Pyoderma Gangrenosum presented by Jennifer Armstrong. UCI Emergency Medicine�, August 24, 2011
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UCI EMERGENCY MEDICINE
AUGUST 24, 2011
JENNIFER ARMSTRONG
Pyoderma Gangrenosum
Pyoderma gangrenosum
Uncommon noninfectious
ulcerative cutaneous
condition of uncertain
etiology.
What is Pyoderma Gangrenosum
Often mistaken as an
infection
Pustules form and give
way to ulcers with
necrotic, undermined
margin
Primarily sterile
inflammatory neutrophilic
dermatosis.
Epidemiology
•Any age 20-50
•F>M
•All races equal
•Takes on a
number of
differing
clinical
presentations
Pyoderma Gangrenosum: 2 varients
2 primary variants are a classic ulcerative
form
1 – Lower extremities
2 – Hands
More superficial
Called atypical
The different clinical types 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
Pathophysiology
• Poorly understood
• Dysregulation of the immune system, specifically altered
neutrophil chemotaxis, is believed to be involved
What to look for
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
Who is at risk 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
Key Clinical Findings
Starts quite 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
Treatment - Pyoderma Gangrenosum
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
Wound Care
All Cases
Saline soaked dressing
Occlusive dressing
discouraged
Until disease is controlled
Diagnosis
Often misdiagnosed
Diagnosed by its 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).