PG pyoderma gangrenosum

Question 1

What are the associations

Answer 1

• IBD
• heme disorder (IgA monoclonal gammopathy, AML, CML, hairy cell leukemia, polycythemia vera)
• Inflammatory arthritis

**while sweet’s syndrome is a/w pregnancy, IBD, and heme cancers

Question 2

pathogenesis

Answer 2

immunologic disorder; some cases are caused by mutation CD2-binding protein; + pathergy

Question 3

what are the major types?

Answer 3

classic, bullous, pustular, superficial granulomatous/vegetative

• classic PG in kids a/w cancer (LEUKEMIA) and IBD
• bullous is a/w heme cancer (AML)
• pustular is a/w IBD
• vegetative is the least aggressive

Question 4

clinical presentation for classic, bullous, pustular, vegetative

Answer 4

• painful ulcer with overhanging violaceous border and purulent base; satellite lesions at periphery of ulcer
• classic PG in kids –> on head or anogenital region
• pustular –> multiple small pustules that do NOT ulcerate
• bullous –> more widespread, less destructive
• vegetative –> superficial, painless cribriform ulcers on trunk

Question 5

pathology

Answer 5

epidermal ulceration with superficial and deep dermal neutrophilic infiltrate

Question 6

is this a diagnosis of exclusion

Answer 6

HELL YEAH

Question 7

Tx

Answer 7

wound care, must evaluate underlying diseases, and/or topical/intralesional/systemic steroids

for very severe disease, consider infliximab and cyclosporine