pyoderma gangrenosum
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Etiology
- idiopathic 30-50%
- inflammatory bowel disease
- ulcerative colitis (15%)
- Crohn's disease (26%)
- may occur adjacent to enterostomy site[4]
- inflammatory arthritis
- malignancies & related conditions (12%)
- myelocytic leukemia (bullous form)
- myelodysplastic syndrome (5%)
- lymphoproliferative disorders
- polycythemia vera
- hidradenitis suppurativa (6%)[13]
- chronic active hepatitis
- cutaneous anergy (pathergy) (28%)
Epidemiology
Pathology
- neutrophilic, ulcerative skin disease[4]
- histopathologic findings are non-specific
- biopsy in of itself cannot be diagnostic
- special stains to exclude other causes
- infection (bacterial, viral, fungal, mycobacterial)
- neoplasm (immunohistochemical stains)
Clinical manifestations
- ulcerating, painful skin lesions with characteristic borders with a necrotic blueish edge (overhanging border) & a peripheral erythematous halo
- lesions begin as small pustules with rapid evolution of central necrosis
- ulcers with irregular, undermined, inflammatory, violaceous borders
- lesions predominantly on the lower extremities
- lesions occur at sites of trauma
- ulcers heal with cribriform scarring[4]
Diagnostic criteria
- major criteria
- rapidly progressing painful ulcer with irregular, violaceous, & undermined border
- exclusion of other causes of ulceration[4]
- minor criteria
- skin biopsy shows sterile inflammation with neutrophils
- pathergy or healing with cribriform scarring
- systemic disease associated with pyoderma gangrenosum
- modest response to systemic glucocorticoid[4]
- 50% reduction in lesion size in 4 weeks
Laboratory
- complete blood count
- serum chemistres
- hepatitis panel
- urinalysis
- serum & urine protein electrophoresis
- immunofixation electrophoresis (IFE) as indicated
- antinuclear antibody
- antiphospholipid antibody
- ANCA
- rheumatoid factor, anti-CCP in serum
- erythrocyte sedimentation rate
- cryoglobulins
- biopsy (see pathology)
- tissue culture for bacteria, mycobacteria & fungi
- viral cultures on selected individuals
- bone marrow biopsy as indicated
- age & gender appropriate cancer screening
Diagnostic procedures
- gastrointestinal endoscopy as indicated
- venous & arterial flow studies as indicated
Radiology
- chest C-ray to rule out
- pulmonary infection
- systemic vasculitis with pulmonary involvement
Differential diagnosis
Management
- evaluate underlying causes as appropriate
- cyclosporine vs glucocorticoids depending upon cormorbid factors[9]
- neither are very effective[9]
- cyclosporine A (Sandimmune) 3 mg/kg divided BID
- baseline BUN/creatinine
- weekly renal function tests
- glucocorticoids
- potent topical glucocorticoid
- IV pulse therapy
- up to 1-2 g of prednisolone/day may be required
- high dose prednisone
- intralesional triamcinolone
- 50% reduction in lesion size in 4 weeks[4]
- infliximab may be of benefit[4]
- anakinra may be of benefit[6]
- sulfasalazine
- sulfones
- debridement of wound is contraindicated
- may extend ulcer
More general terms
More specific terms
Additional terms
- ecthyma gangrenosum
- PAPA syndrome; pyogenic sterile arthritis, pyoderma gangrenosum, & acne; familial recurrent arthritis
References
- ↑ Harrison's Principles of Internal Medicine, 13th ed. Isselbacher et al (eds), McGraw-Hill Inc. NY, 1994, pg 307
- ↑ Color Atlas & Synopsis of Clinical Dermatology, Common & Serious Diseases, 3rd ed, Fitzpatrick et al, McGraw Hill, NY, 1997, pg 396-97
- ↑ Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 171, 174
- ↑ 4.0 4.1 4.2 4.3 4.4 4.5 4.6 4.7 4.8 4.9 Medical Knowledge Self Assessment Program (MKSAP) 11, 15, 16, 17, 18. American College of Physicians, Philadelphia 1998, 2009, 2012, 2015, 2018
- ↑ Callen JP and Jackson JM, Pyoderma gangrenosum: an update Rheum Dis Clin of North Am 2007 33:787 PMID: https://www.ncbi.nlm.nih.gov/pubmed/18037117
- ↑ 6.0 6.1 Brenner M et al. Targeted treatment of pyoderma gangrenosum in PAPA (pyogenic arthritis, pyoderma gangrenosum and acne) syndrome with the recombinant human interleukin-1 receptor antagonist anakinra. Br J Dermatol 2009 Nov; 161:1199. PMID: https://www.ncbi.nlm.nih.gov/pubmed/19673875
- ↑ Ruocco E, Sangiuliano S, Gravina AG, Miranda A, Nicoletti G. Pyoderma gangrenosum: an updated review. J Eur Acad Dermatol Venereol. 2009 Sep;23(9):1008-17 PMID: https://www.ncbi.nlm.nih.gov/pubmed/19470075
- ↑ Miller J, Yentzer BA, Clark A, Jorizzo JL, Feldman SR. Pyoderma gangrenosum: a review and update on new therapies. J Am Acad Dermatol. 2010 Apr;62(4):646-54 PMID: https://www.ncbi.nlm.nih.gov/pubmed/20227580
- ↑ 9.0 9.1 9.2 Ormerod AD et al. Comparison of the two most commonly used treatments for pyoderma gangrenosum: Results of the STOP GAP randomised controlled trial. BMJ 2015; 350:h2958 PMID: https://www.ncbi.nlm.nih.gov/pubmed/26071094
- ↑ 10.0 10.1 Jackson JM, James WD (images) Medscape: Pyoderma Gangrenosum http://emedicine.medscape.com/article/1123821-overview
- ↑ 11.0 11.1 DermNet NZ. Pyoderma gangrenosum (images) http://www.dermnetnz.org/reactions/pyoderma-gangrenosum.html
- ↑ Dabade TS, Davis MD. Diagnosis and treatment of the neutrophilic dermatoses (pyoderma gangrenosum, Sweet's syndrome). Dermatol Ther. 2011 Mar-Apr;24(2):273-84 PMID: https://www.ncbi.nlm.nih.gov/pubmed/21410617
- ↑ 13.0 13.1 13.2 Ashchyan HJ, Butler DC, Nelson CA et al. The association of age with clinical presentation and comorbidities of pyoderma gangrenosum. JAMA Dermatol 2018 Apr 1; 154:409. PMID: https://www.ncbi.nlm.nih.gov/pubmed/29450453 https://jamanetwork.com/journals/jamadermatology/fullarticle/2672271
- ↑ Pompeo MQ. Pyoderma Gangrenosum:Recognition and Management. Wounds. 2016 Jan;28(1):7-13. Review. PMID: https://www.ncbi.nlm.nih.gov/pubmed/26779805 Free Article
- ↑ Herberger K, Dissemond J, Hohaus K et al Treatment of pyoderma gangrenosum: retrospective multicentre analysis of 121 patients. Br J Dermatol. 2016 Nov;175(5):1070-1072. PMID: https://www.ncbi.nlm.nih.gov/pubmed/27060666
- ↑ Hou YL, Lee CH Images in Dermatology. Pyoderma Gangrenosum JAMA Dermatol. Published online December 15, 2021 PMID: https://www.ncbi.nlm.nih.gov/pubmed/3491008 https://jamanetwork.com/journals/jamadermatology/fullarticle/2787273
- ↑ Ashchyan HJ, Nelson CA, Stephen S et al Neutrophilic dermatoses: Pyoderma gangrenosum and other bowel- and arthritis-associated neutrophilic dermatoses. J Am Acad Dermatol. 2018 Dec;79(6):1009-1022 PMID: https://www.ncbi.nlm.nih.gov/pubmed/29653213 Review.