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pyoderma gangrenosum

Etiology: 1) idiopathic 30-50% 2) inflammatory bowel disease a) ulcerative colitis (15%) b) Crohn's disease (26%) c) may occur adjacent to enterostomy site [4] 3) inflammatory arthritis a) rheumatoid arthritis, seropositive (21%) b) seronegative spondyloarthropathy [4] 4) malignancies & related conditions (12%) a) myelocytic leukemia (bullous form) - acute myelocytic leukemia (AML) - chronic myelocytic leukemia (CML) b) myelodysplastic syndrome (5%) c) lymphoproliferative disorders - monoclonal gammopathy - multiple myeloma - benign monoclonal gammopathy d) polycythemia vera 5) hidradenitis suppurativa (6%) [13] 6) chronic active hepatitis 7) cutaneous anergy (pathergy) (28%) Epidemiology: - mean age 52 years, 32% > 65 years [13] - 75% women Pathology: 1) neutrophilic, ulcerative skin disease [4] 2) histopathologic findings are non-specific 3) biopsy in of itself cannot be diagnostic 4) special stains to exclude other causes a) infection (bacterial, viral, fungal, mycobacterial) b) neoplasm (immunohistochemical stains) Clinical manifestations: 1) ulcerating, painful skin lesions with characteristic borders with a necrotic blueish edge (overhanging border) & a peripheral erythematous halo 2) lesions begin as small pustules with rapid evolution of central necrosis 4) ulcers with irregular, undermined, inflammatory, violaceous borders 5) lesions predominantly on the lower extremities 6) lesions occur at sites of trauma 7) ulcers heal with cribriform scarring [4] * images [10,11] 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: 1) complete blood count 2) serum chemistres a) renal function test b) liver function test c) serum iodide d) serum bromide 3) hepatitis panel 4) urinalysis 5) serum & urine protein electrophoresis - immunofixation electrophoresis (IFE) as indicated 6) antinuclear antibody 7) antiphospholipid antibody 8) ANCA 9) rheumatoid factor, anti-CCP in serum 10) erythrocyte sedimentation rate 11) cryoglobulins 11) biopsy (see pathology) a) tissue culture for bacteria, mycobacteria & fungi b) viral cultures on selected individuals c) bone marrow biopsy as indicated 12) age & gender appropriate cancer screening Special laboratory: - gastrointestinal endoscopy as indicated - EGD & colonoscopy - venous & arterial flow studies as indicated - ultrasound doppler Radiology: - chest C-ray to rule out a) pulmonary infection b) systemic vasculitis with pulmonary involvement Differential diagnosis: 1) Meleny's ulcer 2) dimorphic fungi 3) cutaneous amebiasis 4) spider bites 5) factitial ulcers Management: 1) evaluate underlying causes as appropriate 2) cyclosporine vs glucocorticoids depending upon cormorbid factors [9] a) neither are very effective [9] b) cyclosporine A (Sandimmune) 3 mg/kg divided BID - baseline BUN/creatinine - weekly renal function tests c) glucocorticoids - potent topical glucocorticoid - topical clobetasol [4] - 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] 3) infliximab may be of benefit [4] 4) anakinra may be of benefit [6] 5) sulfasalazine 6) sulfones 7) debridement of wound is contraindicated - may extend ulcer

Related

ecthyma gangrenosum PAPA syndrome; pyogenic sterile arthritis, pyoderma gangrenosum, & acne; familial recurrent arthritis

Specific

pyostomatitis vegetans

General

cutaneous ulcer neutrophilic dermatosis

References

  1. Harrison's Principles of Internal Medicine, 13th ed. Isselbacher et al (eds), McGraw-Hill Inc. NY, 1994, pg 307
  2. Color Atlas & Synopsis of Clinical Dermatology, Common & Serious Diseases, 3rd ed, Fitzpatrick et al, McGraw Hill, NY, 1997, pg 396-97
  3. Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 171, 174
  4. Medical Knowledge Self Assessment Program (MKSAP) 11, 15, 16, 17, 18. American College of Physicians, Philadelphia 1998, 2009, 2012, 2015, 2018
  5. Callen JP and Jackson JM, Pyoderma gangrenosum: an update Rheum Dis Clin of North Am 2007 33:787 PMID: 18037117
  6. 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: 19673875
  7. 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: 19470075
  8. 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: 20227580
  9. 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: 26071094
  10. Jackson JM, James WD (images) Medscape: Pyoderma Gangrenosum http://emedicine.medscape.com/article/1123821-overview
  11. DermNet NZ. Pyoderma gangrenosum (images) http://www.dermnetnz.org/reactions/pyoderma-gangrenosum.html
  12. 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: 21410617
  13. 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: 29450453 https://jamanetwork.com/journals/jamadermatology/fullarticle/2672271
  14. Pompeo MQ. Pyoderma Gangrenosum:Recognition and Management. Wounds. 2016 Jan;28(1):7-13. Review. PMID: 26779805 Free Article
  15. 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: 27060666
  16. Hou YL, Lee CH Images in Dermatology. Pyoderma Gangrenosum JAMA Dermatol. Published online December 15, 2021 PMID: 3491008 https://jamanetwork.com/journals/jamadermatology/fullarticle/2787273
  17. 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: 29653213 Review.