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Pyoderma Gangrenosum
Pyoderma gangrenosum is an uncommon inflammatory skin condition of unknown cause characterized by sterile pustules that form ulcers with necrotic margins. It typically affects adults ages 20-50 and presents as painful lesions that can develop rapidly. While the pathophysiology is poorly understood, it involves dysregulation of the immune system and neutrophils. Treatment involves immunosuppressants like corticosteroids and cyclosporine. Proper diagnosis requires ruling out infection and avoiding biopsy or surgery, as trauma can worsen lesions.
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Pyoderma Gangrenosum
- 1. UCI EMERGENCY MEDICINE AUGUST24, 2011 JENNIFER ARMSTRONG Pyoderma Gangrenosum
- 2. Pyoderma gangrenosum Uncommon noninfectious ulcerativecutaneous condition of uncertain etiology.
- 3. What is PyodermaGangrenosum Often mistaken as an infection Pustules form and give way to ulcers with necrotic, undermined margin Primarily sterile inflammatory neutrophilic dermatosis.
- 4. Epidemiology •Any age 20-50 •F>M •Allraces equal •Takes on a number of differing clinical presentations
- 5. Pyoderma Gangrenosum: 2varients 2 primary variants are a classic ulcerative form 1 – Lower extremities 2 – Hands More superficial Called atypical
- 6. The different clinicaltypes of PG Variants-Typical findings Ulcerative PG Ulceration with rapidly evolving purulent wound ground Pustular PG Discrete pustules, sometimes self-limited, commonly associated with inflammatory bowel disease Bullous PG Superficial bullae with development of ulcerations Vegetative PG Erosions and superficial ulcers
- 7. Pathophysiology • Poorly understood •Dysregulation of the immune system, specifically altered neutrophil chemotaxis, is believed to be involved
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- 9. What to lookfor Begin as extremely painful solitary nodules Deep seated pustules Rupture and form a shaggy ulcer Some pustules do not progress to ulcers Boarders are deep violaceous or dusky color Bright erythema extend from the ulcer Can be lesions of oral mucosa, vulva, eyes Necrosis is common Purulent coating in the center common Can have oder
- 10. Who is atrisk of pyoderma gangrenosum? Pyoderma gangrenosum often affects a person with an underlying internal disease such as Inflammatory bowel diseases (ulcerative colitis and Crohn disease) Rheumatoid arthritis Myeloid blood dyscrasias Chronic active hepatitis Wegener granulomatosis
- 11. Key Clinical Findings Startsquite suddenly, often at the site of a minor injury. It may start as a small pustule, red bump or blood-blister. The skin then breaks down resulting in an ulcer which can deepen and widen rapidly. Any trauma worsens the lesion
- 12. Treatment - PyodermaGangrenosum Options Mild • Intralesional corticosteroids Moderate/Severe • Prednisone: 0.5-2mg/kg PO. Divided into 4x daily • Usually weeks to months • Cyclosporine-A: 0.5-1.0 mg /kg/day. Divide BID • Rapid response and marked improvement of pain Surgical • NEVER! • NO I/D, NO biopsy Referral • Dermatology for management is indicated for multiple lesions
- 13. Wound Care All Cases Saline soaked dressing Occlusive dressing discouraged Until disease is controlled
- 14. Diagnosis Often misdiagnosed Diagnosed byits characteristic appearance. There is no specific test. The wound should be swabbed and cultured for micro- organisms, but these are not the cause of pyoderma gangrenosum. Mostly, blood tests are not particularly helpful. Some patients may have a positive ANCA. The pathergy test is usually positive (a skin prick test causing a papule, pustule or ulcer).
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