Pyoderma gangrenosum

Published on 18/03/2015 by admin

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Last modified 22/04/2025

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[level-membership-for-dermatology-category]

Pyoderma gangrenosum

John Berth-Jones

Evidence Levels:  A Double-blind study  B Clinical trial ≥ 20 subjects  C Clinical trial < 20 subjects  D Series ≥ 5 subjects  E Anecdotal case reports

image

Pyoderma gangrenosum (PG) is a clinically diagnosed entity presenting as pustules which enlarge, forming ulcers with a dark, necrotic, undermined margin. Any skin site may be affected. Although PG is usually self-limiting there is a danger of disfiguring scarring.

Management strategy

No treatment for PG is always effective. The most consistent results are reported with systemic corticosteroids and cyclosporine, and these effective but potentially toxic modalities can be employed when the severity of the disease justifies the risks (e.g., when there is facial involvement or rapid progression). Infliximab has been shown to be beneficial in a controlled trial. The evidence regarding other modalities is less conclusive. PG tends to resolve spontaneously, so some of the reports of therapeutic success may simply be the result of the disease following its natural course.

In cases where scarring is not a major concern, consideration should be given to conservative treatment with wound dressing only, as most lesions resolve spontaneously. When required, relatively non-toxic first-line treatments should be tried first and second-line modalities employed if there is no response. Topical tacrolimus offers an interesting novel approach to first-line therapy. A wide variety of potentially hazardous and expensive treatments are employed occasionally, and these third-line modalities should be considered only when others are ineffective.

The many recognized associations with PG include rheumatoid disease, inflammatory bowel disease, myeloproliferative disease such as acute myeloblastic leukemia, plasma cell dyscrasias, and Wegener’s granulomatosis. These may warrant investigation, and may also influence the choice of treatment. Effective management of an underlying pathology such as ulcerative colitis often seems to result in improvement of the PG.

PG may demonstrate the Koebner phenomenon; care should be taken to avoid trauma to the skin. When surgery is unavoidable in patients with a history of PG, the surgeon should be made aware of this risk. Surgical incisions should be kept as short as possible. Careful wound closure may be helpful. Prophylactic systemic corticoids or cyclosporine may be indicated perioperatively.

First-line therapies

image Topical tacrolimus C
image Topical corticoids D
image Dapsone D
image Intralesional corticoids D
image Minocycline D
image Nicotine D
image Topical pimecrolimus E
image Sodium cromoglycate D
image Sulfasalazine D

Second-line therapies

image Cyclosporine C
image Systemic corticoids C

Third-line therapies

image Infliximab A
image Other TNF&#x03B1;-antagonists C
image Alefacept E
image Alkylating agents (cyclophosphamide, chlorambucil) D
image Plasmapheresis (plasma exchange) E
image Leukocytapheresis D
image IVIG C
image Intralesional cyclosporine E
image Tacrolimus (FK506) D
image Azathioprine or mercaptopurine D
image Colchicine D
image Thalidomide D
image Potassium iodide E
image Topical nitrogen mustard (mechlorethamine) E
image Mycophenolate mofetil D
image GM-CSF E
image Methotrexate E
image Topical platelet-derived growth factor E
image Recombinant human epidermal growth factor E
image Clofazimine D
image Hyperbaric oxygen D
image Isotretinoin E
image Anakinra E
image Ustekinumab E
image Imiquimod E
image Visilizumab E
image Topical phenytoin D
image Surgical repair by graft or flap E

[/level-membership-for-dermatology-category][not-level-membership-for-dermatology-category]

Pyoderma gangrenosum

John Berth-Jones

Evidence Levels:  A Double-blind study  B Clinical trial ≥ 20 subjects  C Clinical trial < 20 subjects  D Series ≥ 5 subjects  E Anecdotal case reports

image

Pyoderma gangrenosum (PG) is a clinically diagnosed entity presenting as pustules which enlarge, forming ulcers with a dark, necrotic, undermined margin. Any skin site may be affected. Although PG is usually self-limiting there is a danger of disfiguring scarring.

Management strategy

No treatment for PG is always effective. The most consistent results are reported with systemic corticosteroids and cyclosporine, and these effective but potentially toxic modalities can be employed when the severity of the disease justifies the risks (e.g., when there is facial involvement or rapid progression). Infliximab has been shown to be beneficial in a controlled trial. The evidence regarding other modalities is less conclusive. PG tends to resolve spontaneously, so some of the reports of therapeutic success may simply be the result of the disease following its natural course.

In cases where scarring is not a major concern, consideration should be given to conservative treatment with wound dressing only, as most lesions resolve spontaneously. When required, relatively non-toxic first-line treatments should be tried first and second-line modalities employed if there is no response. Topical tacrolimus offers an interesting novel approach to first-line therapy. A wide variety of potentially hazardous and expensive treatments are employed occasionally, and these third-line modalities should be considered only when others are ineffective.

The many recognized associations with PG include rheumatoid disease, inflammatory bowel disease, myeloproliferative disease such as acute myeloblastic leukemia, plasma cell dyscrasias, and Wegener’s granulomatosis. These may warrant investigation, and may also influence the choice of treatment. Effective management of an underlying pathology such as ulcerative colitis often seems to result in improvement of the PG.

PG may demonstrate the Koebner phenomenon; care should be taken to avoid trauma to the skin. When surgery is unavoidable in patients with a history of PG, the surgeon should be made aware of this risk. Surgical incisions should be kept as short as possible. Careful wound closure may be helpful. Prophylactic systemic corticoids or cyclosporine may be indicated perioperatively.

First-line therapies

image Topical tacrolimus C
image Topical corticoids D
image Dapsone D
image Intralesional corticoids D
image Minocycline D
image Nicotine D
image Topical pimecrolimus E
image Sodium cromoglycate D
image Sulfasalazine D