Saturday, November 15, 2008

Pyoderma Gangrenosum

Abstract: 46 yo man with 1.5 year history of leg ulcers
History: The patient, a disabled 46 yo Cambodian man, has a four year history of poorly controlled ulcerative colitis. He has had painful leg ulcers for the past two years. These begin with pustules or vesicles by history. At present he is taking 1200 mg of Asacol t.i.d. and prednisone 30 mg. per day. In addition to the prednisone he has used potent topical steroids for his ulcers and has been treated at a wound care clinic.
Social History: The patient emigrated from Cambodia 25 years ago. He is married with three children and was employed until he became disabled 2 years ago from colitis and leg ulcers. His English is limited and I had no Cambodian translator.
O/E: There are two ulcers with raised overhanging borders on the left medial malleolus. In addition, there is post-inflammatory hyperpigmentation and proximal scarring secondary to previous ulcerations. The patient has Cushingoid facies.
Clinical Photos:



Lab: N/A
Histopath: N/A
Diagnosis: Pyoderma gangrenosum (P.g.)
Discussion: There is no effective therapeutic protocol for P.g. He has been treated with high dose prednisone for months and his P.g. is only poorly controlled. Super-potent topical steroids have been used without improvement. It seems to us that tacrolimus ointment should be tried because there are many reports of its efficacy with P.g. and it is a more benign therapy than oral cysclsporin or mycophenolate mofetil. Colectomy may be a more permanent solution, but the patient and his gastroenterologists are not ready for that.
Questions: Your suggestions are welcome.
References:
1. eMedicine.com: P.G.

2. Reichrath J, Bens G, Bonowitz A, Tilgen W. Treatment recommendations for pyoderma gangrenosum: an evidence-based review of the literature based on more than 350 patients. J Am Acad Dermatol. 2005 Aug;53(2):273-83.

Dermatology Clinic, The Saarland University Hospital, Homburg/Saar, Germany. hajrei@uniklinik-saarland.de

Because the incidence of pyoderma gangrenosum (PG) is low, no prospective randomized controlled trials and only a few studies with case numbers of more than 15 patients have been published. To date no guidelines for treatment of PG have been established far. The aim of the study was to provide an evidence-based review of the literature and an evaluation of recommendations for PG treatment. We performed an electronic search using the PubMed database and the term "pyoderma- gangrenosum." Literature published in the English language during the past two decades was reviewed. All relevant studies that could be obtained regardless of the study design were evaluated for grades of recommendation and levels of evidence. Data on patient characteristics including severity of the disease, localization of lesions, associated diseases, and treatment procedures were abstracted and evaluated for therapeutic outcome. We conclude that therapeutic efficacy of systemic treatment with corticosteroids and cyclosporine is best documented in the literature for disseminated as well as for localized disease and should be considered first-line therapy. In cases that do not respond to this treatment, we recommend alternative therapeutic procedures (eg, systemic treatment with corticosteroids and mycophenolate mofetil; mycophenolate mofetil and cyclosporine; tacrolimus; infliximab; or plasmapheresis), considering additional factors including associated diseases.

10 comments:

  1. Ashok Kumar SharmaNovember 17, 2008

    Given the limitations mentioned about prescribing costly medications for this patient, may I just mention that I have had good results with perilesional Triamcinolone acetonide injections repeated fortnightly till the ulcers heal.

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  2. While cyclosporine, infliximab and etanercept work well for PG, less expensive but effective options are azathioprine and dapsone. These have worked for me.

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  3. After excluding other significant causes of chronic leg ulcers (such as arterial/venous insufficiency, infections, diabetes, Collagen Vascular diseases) and its strong clincial association with poorly controlled ulcerative collitis for almost 2 yrs PG is the diagnosis.
    I feel along with the treatment for PG with topical super potent steroids and UC with mesalazine & oral steroids, we must also concentrate on the other possible means of gaining much better control of his collitis. Since immunomodulators have never been used in this patient, i think it would be a better idea to give a trail of immunomodulators both topically and (if possible) orally.

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  4. The ulcer is quite characteristic of pyoderma gangrenosum though PG is essentially a diagnosis of exclusion.

    Once the ulcer is healing with oral prednisolone +/- cyclosporin and reduced to the lowest dose possible to minimise side effects, topical tacolimus would be useful topical agent to heal the ulcer. There is a recent report by Azizan NZ who treated four cases of PG successfully with topical cyclosporin.

    Ref: Azizan NZ, Gangaram HB, Hussein SH. A novel therapy for the treatment of pyoderma gangrenosum. Med J Malaysia. 2008 Mar;63(1): 51-4

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  5. Oral Minocycline may also be tried as an alternative - has been successfully used in some refractory patients. Thanks for posting this.

    Best,

    Aisha Sethi
    University of Chicago

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  6. azathioprine 3 mg /kg body weight tapered over 2- 3 months may do wonders. I have used in at least five patients
    dr Manish Pahwa
    MD DNB New Delhi, India

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  7. Agree with attempts to treat underlying UC. Would consider infliximab or old school use of clofazamine. I have similar patient who has failed intralesional kenalog + mycophenolate Mofet and azathioprine. Our case was limited by chronic renal insufficiency and thus cyclosporine was not an option

    Lisa Scatena, MD
    Boulder CO

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  8. Hi doctors,

    My little brother who is 12 is recently diagnosed with P.g. in Hong Kong. Ulcers and wounds were found on both of this ankle. He has done blood test and a biopsy of the tissue. The result were negative to autoimmune markers: ANF, anti-dsDNA, anti-ENA, ANCA, and Cryoglobulin. He was treated on April 09 with 17.5 mg / day of prednsiolone. We saw an improvement of his ulcers and the doc. then lowered his dose to 12.5 mg / day. However, we found new blisters on his feet now in June and the doc. raise the dose of prednisolone to 20 mg/day at the end of last week. Can anyone give me any possible consultation to this? any other possible treatment for a children? Any doctors here have treated a child with P.g. before?

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    ReplyDelete
  10. AnonymousJuly 01, 2010

    GReat post

    ReplyDelete

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