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The Scar That Wouldn’t Heal Nancy Fuller, M.D. November 23, 2005.

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Presentation on theme: "The Scar That Wouldn’t Heal Nancy Fuller, M.D. November 23, 2005."— Presentation transcript:

1 The Scar That Wouldn’t Heal Nancy Fuller, M.D. November 23, 2005

2 55 year old woman with skin lesion on back Referred to Derm for removal Dx: basal cell carcinoma Wide reexcision done after dx established Wound dehiscence 2 weeks later, treated with antibiotics with no improvement

3 Over the next 8 months: progressive and persistent dehiscence Resuturing Stapling Bx: supperative and granulomatous dermatitis, dermal scar and chronic FB rx Cultures for fungus, mycobacteria, bacteria

4 ? Foreign body reaction? Split thickness skin graft done; continued episodes of dehiscence ?allergy to suture material? Labs done: CBC, ESR, CRP, immunoglobulins, RF, ANA

5 Patient developed 2 new small lesions- started as pustules, progressed to small ulcers Started on Prednisone and antibiotics Tacrolimus added Significant improvement!!

6 Dx: Pyoderma Gangrenosum

7 Objectives: Consider pyoderma gangrenosum in differential for ulcerative skin lesions Recognize potential problems in identification and diagnosis, treatment of PG No financial disclosures

8 1930 : “rapidly progressive painful supperative cutaneous ulcers with edematous, boggy, undermined and necrotic borders”-coined “ pyoderma gangrenosum”

9 Neutrophilic Dermatoses Intense epidermal and/or dermal inflammatory infiltrates Composed mainly of neutrophils No evidence of vasculitis or infection Pathogenesis: unknown; ?cytokine disregulation? Altered immune reactivity?

10 Pyoderma Gangrenosum Sweet's Disease Generalized Pustular Psoriasis Reactive Arthritis (Reiter’s Syndrome)- Balanitis, keratoderma blennorrhagica

11 Sweet’s Disease Acute onset of fever/leukocytosis/erythematous plaques infiltrated by neutrophils  Uncommon  Female to male 4:1

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13 Associated with many underlying diseases:  Malignancies(25%)-most hematopoetic  Bacterial infections-strep, mycobacterium, yersinia, typhus, salmonella  Vaccinations  Viral infections-CMV, CAH, HIV  Drugs-lithium, furosemide, OCPs, TMP/SMZ  Autoimmune and Collagen vascular diseases-RA, SLE, MCTD, Behcet’s,Hashimoto’s thyroiditis  IBD-Crohns, Ulcerative colitis

14 Diagnostic Criteria:  MAJOR: abrupt onset of typical lesions  Histopathology consistent  MINOR: antecedent fever or infection  Accompanying fever, arthralgias  Leukocytosis  Good response to systemic corticosteroids, not to antibiotics

15 Pyoderma Gangrenosum Ulcerative chronic inflammatory skin lesions Single or multiple Most common on legs, but can be anywhere Pathergy Painful

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17 Rapid progression of ulceration Usually preceded by a papule, pustule, or vesicle Histopathology depends on stage, but always dense neutrophilic infiltrates No evidence of vasculitis on bx

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20 Associated with underlying systemic diseases 50% of the time -Inflammatory bowel disease: 5% of ulcerative colitis, 2% Crohn’s -Inflammatory arthritis -lymphproliferative disorders

21 Differential diagnosis Deep mycotic infections Bacterial infections, including mycobacteria, Herpes simplex Vasculitis Insect reactions (eg, brown recluse spider) Warfarin skin necrosis Factitial ulcer gumma

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23 Diagnosis All patients with suspected PG: must rule out other causes of ulcers prior to tx Skin biopsy Labs: CBC, ESR/CRP, LFTs, renal function studies, SPEP, CXR, coag profile, ANCA, cryoglobulins

24 Mistaken Identity? Antiphospholipid syndrome Wegeners granulomatosis Chronic venous stasis ulcers Vasculitis Infection Cancer (cutaneous lymphoma, etc)

25 Treatment No well controlled studies For mild disease: local treatment such as topical steroids, topical tacrolimus ointment, colloidal membrane dressings

26 For severe disease or failure with topical treatments: -steroids: mg prednisone per day pulse methylprednisolone For refractory cases: dapsone, thalidomide, mycophenolate, cyclosporine, azothioprine, IVIG Surgery: split thickness skin grafts; also must use systemic immunosuppression

27 Conclusions PG-fortunately uncommon Diagnosis of exclusion because of the lack of any specific diagnosis certainties Big mimicker Treatment often requires major immunosuppression Keep it in your differential!


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