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Case Note

Case Note: Painful Ulcerations on the Legs

History

A 37-year-old, thin, ill-appearing male, with a past medical history notable for inflammatory bowel disease (IBD), presents for management of extremely painful leg ulcers on bilateral ankles, which have developed rapidly over the past 2 weeks without inciting trauma.

Initial evaluation

  • Past medical history of IBD, for which patient takes 6-mercaptopurine
  • Review of systems notable for 1-month history of weight loss, fatigue, and bloody diarrhea - consistent with patient's IDB flares
  • Presents for management of bilateral leg ulcers approximately 2x6 cm in size with a violaceous, undermined border and sieve-like ulceration within
    • Currently covering ulcers with bandages only
    • Reports rapid progression of the ulcers over the past 2 days
  • Image 1
    Image 1

Enter your diagnosis

Diagnosis

  • A diagnosis of pyoderma gangrenosum is rendered

Initial Treatment

  • Skin biopsy performed to exclude infectious or other inflammatory etiology
  • Tacrolimus ointment 0.1% applied daily under an occlusive hydrocolloid dressing is recommended
  • Concern for an underlying inflammatory condition or malignancy triggers diagnostic evaluation
    • Age-appropriate cancer screening, chest X-ray, and blood tests (complete blood count with differential, erythrocyte sedimentation rate, and rheumatologic serologies)
  • Patient is urgently referred to gastroenterology for colonoscopy to confirm suspected IBD flare
    • Given high suspicion of recurrent IBD flare, the patient is initiated on prednisone 1 mg/kg/day
  • Follow up in 1 week

Follow-up evaluation strategy

1-week follow-up evaluation:

  • Patient's ulcers have improved significantly, with marked reduction in ulcer pain
  • Blood tests indicate evidence of systemic inflammation and mild anemia; age-appropriate malignancy screening does not reveal an underlying malignancy
  • Ongoing wound care with tacrolimus ointment under occlusion is recommended
  • Follow up in 1 week

Subsequent 1-week follow-up evaluation:

  • Ongoing interval improvement
  • Patient's diarrhea and skin ulcerations are greatly improved
  • Patient's prednisone is tapered and tumor necrosis factor (TNF) cytokine blockade is initiated for management of both IBD and pyoderma gangrenosum
    • Subsequent clinical improvement

Further recommendations:

  • Vapor-permeable, moisture-retentive, hydrophilic membranes help control pain and may hasten re-epithelialization
  • Grafts of human skin equivalents or cultured keratinocytes are very effective in reducing ulceration pain and may shorten the course of immunosuppressive treatment required to heal the ulceration
  • Hyperbaric oxygen can accelerate healing rates in some patients
  • Treatment for an associated disease is mandatory, since control of pyoderma gangrenosum can often be achieved with therapy of the underlying disease process
  • Emollients should be employed routinely, and both elevation and support stockings should be prescribed to minimize venous pooling that can lead to leg ulcers

Subsequent treatment steps:

  • If the response to systemic steroids is inadequate and the process is extremely rapid, treat with cyclosporine A at an initial dose of 5-10 mg/kg/day (in addition to systemic steroids) with close monitoring for side effects
    • Response should be dramatic and will permit rapid reduction of systemic steroids (as long as cyclosporine complications are not encountered)
  • For superficial lesions with limited progression, consider minocycline 100-200 mg/day; dapsone 100-200 mg/day (monitoring for anemia); colchicine 0.6-1.2 mg twice daily, to tolerance (diarrhea limits dosing); clofazamine 100 mg twice daily; or thalidomide 100-400 mg daily for 2-4 weeks (tapered if the patient responds)
  • In refractory cases, the following agents can be used individually or added to treatment with systemic steroids and cyclosporine:
    • Monthly pulse systemic steroids (with intravenous [IV] methylprednisolone up to 1 g or oral dexamethasone 100 mg daily for 5 consecutive days) may be added to the cyclosporine and daily systemic steroid doses
    • Mycophenolate mofetil 1-1.5 g twice daily may be added to the combination of systemic steroids and cyclosporine
    • Anti-TNF blockade
      • IV infliximab 5 mg/kg at Week 0, 2, and 6, followed by infusions every 4-6 weeks until improvement
      • Etanercept 50 mg twice weekly
    • Azathioprine 100-300 mg daily can be an effective steroid-sparing agent or adjunctive treatment given with systemic corticosteroids

General discussion

Pyoderma gangrenosum is a neutrophilic dermatosis defined by its characteristic morphology. Ulcerating lesions marked by a violaceous rim and undermined border may develop rapidly (in days) and may be triggered or exacerbated even by minimal trauma (pathergy). Pyoderma gangrenosum lesions may also be bullous, vegetative, pustular, or peristomal (i.e., adjacent to an intestinal stoma). It is a diagnosis of exclusion and can be simulated by various inflammatory, vascular and infectious conditions.

Pyoderma gangrenosum lesions may occur in isolation or in association with an underlying systemic disorder, such as a rheumatologic disease, IBD, malignancy (especially hematologic malignancy), immunodeficiency, or infection. Lesions may also be induced by medications; for example, exogenous granulocyte-monocyte colony stimulating factor, isotretinoin, and sunitinib may cause pyoderma gangrenosum-like lesions. In these cases, lesions may respond by stopping the inciting medication and topical treatment. Rare familial forms have also been described (the syndrome of pyoderma gangrenosum, acne, and pyogenic arthritis [PAPA syndrome]). The diagnostic evaluation of pyoderma gangrenosum thus requires formal exclusion of conditions that may mimic this disorder in addition to diagnostic work-up for associated systemic diseases.

Treatment is determined by the extent and rapidity of the cutaneous process and requires both aggressive management of inflammation and optimal wound care. Though treatment of early lesions can often be managed through topical medications and good wound care, advanced or rapidly expanding lesions may require systemic immunosuppression to halt progression and promote healing. Pyoderma gangrenosum lesions may be exquisitely painful, sometimes out of proportion to their clinical appearance or size; reduction in pain associated with a lesion represents an important early sign of effective treatment. Lesions often heal with a very characteristic cribiform, atrophic scar pattern.

Further reading

Ahronowitz I, Harp J, Shinkai K. Etiology and management of pyoderma gangrenosum: A comprehensive review. Am J Clin Derm 2012;13:191-211.

Fox L. Pyoderma Gangrenosum (PG). Available at: https://www.dermquest.com/expert-opinions/clinical-updates/2012/pyoderma-gangrenosum-(pg)/ (accessed October 2014).

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