Pyoderma gangrenosum: Difference between revisions
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**[[arthritis|Polyarthritis]], sero-negative and -positive | **[[arthritis|Polyarthritis]], sero-negative and -positive | ||
**[[Leukemia]] | **[[Leukemia]] | ||
**Less commonly [[psoriatic arthritis]], [[osteoarthritis]], [[RA]], spondyloarthopathy, [[hepatitis]], primary biliary [[cirrhosis]], [[multiple myeloma]], [[SLE]], | **Less commonly [[psoriatic arthritis]], [[osteoarthritis]], [[RA]], spondyloarthopathy, [[hepatitis]], primary biliary [[cirrhosis]], [[multiple myeloma]], [[SLE]], [[Sjögren Syndrome]] | ||
==Clinical Features== | ==Clinical Features== | ||
[[File:Crohnie Pyoderma gangrenosum.jpg|thumb|Pyoderma gangrenosum on the leg of a person with [[ulcerative colitis]].]] | |||
[[File:Pyoderma gangrenosum 01.jpg|thumb|Pyoderma gangrenosum of upper back.]] | |||
*Extracutaneous manifestations (due to sterile neutrophilic infiltrates): | *Extracutaneous manifestations (due to sterile neutrophilic infiltrates): | ||
**Culture-negative pulmonary infiltrates most common | **Culture-negative pulmonary infiltrates most common | ||
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**[[Vasculitis]] | **[[Vasculitis]] | ||
**Ecthyma gangrenosum (pseudomonas) | **Ecthyma gangrenosum (pseudomonas) | ||
==Evaluation== | ==Evaluation== | ||
Latest revision as of 13:23, 12 December 2020
Background
- 1/100,000 people per year in US, primarily 40s-50s year of age
- Dysfunction of activated neutrophils
- >50% associated with systemic disease:
- IBD
- Polyarthritis, sero-negative and -positive
- Leukemia
- Less commonly psoriatic arthritis, osteoarthritis, RA, spondyloarthopathy, hepatitis, primary biliary cirrhosis, multiple myeloma, SLE, Sjögren Syndrome
Clinical Features
Pyoderma gangrenosum on the leg of a person with ulcerative colitis.
- 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
- Small ulcers
- Topical steroids and/or steroid injections, silvadene compression dressings
- Dapsone or minocycline PO
- Severe ulcers
- Corticosteroids
- Immunologic treatment/biologics
- IVIG, plasmapheresis
- Small ulcers
Disposition
- Usually discharge with derm and/or surgery follow-up and referral for IBD and malignancy workup