Pyoderma gangrenosum

Background

  • 1/100,000 people per year in US, primarily 40s-50s year of age
  • Dysfunction of activated neutrophils
  • >50% associated with systemic disease:

Clinical Features

  • Extracutaneous manifestations (due to sterile neutrophilic infiltrates):
    • Culture-negative pulmonary infiltrates most common
    • cardiovascular, CNS, GI, eyes, liver, spleen, bones, lymph nodes
  • Initial lesion: bite-like, small, red papule or pustule (many patients attribute spider bite)
  • Changes into larger, ulcerative lesion
  • Two primary variants:
    • Classic ulcerative form - usually on legs, deep ulceration, violaceous border along ulcer bed
    • Superficial, atypical form - hands/forearms/face, vesiculopustular
    • Less common variants: 1) Peristomal pyoderma, 2) Genital pyoderma (must differentiate from STI), 3) Pyostomatitis vegetans (intraoral)

Differential Diagnosis

  • Systemic diseases
    • Neutrophilic dermatoses (Sweet, Behcet's disease, etc.)
    • Calciphylaxis - very important to differentiate due to treatment
    • Arterial/venous insufficiency
    • Blastomycosis
    • Hidradenitis suppurative
    • TB gumma, mycobacterial infections
    • Malignancy (squamous cell)
    • Vasculitis
    • Ecthyma gangrenosum (pseudomonas)

Evaluation

  • Diagnosis of exclusion
  • No specific criteria
  • CBC, CMP, UA, hepatitis profile, coags, VDRL
  • Autoimmune labs: ANCA, antiphospholipid antibody
  • Tissue cultures (comprehensive, all organisms)
    • Coags
    • Anti-phospholipid antibody
    • Referral for heme malignancy workup
    • Referral for IBD workup
  • CXR if systemic disease suspected

Management

  • Nonsurgical management
    • Necrotic tissue should be gently removed
    • Wide surgical debridement results in enlargement of ulcer
  • Medical management

Disposition

  • Usually discharge with derm and/or surgery follow-up and referral for IBD and malignancy workup

See Also

References

    This article is issued from Wikem. The text is licensed under Creative Commons - Attribution - Sharealike. Additional terms may apply for the media files.