Pyoderma gangrenosum

Question 1

Pyoderma Gangrenosum age of onset:

Answer 1

adults 20-60 y/o

Question 2

What percent of Pyoderma Gangrenosum patients have underlying systemic inflammatory disorder?

Answer 2

50%

Question 3

What is most common underlying disease in Pyoderma Gangrenosum? What are some other associations with PG?

Answer 3

• IBD (30%)
• IgA monoclonal gammopathy, AML, CML, hairy cell leukemia, polycythemia vera, inflammatory arthritis

Question 4

Pyoderma Gangrenosum showing up/worsening at sites of trauma is called:

Answer 4

Pathergy

Question 5

What is the proposed pathogenesis of Pyoderma Gangrenosum?

Answer 5

Likely immunologic

Question 6

What are the different types of Pyoderma Gangrenosum?

Answer 6

• classic (ulcerative)
• bullous (a/w myeloproliferative disorders)
• pustular
• superficial granulomatous

Question 7

Presentation of classic Pyoderma Gangrenosum:

Answer 7

starts as inflamed papulopustule/bulla-→ painful undermining ulcer w/ overhanging, irregular violaceous border and purulent/vegetative base; satellite lesions arise at periphery of ulcer

Question 8

How does Pyoderma Gangrenosum heal?

Answer 8

with atrophic cribriform scar

Question 9

Pustular Pyoderma Gangrenosum presents as:

Answer 9

• multiple small pustules that do not progress to ulcers
• usually a/w IBD

Question 10

Bullous Pyoderma Gangrenosum is more strongly a/w______

Answer 10

hematologic malignancy

Question 11

Vegetative Pyoderma Gangrenosum presents as:

Answer 11

superficial, painless cribriform ulcers on the trunk

• least aggressive form
• usually arises as a result of trauma (surgery)

Question 12

Histology of Pyoderma Gangrenosum:

Answer 12

• epidermal ulceration w/ dense underlying superficial and deep dermal neutrophilic infiltrate
• neutrophilic infiltrate extends laterally beyond overlying ulcer (“undermining infiltrate”)
• Inflam is deeper than sweets

Question 13

Pyoderma Gangrenosum is a diagnosis of exclusion and we must rule out:

Answer 13

• infection
• vasculitis
• vasculopathy
• malignancy

Question 14

What are treatments for Pyoderma Gangrenosum for mild, mod-severe, and recalcitrant disease?

Answer 14

• mild: high potency topical CS or intralesional
• mod-severe: Prednisone 0.5-2 mg/kg/day
• Recalcitrant: Infliximab and cyclosporine
• Good wound care is essential

Question 15

What workup do you do in Pyoderma Gangrenosum patient?

Answer 15

• GI: refer for possible colonoscopy, get P-ANCA and C-ANCA to check for IBD
• Heme: CBC w/ diff, peripheral smear, SPEP, UPEP
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