Pyoderma gangrenosum: Difference between revisions
ClaireLewis (talk | contribs) (Created page with "==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 **arth...") |
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***[[Corticosteroids]] | ***[[Corticosteroids]] | ||
***Immunologic treatment/biologics | ***Immunologic treatment/biologics | ||
***[[IVIG]], plasmapheresis | ***[[IVIG]], [[plasmapheresis]] | ||
==Disposition== | ==Disposition== | ||
Revision as of 17:52, 3 April 2017
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, Sjogren
Clinical Features
- 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)
- Papules
- Insect bites
- Scabies
- Seabather's eruption
- Cercarial dermatitis (Swimmer's Itch)
- Macular
- Sub Q Swelling and Nodules
- Ulcers
- Tropical pyoderma
- Leishmaniasis
- Mycobacterium marinum
- Buruli ulcer
- Dracunculiasis (Guinea Worm disease)
- Linear and Migratory Lesions
- Cutaneous larvae migrans
- Photodermatitis
See also domestic U.S. ectoparasites
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