Cytokine Blockers: Nye ** IVIG (2 g/kg karịrị ụbọchị 3 ruo 5)** mgbe malignancy na-arụ ọrụ ma ọ bụ sepsis na-emegide ndị steroid. Jikọta steroid ma ọ bụ ihe ndị dị ndụ na ** Oral Dapsone (50 ruo 200 mg / ụbọchị mgbe nyocha G6PD gasịrị) **, ** Mycophenolate Mofetil , ** Azthioprine, ma ọ bụ Colchicine. N'ihi na refractory ikpe ma ọ bụ PAPA / PASH syndromes **, ** IL-1 antagonist(Anakinra, Canakinumab), ** IL-23 inhibitors (Ustekinumab)**na ** JAK inhibitors (Upadacitinib) nweta mgbaghara ngwa ngwa.

3. Kedu mgbe anabatara ịwa ahụ akpụkpọ anụ na Pyoderma Gangrenosum?¶

• N'oge ọgbaghara na-arụ ọrụ: ** Ọ dịghị mgbe.** Mmachibido ịwa ahụ ọ bụla, nhụsianya dị nkọ, ma ọ bụ owuwe ihe ubi akpụkpọ anụ (nke na-ebute ọnya PG ọhụrụ n'apata ụkwụ ** saịtị inye onyinye ***) amachibidoro kpamkpam.
• ** N'oge Quiescent Healing Phase Under Full Immunosuppression: ** Ozugbo steroid, cyclosporine, ma ọ bụ infliximab kpochapụrụ njedebe neutrophilic kpamkpam na mgbu na-apụkwa, ** iwepụ nwayọọ nwayọọ, Ngwọta Ahụhụ Na-adịghị mma (NPWT), ma ọ bụ bioengineered dermal nnọchi ** nwere ike ime ngwa ngwa.

Nọmba mberede mpaghara na ụkpụrụ ụlọ ọrụ¶

• United States na Canada: Kpọọ 911 maka ọnya anụ ahụ na-agbasa ngwa ngwa na oke ahụ ọkụ, mgbakasị ahụ, ma ọ bụ mgbu na-adịghị agwụ agwụ. Ndị dọkịta na-agbaso ** American Academy of Dermatology (AAD)** na Delphi International Consensus usoro nchọpụta nchọpụta na mgbochi mgbochi.
• United Kingdom: Kpọọ 999 ma ọ bụ gawa na ngalaba mberede NHS. N'okpuru British Association of Dermatologists (BAD) na ** UK Dermatology Clinical Trials Network (UK DCTN STOP GAP Trial)** ntuziaka, ndị otu ụlọ ọgwụ na-ebute prednisolone ọnụ ($ 0.75\text{mg/kg/day}\() ma ọ bụ cyclosporine (\)4\text{mg/kg/day}) mgbe ị na-eme ihe ike.
• European Union: Kpọọ 112 gafee mba ndị otu EU niile. Soro European Academy of Dermatology and Venereology (EADV) na **German Dermatologic Society (DDG) PARAACELSUS Score **.
• Australia, New Zealand, na Asia-Pacific: Kpọọ ** 000** na Australia, ** 111** na New Zealand (Australasian College of Dermatologists [ACD]), 119 na Japan na South Korea (** Japanese Dermatological Association [JDA] Pyoderma Gangrenosummagulatory Guidelines), ebe nkwado nke Pyoderma Gangrenosum Adalimumabries. 995 na Singapore, ma ọ bụ 112/108 na India.

Ajụjụ a na-ajụkarị¶

Kedu ihe kpatara mmebi ịwa ahụ ji eme ka ọnya Pyoderma Gangrenosum ka njọ?¶

N'adịghị ka necrotizing fasciitis (nke kpatara nje bacteria na-eri anụ nke a ga-ebipụ n'ime ụlọ ọrụ), Pyoderma Gangrenosum (PG) bụ ọnọdụ autoimmune na-adịghị mma na nke autoinflammatory nke mkpụrụ ndụ ọbara ọcha (neutrophils) nke onye ọrịa na-ezighị ezi na-awakpo akpụkpọ ahụ mgbe ọ bụla ọ merụrụ ahụ. Mmeghachi omume ikwubiga okwu ókè na trauma bụ nke a na-akpọ pathergy akpụkpọ. Mgbe dọkịta na-awa ahụ na-emehie ihe ọnya PG na-egbuke egbuke maka ọrịa nje ma wepụ ihe mkpuchi akpụkpọ anụ ahụ na-acha odo odo, mkpụrụ ndụ akpụkpọ ahụ mebiri emebi na-ewepụta akara ngosi (interleukin-1 beta na TNF-alpha) nke na-akpọ ọtụtụ nde neutrophils ọhụrụ n'ọnụ ọnụ ọhụrụ. N'ime awa 24 ruo 48, ndị neutrophils na-agbari collagen dị mma n'akụkụ akụkụ ịwa ahụ, na-eme ka ọnya ahụ okpukpu abụọ ma ọ bụ okpukpu atọ n'ogo.

Kedu ka ndị dọkịta ga-esi gwa Pyoderma Gangrenosum mgbe arụchara ọrụ ma ewezuga ọrịa ọnya nke nje na-egbu egbu mgbe ịwachara ara ma ọ bụ afọ?¶

Ihe ngosi ụlọ ọgwụ anọ kewapụrụ Pyoderma Gangrenosum Postoperative (PPG) site na ọrịa ọnya nje: (1) Oge: PPG fọrọ nke nta ka ọ bụrụ na ọ na-amalite ụbọchị 4 ruo 14 mgbe ịwachara ahụ dị ka obere pustules na-adịghị mma n'akụkụ eriri mkpọ ahụ nke na-ejikọta na mberede na ọnya ahụ meghere, ebe ajọ ọrịa Group A Strep necrotizing ọrịa na-apụta n'ime awa 24; (2) Ọdịdị nke oke ala: PPG na-emepụta akụkụ akpụkpọ anụ boggy, gunmetal-purple, overhanging ("na-emebi emebi"); (3) Ịchekwa ọnụ ara n'ịwa ahụ ara: na mbelata ara ma ọ bụ nrụzigharị PPG, akpụkpọ anụ ara na-agbaze ebe a na-echekwa ihe mgbagwoju anya nke nipple-areolar; na (4) Ọdịda ọgwụ nje: PPG na-akawanye njọ n'agbanyeghị ọgwụ nje na-egbu egbu na-egbu egbu na ịwa ahụ, ma na-akwụsị ịgbasa n'ime awa 48 nke ịmalite corticosteroids dị elu.

Kedu ihe kpatara eji akpọ Pyoderma Gangrenosum "Pyoderma" ma ọ bụrụ na ọ nweghị nje nje?¶

Mgbe ndị ọkachamara n'ọrịa akpụkpọ anụ Brunsting, Goeckerman, na O'Leary bu ụzọ kpọọ ọnọdụ ahụ na 1930, ha kwenyere na ọ bụ ọrịa streptococcal na-agba ọsọ ma ọ bụ staphylococcal kpatara ya ("pyoderma" pụtara pus na akpụkpọ ahụ) nke butere ọnwụ anụ ahụ ("gangrenosum"). Ọgwụ ọgbara ọhụrụ mechara gosi na ọnyà na-acha odo odo na-ekpuchi isi nke ọnya PG ọhụrụ nwere nje bacteria efu; ọ bụ n'ezie ofe na-adịghị mma nke ọtụtụ nde mkpụrụ ndụ ọbara ọcha (neutrophils) na-eme nke ọma bụ nke rijuworo dermis n'ihi usoro ahụ́ ji alụso ọrịa ọgụ na-adịghị ebu ibu. Ọ bụ ezie na aha ahụ bụ akụkọ na-ezighị ezi, ọ dịgidere n'akwụkwọ nkà mmụta ọgwụ ruo ihe fọrọ nke nta ka ọ bụrụ otu narị afọ.

Kedu ọnọdụ ahụike dị n'okpuru na-ejikọta ya na Pyoderma Gangrenosum?¶

N'etiti pasent 50 na 70 nke ndị ọrịa a chọpụtara na Pyoderma Gangrenosum nwere ọrịa na-adịghị ala ala. Njikọ anọ a na-ahụkarị bụ: (1) Ọrịa afọ na-egbuke egbuke (Ulcerative Colitis na Crohn Disease, hụrụ na 20% ruo 30% nke ikpe); (2) Ọrịa ogbu na nkwonkwo (ọrịa ogbu na nkwonkwo na ankylosing spondylitis); (3) ọrịa cancer hematologic na ọrịa ọbara (kpọmkwem Acute Myeloid Leukemia [AML], Myelodysplastic Syndrome [MDS], na IgA Monoclonal Gammopathy, bụ nke jikọtara ya na ụdị dị iche iche nke PG na aka na ihu); na (4) Genetic autoinflammatory syndromes dị ka PAPA na ọrịa PASH.

Kedu nke na-arụ ọrụ ka mma maka Pyoderma Gangrenosum: Prednisone ma ọ bụ Cyclosporine?¶

Dị ka akara ngosi UK STOP GAP na-achịkwa ule a na-achịkwaghị achịkwa, oral Prednisolone (0.75 mg / kg / day) na oral Cyclosporine (4 mg / kg / day) na-adịkwa irè: ma kwụsị ọnya ọnya na-aga n'ihu n'otu ọsọ ahụ wee nweta ụdị ọgwụgwọ zuru oke (ihe fọrọ nke nta ka ọ bụrụ 50% gwọọ site na ọnwa isii). Ndị dọkịta na-ahọrọ n'etiti ha dabere na ọnọdụ ahụike ndị ọzọ nke onye ọrịa: Prednisone (ma ọ bụ IV Methylprednisolone) na-akwado ndị ọrịa nwere ọrịa akụrụ ma ọ bụ ọbara mgbali elu, ebe Cyclosporine na-enwe mmasị na ndị ọrịa nwere ọrịa shuga, oke ibu, osteoporosis, ma ọ bụ akụkọ ihe mere eme nke steroid na-akpata mmetụta psychiatric. Mgbe ejikọtara PG na ọrịa Crohn ma ọ bụ Ulcerative Colitis, Infliximab intravenous (5 mg/kg) bụ ọgwụgwọ usoro ndu ọla edo.

Isi mmalite na nduzi ọchịchị¶

• ** JAMA Dermatology Delphi International Consensus (Maverakis E, Ma C, Shinkai K, et al., 2018):** * Nchọpụta nchọpụta nke Ulcerative Pyoderma Gangrenosum: A Delphi Consensus of International Experts*.
• BMJ (Kwụsị GAP Randomized Controlled Trial, Ormerod AD, Thomas KS, Craig FE, et al., UK Dermatology Clinical Trials Network): * Ntụnyere ọgwụgwọ abụọ a na-ejikarị maka Pyoderma Gangrenosum: Nsonaazụ nke STOP GAP Randomized Controlled Trial (Prednisolone) vs. Ciclospor
• ** JAMA Dermatology (Jockenhovel F, et al.):** * Akara PARACELSUS: Ngwá Ọrụ Nchọpụta Ọhụụ maka Pyoderma Gangrenosum*.
• ** Òtù Na-ahụ Maka Ndị Na-ahụ Maka Ọrịa Ọkpụkpụ nke Britain (BAD) na Ụlọ Akwụkwọ American Academy of Dermatology (AAD): *** Ntuziaka nlekọta ahụike na Neutrophilic Dermatoses, Pyoderma Gangrenosum Nkwenye, na Mgbochi Pathergy *.
• Gut (Brooklyn TN, Dunnill MG, Shetty A, et al.): Infliximab maka ọgwụgwọ Pyoderma Gangrenosum: A Randomised, Double-ìsì, Plasebo-Controlled Trial.

Ntuziaka emetụtara¶

• Calciphylaxis (calcific uremic arteriolopathy): plaques violaceous, eschars ojii, na sodium thiosulfate.
• Erysipelas vs necrotizing fasciitis vs stasis dermatitis: mgbu nke oke, bullae, na LRINEC akara triage
• Nwanyị di ya nwụrụ latrodectism muscle rigidity vs brown recluse loxoscelism necrotic ọnyá afọ triage
• Necrotizing fasciitis (ọrịa na-eri anụ) na ọkọlọtọ ọbara ọbara cellulitis
• Nọmba mberede site na obodo