How Do You Treat Pyoderma Gangrenosum?

Pyoderma gangrenosum is a rare autoimmune condition that causes skin ulcers, often in older adults who have other autoimmune conditions.

Pyoderma gangrenosum is a rare condition that causes painful ulcers on the skin. It most commonly occurs in people from ages 40 to 60, but it can happen at any age. About half of all patients also have other systemic conditions like inflammatory bowel disease (IBD) such as Crohn’s disease and ulcerative colitis, blood diseases and blood cancers, rheumatoid arthritis, and ankylosing spondylitis.

Symptoms of pyoderma gangrenosum include a raised bump or blister. Characteristics of the bump or blister include:

• Initial lesion may appear as a bug bite reaction 
• Bump or blister may be filled with fluid or pus
• The bump or blister may spread out and form an open sore 
• Edges may appear purple
• Blisters and sores are painful
• Bumps, blisters, and sores most commonly appear on the legs, but may also appear on other parts of the body
• Lesions may also appear around surgical opening (stoma) sites
• Pyoderma gangrenosum may rarely occur inside the body

Other symptoms of pyoderma gangrenosum may include:

• Joint pain
• Feeling unwell (malaise)

The cause of pyoderma gangrenosum is not well understood, but it is believed to be due to an imbalance of the immune system (specifically, altered neutrophil chemotaxis).

Risk factors for developing pyoderma gangrenosum include certain systemic conditions such as:

• Inflammatory bowel disease (IBD) such as Crohn’s disease and ulcerative colitis
• Blood diseases and blood cancers
• Rheumatoid arthritis
• Ankylosing spondylitis

The diagnosis of pyoderma gangrenosum is made by exclusion, which means other conditions are first ruled out.

Tests used to rule out other conditions and to aid in diagnosis of pyoderma gangrenosum include:

• Complete blood count (CBC)
• Comprehensive chemistry profile, including a liver function test
• Urinalysis
• Hepatitis profile 
• Blood and/or urine protein electrophoresis, peripheral smear, and bone marrow aspiration or biopsy may be used to evaluate for blood cancers 
• Venereal Disease Research Laboratory (VDRL) test
• Antineutrophil cytoplasmic antibody test
• Partial thromboplastin time test
• Antiphospholipid antibody test
• Anti–Saccharomyces cerevisiae antibodies (IgG and IgA) 
• Perinuclear antineutrophil cytoplasmic antibodies (p-ANCA) 
• Fecal calprotectin 
• Serum immunofixation electrophoresis 
• Tissue cultures of the ulcer/erosion for bacteria, fungi, atypical mycobacteria, and viruses 
• Chest X-ray
• Angiography or Doppler to check for arterial or venous insufficiency
• Colonoscopy to exclude inflammatory bowel disease or ulcerative colitis 

There is no specific treatment that is effective for all patients with pyoderma gangrenosum. If patients have an associated, underlying disease, treatment for that condition may help relieve some pyoderma gangrenosum symptoms.

Topical therapies for pyoderma gangrenosum include:

• Gentle local wound care and dressings
• Super-potent topical corticosteroids
• Cromolyn sodium 2% solution
• Nitrogen mustard
• 5-aminosalicylic acid
• Topical immune modifiers: tacrolimus and pimecrolimus 

Systemic therapies for pyoderma gangrenosum include:

• Corticosteroids
• Cyclosporine
• Mycophenolate mofetil
• Azathioprine
• Dapsone
• Tacrolimus
• Cyclophosphamide
• Chlorambucil
• Thalidomide
• Tumor necrosis factor-alpha (TNF-alpha) inhibitors (e.g., thalidomide, etanercept, infliximab, adalimumab, clofazimine)
• Nicotine

Intravenous (IV) therapies for pyoderma gangrenosum include:

• Pulsed methylprednisolone
• Pulsed cyclophosphamide
• Infliximab
• IV immunoglobulin
• Ustekinumab

Other therapies for pyoderma gangrenosum include hyperbaric oxygen.

The outlook for pyoderma gangrenosum is generally good.

Complications of pyoderma gangrenosum include:

• Disease recurrence
• Residual scarring
• Pain that requires medication to control
• Infection