| Pyoderma gangrenosum | |
|---|---|
| Classification and external resources | |
Pyoderma gangrenosum on the leg of a patient with Crohn's disease. |
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| ICD-10 | L88 |
| ICD-9 | 686.01 |
| eMedicine | article/1123821 |
| MeSH | D017511 |
Pyoderma gangrenosum is a disease that causes tissue to become necrotic, causing deep ulcers that usually occur on the legs. When they occur, they can lead to chronic wounds. Ulcers usually initially look like small bug bites or papules, and they progress to larger ulcers. Though the wounds rarely lead to death, they can cause pain and scarring.
The disease was identified in 1930. It affects approximately 1 person in 100,000 in the population. Though it can affect people of any age, it mostly affects people in their 40s and 50s.[1]
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There are two main types of pyoderma gangrenosum:[1]
Other variations are[2]:
Though the etiology is not well understood, the disease is thought to be due to immune system dysfunction, and particularly improper functioning of neutrophils. At least half of all pyoderma gangrenosum patients also suffer from illnesses that affect their systemic function.[1] For instance, ulcerative colitis, rheumatoid arthritis, and multiple myeloma (MM) sufferers have the condition. It can also be part of a syndrome, for instance in PAPA syndrome. Major and minor trauma are also believed to play a role. [5]
The common conditions associated with pyoderma gangrenosum are:
First-line therapy for disseminated or localized instances of pyoderma gangrenosum is systemic treatment by corticosteroids and cyclosporine. If ineffective, alternative therapeutic procedures include systemic treatment with corticosteroids and mycophenolate mofetil; mycophenolate mofetil and cyclosporine; tacrolimus; thalidomide; infliximab; or plasmapheresis.[7]
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