Pyoderma Gangrenosum: Case Presentation and Practical Review

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Pyoderma GangrenosumCase Presentation and
Practical Review

• Frank Don, DO
• Family Medicine Resident
• Westchester General Hospital and Clinics
• Miami, Florida

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HISTORY

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

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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

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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

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DIAGNOSIS

• PYOGENIC GANGRENOSUM
• New lesion
• Older, healed lesions

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HYPOPIGMENTED ATROPHIC CRIBIFORM SCARS
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MANAGEMENT

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

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Pyoderma Gangrenosum

• Fresh lesion
• Blister
• Dark erythema in the edges
• Central area of yellowish necrotic material
• Extremely tender to touch

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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

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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

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PYODERMA GANGRENOSUM

• CLINICAL FEATURES
• UNCOMMON, CHRONIC, RECURRENT
• NEITHER INFECTIOUS OR GANGRENOUS
• WOMEN 20s-50s,
• 50 OF APTIENTS HAVE AN UNDERLYING DISEASE

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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)

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PYODERMA GANGRENOSUM PATHOGENESIS

• IMMUNOLOGIC ABNORMALITY
• PATHERGY INITIATION AND AGGRAVATION
• EARLY LESIONS NEUTROPHILIC INFILTRATE
• ULCER MARKED TISSUE NECROSIS WITH SURROUNDING
  MONONUCLEAR CELL INFILTRATES

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PYODERMA GANGRENOSUM

• Early lesion

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PYODERMA GANGRENOSUM

• Ulcerative
• lesion

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PYODERMA GANGRENSOSUM

• Late sclerosis

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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

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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)

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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