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Patient handout

Pyoderma gangrenosum (neutrophilic ulcerative dermatosis)

PRODUCTION

1. Your condition

This handout is for pyoderma gangrenosum (neutrophilic ulcerative dermatosis). Your care team identified this based on: rapidly enlarging, exquisitely painful ulcer with a violaceous, undermined, overhanging border (paracelsus — violaceous border in 98% of pg; jockenhöfer br j dermatol 2019 pmid 29388188).

Other reasons your team may use this plan: a papule/pustule/vesicle that ulcerated within ~4 days of appearing (maverakis delphi minor criterion; jama dermatol 2018 pmid 29450466); ulcer that enlarged after surgical debridement, biopsy, or minor trauma — pathergy; commonly misdiagnosed as wound/necrotising infection (pmid 31498172); painful ulcer not responding to antibiotics with sterile cultures — the dominant infective-misdiagnosis entry (shaigany arch dermatol res 2021 pmid 34714405).

2. Your medications

Take these medications exactly as prescribed. Do not stop or change a dose without talking to your provider.

MedicationStarting doseHowWhenWhat it does
avoid_surgical_debridement_and_aggressive_surgery_pathergyPMID 31498172 — PG is routinely misdiagnosed as wound/necrotising infection and surgically debrided, which enlarges it catastrophically via pathergy. NO debridement / aggressive surgery. This rule precedes all therapy.
exclusion_workup_tissue_culture_edge_biopsy_associated_diseaseMaverakis Delphi (PMID 29450466) — PG has no confirmatory test: 1 major (edge-biopsy neutrophilic infiltrate, supportive only) + 8 minor, ≥4/8 → Sn 86% / Sp 90%. Biopsy EXCLUDES mimics; it does not confirm. Mandatory associated-disease workup (IBD/heme/arthritis).
meticulous_moist_nonadherent_wound_care_and_analgesiaPartridge Br J Dermatol 2018 (PMID 29478243) — gentle moist non-adherent dressings + adequate analgesia (pain is severe and out of proportion) in parallel with immunosuppression; autolytic, NOT surgical, debridement only.

Plan: Pyoderma gangrenosum — exclusion-first immunosuppressive ladder by extent/tempo (Maverakis Delphi PMID 29450466; STOP GAP PMID 26071094; Partridge PMID 29478243)

3. When to call your provider

Contact your care team if any of the following happen:

  • Rapidly progressive / fulminant / systemically toxic PG → admit + IV immunosuppression (Partridge PMID 29478243)
  • Ulcer enlarging after a debridement/surgery (pathergy) → STOP surgery, immunosuppress, recognise misdiagnosis (PMID 31498172)
  • True necrotising soft-tissue infection features (crepitus/gas/sepsis) → route OUT to id.necrotising-fasciitis.core.v1 (surgical emergency)

4. When to seek emergency care

Call 911 or go to the nearest emergency room right away if you have:

  • Rapidly progressive / fulminant / multifocal PG with systemic toxicity (fever, malaise, rapid expansion) (Partridge Br J Dermatol 2018 PMID 29478243)(life-threatening)
  • Ulcer misdiagnosed as wound/necrotising infection and surgically debrided, now enlarging — pathergy (Flynn Adv Skin Wound Care 2019 PMID 31498172)
  • PG with a newly identified haematologic malignancy / myelodysplasia / IgA monoclonal gammopathy (worse prognosis) (Shaigany Arch Dermatol Res 2021 PMID 34714405; Partridge PMID 29478243)
  • Genuine necrotising soft-tissue infection features (crepitus, gas, fulminant sepsis, true rapid necrosis) — NOT PG (IDSA SSTI; PMID 31498172)(life-threatening)

5. Follow-up

Chronic surveillance: continued meticulous moist non-adherent wound care + analgesia, slow steroid taper with steroid-sparing maintenance, recurrence vigilance (PG recurs in ~28-30% — STOP GAP), pathergy education (avoid elective surgery/debridement; flag PG to any future surgeon; perioperative immunosuppressive cover if surgery unavoidable), cribriform-scar counselling, and ongoing associated-disease monitoring (repeat heme screen if new cytopenia; IBD/arthritis activity). Dermatology continuity for any systemic agent.

6. Sources

Guideline: Maverakis et al, Diagnostic Criteria of Ulcerative Pyoderma Gangrenosum — Delphi consensus (JAMA Dermatol 2018; PMID 29450466; DOI 10.1001/jamadermatol.2017.5980) + Jockenhöfer et al PARACELSUS diagnostic score (Br J Dermatol 2019; PMID 29388188; DOI 10.1111/bjd.16401) + Ormerod/Thomas STOP GAP ciclosporin-vs-prednisolone RCT (BMJ 2015; PMID 26071094; DOI 10.1136/bmj.h2958) + Brooklyn et al infliximab RCT (Gut 2006; PMID 16188920; DOI 10.1136/gut.2005.074815) + Partridge et al systematic review of systemic therapy (Br J Dermatol 2018; PMID 29478243; DOI 10.1111/bjd.16485)

  1. pubmed.ncbi.nlm.nih.gov/29450466
  2. pubmed.ncbi.nlm.nih.gov/29388188
  3. pubmed.ncbi.nlm.nih.gov/26071094