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Pyoderma Gangrenosum: Case Presentation and Practical Review

HISTORY. 36 Y/O FEMALE2-DAY R ANKLE PAIN PAPULE?BULLAEFEVERWORSENING PAINROS: N/V, DRAINING ULCER. Past Medical and Surgical History. 13 YEAR HX CROHN'S DZ, WELL CONTROLLEDONSET HERALDED BY PYODERMA GANGRENOSUMCHARACTERIZED BY: LOWER ABDOMEN PAIN LOWER EXTREMITIES PAIN AND SKIN LESIONSMED

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Pyoderma Gangrenosum: Case Presentation and Practical Review

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    1. Pyoderma Gangrenosum: Case Presentation and Practical Review Frank Don, DO Family Medicine Resident Westchester General Hospital and Clinics Miami, Florida

    2. HISTORY 36 Y/O FEMALE 2-DAY R ANKLE PAIN PAPULE?BULLAE FEVER WORSENING PAIN ROS: +N/V, DRAINING ULCER

    3. Past Medical and Surgical History 13 YEAR HX CROHNS DZ, WELL CONTROLLED ONSET HERALDED BY PYODERMA GANGRENOSUM CHARACTERIZED BY: LOWER ABDOMEN PAIN LOWER EXTREMITIES PAIN AND SKIN LESIONS MEDS: EMLA CRM, SULFASALAZINE CRM PRN SX: CHOLECYSTECTOMY, APPENDECTOMY, B/L SALPINGOOPHERECTOMY

    4. PHYSICAL EXAMINATION SKIN: PRETIBIAL HEMORRHAGIC BULLAE WITH SURROUNDING RING OF DARK ERYTHEMA AND YELLOWISH CENTRAL AREA SUGGESTIVE OF PUS MULTIPLE AREAS OF HYPOPIGMENTED ATROPHIC CRIBIFORM SCARS

    5. DIAGNOSIS PYOGENIC GANGRENOSUM New lesion Older, healed lesions

    6. HYPOPIGMENTED ATROPHIC CRIBIFORM SCARS

    7. MANAGEMENT SOLUMEDEROL 20 MG IV Q12 HR LEVAQUIN 500 MG IV QDAY DILAUDID 1 MG IV Q 3-4 HR PAIN DERM CONSULT PENDING

    8. Pyoderma Gangrenosum Fresh lesion Blister Dark erythema in the edges Central area of yellowish necrotic material Extremely tender to touch

    9. PYODERMA GANGRENOSUM: CLINICAL PAINFUL PRETIBIAL LOCATION M/C TENDER PAPULOPUSTULE W SURROUNDING ERYTHEMA?NECROSIS?ULCER?IRREGULAR, UNDERMINED-OVERHANGING GUNMETAL-COLORED BORDER, EXTENDS CENTRIFUGALLY?RE-EPITHELIALIZATION, ULCER HEALS WITH SCAR SINGLE-MULTIPLE, EXPAND, COALESCE

    10. PYODERMA GANGRENOSUM: CLINICAL FOUR MAJOR CLINICAL FORMS: ULCERATIVE, BULLOUS, PUSTULAR, SUPERFICIAL GRANULOMATOUS INITIAL LESION OFTEN A PUSTULE ON ERYTHEMATOUS/VIOLACEOUS BASE, ERYTHEMATOUS NODULE OR BULLA CHARACTERISTIC SUBSEQUENT LESION IS AN ULCER WITH NECROTIC UNDERMINED BORDER

    11. PYODERMA GANGRENOSUM CLINICAL FEATURES UNCOMMON, CHRONIC, RECURRENT NEITHER INFECTIOUS OR GANGRENOUS WOMEN 20s-50s, 50% OF APTIENTS HAVE AN UNDERLYING DISEASE

    12. DISEASES ASSOCIATED WITH PYODERMA GANGRENOSUM 50-70% INFLAMMATORY BOWEL DISEASE(UC, CROHN DZ) INFLAMMATORY ARTHRITIS (SERONEGATIVE ARTHRITIS) HEMATOLOGIC MALIGNANCY (AML, CML, HAIRY CELL LEUKEMIA, MONOCLONNAL GAMOPATHY) PAPA SYNDROME: PYOGENIC STERILE ARTHRITIS, PG, ACNE (PAPA SYNDROME)

    13. PYODERMA GANGRENOSUM: PATHOGENESIS IMMUNOLOGIC ABNORMALITY PATHERGY: INITIATION AND AGGRAVATION EARLY LESIONS: NEUTROPHILIC INFILTRATE ULCER: MARKED TISSUE NECROSIS WITH SURROUNDING MONONUCLEAR CELL INFILTRATES

    14. PYODERMA GANGRENOSUM Early lesion

    15. PYODERMA GANGRENOSUM Ulcerative lesion

    16. PYODERMA GANGRENSOSUM Late sclerosis

    17. EVALUATION OF PATIENT WITH SIGNS SUGGESTIVE OF PYODERMA GANGRENOSUM: DETAILED HISTORY AND PHYSCIAL EXAMINATION STERILE SKIN BIOPSY WITH SUFFICIENT DEPTH (PANNICULITIS) GI STUDIES: STOOL OCCULT BLOOD, COLONOSCOPY, LFT HEMATOLOGIC STUDIES: CBC, PERIPHERAL BLOOD SMEAR, BONE MARROW EXAMINATION. IF INDICATED CONSIDER FLOW CYTOMETRY SEROLOGIC STUDIES: SPEP, ANA, ANCA, ANTIPHOSPHOLIPID AB, VDRL CXR, UA

    18. PYODERMA GANGRENOSUM: TREATMENT GOAL: REDUCE INFLAMMATION (LOCAL OR COMBINED CORTICOSTEROIDS) EARLY LESION: LOCAL THERAPY, TACROLIMUS BID OR TRIAMCINOLONE SC EDGE LESION AVOID DEBRIDEMENT! PATHERGY!!!!!!!!!!!!! REGIMEN: PREDNISONE 1G QD X3-5 D, ASEESS AT ONE WEEK?NO CHANGE?SECOND AGENT CYCLOSPORINE 10MG/KG/DAY OR TACROLIMUS PO REFRACTORY: PULSE SYSTEMIC STEROIDS UP TO 1 G (MONTHLY), INFLIXIMAB 5MG/KG (Q 3 WEEKS) AZATHIOPRINE 100-300 MG QD (STEROID-SPARING)

    19. References Dermatology, Bolognia, Jorizzo, Rapini Derm101.com Ferkolj, Infliximab for treatment of resistant pyoderma gangrenosum associated with Crohns Disease, Acta Dermatoven Vol 15, p 173-177

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