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Pyoderma Gangrenosum

Pyoderma Gangrenosum. An idiopathic ulcerative neutrophilic inflammatory skin disease characterized by variable clinical presentations and outcomesEqual sex distributionCommon in middle-aged adults (30-50yr)About 5% affects childrenIncidence unknownEstimated at 3 per million in US. Clinical Man

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Pyoderma Gangrenosum

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    1. Pyoderma Gangrenosum Christina Kahl August 22, 2007

    2. Pyoderma Gangrenosum An idiopathic ulcerative neutrophilic inflammatory skin disease characterized by variable clinical presentations and outcomes Equal sex distribution Common in middle-aged adults (30-50yr) About 5% affects children Incidence unknown Estimated at 3 per million in US

    3. Clinical Manifestations Anatomic sites – primarily legs, but can occur anywhere Abdomen, breast, buttucks, arms Single or multiple lesions Skin lesions arise suddenly as painful, erythematous, and tender papules, pustules, vesicles, or fluctuant nodules Rapid progression to expanding ulcers with sharply demarcated, livid borders and a necrotic base Ulcer edge often undermined (worn and ragged) Surrounding skin – erythematous and indurated

    4. Clinical Manifestations Koebner's phenomenon (also called the isomorphic response) The appearance of a skin lesion as a result of trauma Pathergy Exaggerated, uncontrolled, inflammatory response to a nonspecific stimulus Lesions develop at site of minor trauma in 25-50% Surgery or debridement contraindicated

    5. Clinical Manifestations Patients appear systemically unwell Fever, malaise, arthralgias, myalgias Lesions often severely painful Ulcerative PG (classic form) Peristomal PG Occurs close to abdominal stomas Associated with inflammatory bowel disease; malignancy

    6. Clinical Manifestations Pustular PG Rare, process halts at pustule stage and does not evolve into ulcers Bullous PG Arms and face usually; painful bulla progress to ulceration Associated with hematological conditions; leukemia Vegetative PG Less aggressive, but chronic; responds to local tx

    7. Differential Diagnosis Bacterial Infections (including mycobacteria) Fungal Infections Chronic ulcerative HSV Vasculitis Insect reaction (i.e. brown recluse spider bite) Malignancy – sqamous cell, cutaneous lymphoma Antiphospholipid syndrome Systemic Conditions – SLE, RA, Behcet’s, Wegener’s

    8. Diagnosis Diagnosis of Exclusion Diagnosis based on clinical presentation and course Biopsy may help with diagnosis BUT NO findings on pathologic specimens or laboratory evaluation that are pathognomic for PG Biopsy helps to EXCLUDE malignancy, infections, cutaneous vasculitis Up to 10% of patients diagnosed with PG are misdiagnosed

    9. Diagnosis Case Series (18 patients) – Diagnostic Criteria Clinical features of PG Histopathological examination consistent with PG Exclusion of other dermatoses that may mimic PG Laboratory evaluation – CBC, chemistries, syphilis serologies, immunoelectropheresis, ANA, RF, hepatitis serologies, HIV serology, antiphospholipid antibodies, ANCA Other investigations – CXR, BM biopsy, additional imaging

    10. Associated Conditions 50-70% of patients have an associated systemic disease Onset of PG may precede, coincide, or follow that of systemic disease Inflammatory Bowel Disease 30% of patients with PG have Crohn’s or UC 2% of patients with IBD will have PG RA (25% patients) Malignancy Other rheumatological and hematologic diseases Hereditary hypoglobulinemia HIV Sarcoidosis

    11. Treatment Goals Suppression of inflammatory disease activity Promotion of wound healing Treatment of underlying associated conditions Control of pain Systemic Corticosteroids 60-80mg prednisone daily (up to 100-120mg) Cyclosporin Initial response – usually rapid; progression stops Resolution – very slow resolution of existing lesions

    12. Treatment Infliximab (anti-TNFalpha monoclonal Ab) PG associated with inflammatory bowel disease IVIG – effective in certain case reports Others Thalidomide Cellcept Tacrolimus Dapsone Azathioprine

    13. Clinical Outcomes Case Series (18 patients) Complete remission in 83%; mean duration to remission 1.3yr Persistent disease in 3/18; mean duration 8yr Severity of systemic disease – prognostic value in course of PG Successful tx of associated condition leads to improvement or remission of PG

    14. Tips Can occur on any skin surface Consider PG when any ulcer or surgical wound fails to heal Develops rapidly – small lesions progresses to crater in 24-48 hrs Lesions usually very painful Often presents with systemic illness (fever)

    15. References Bhat RM “Management of pyoderma gangrenosum – An update” Indian J Dermatol Venereol Leprol (2004) 70: 329-335. Brooklyn T, Dunnill G, Probert C “Diagnosis and treatment of pyoderma gangrenosum” BMJ (2006) 333: 181-184. Hasselmann DO, Bens G, Tilgen W, Reichrath J “Pyoderma gangrenosum: Clinical presentation and outcome in 18 cases and review of the literature” JDDG (2007) 5: 560-564. Moschella SL “Neutrophilic dermatoses” Up-To-Date.

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