270 likes | 501 Views
The Scar That Wouldn’t Heal. Nancy Fuller, M.D. November 23, 2005. 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.
E N D
The Scar That Wouldn’t Heal Nancy Fuller, M.D. November 23, 2005
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
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
? Foreign body reaction? • Split thickness skin graft done; continued episodes of dehiscence • ?allergy to suture material? • Labs done: CBC, ESR, CRP, immunoglobulins, RF, ANA
Patient developed 2 new small lesions-started as pustules, progressed to small ulcers • Started on Prednisone and antibiotics • Tacrolimus added • Significant improvement!!
Objectives: • Consider pyoderma gangrenosum in differential for ulcerative skin lesions • Recognize potential problems in identification and diagnosis, treatment of PG • No financial disclosures
1930 : “rapidly progressive painful supperative cutaneous ulcers with edematous, boggy, undermined and necrotic borders”-coined “ pyoderma gangrenosum”
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?
Pyoderma Gangrenosum • Sweet's Disease • Generalized Pustular Psoriasis • Reactive Arthritis (Reiter’s Syndrome)- Balanitis, keratoderma blennorrhagica
Sweet’s Disease • Acute onset of fever/leukocytosis/erythematous plaques infiltrated by neutrophils • Uncommon • Female to male 4:1
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
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
Pyoderma Gangrenosum • Ulcerative chronic inflammatory skin lesions • Single or multiple • Most common on legs, but can be anywhere • Pathergy • Painful
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
Associated with underlying systemic diseases 50% of the time -Inflammatory bowel disease: 5% of ulcerative colitis, 2% Crohn’s -Inflammatory arthritis -lymphproliferative disorders
Differential diagnosis • Deep mycotic infections • Bacterial infections, including mycobacteria, • Herpes simplex • Vasculitis • Insect reactions (eg, brown recluse spider) • Warfarin skin necrosis • Factitial ulcer • gumma
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
Mistaken Identity? • Antiphospholipid syndrome • Wegeners granulomatosis • Chronic venous stasis ulcers • Vasculitis • Infection • Cancer (cutaneous lymphoma, etc)
Treatment • No well controlled studies • For mild disease: local treatment such as topical steroids, topical tacrolimus ointment, colloidal membrane dressings
For severe disease or failure with topical treatments: -steroids: 60-120 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
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!