Pyoderma Gangrenosum  

Background: Pyoderma Gangrenosum (PG) is an uncommon ulcerative skin condition and it is not clear what causes it. PG was not defined until 1930 and so there is still not much information or knowledge about it as it is very rare, occurring in about 1 in 100,000 people each year. Being a systemic disease in about 50% of patients it effects the whole body. It is hard to diagnosis and is made by excluding other causes of similar skin disease. The 2 primary variants of PG are the classic ulcerative form, usually observed on the legs, and a more superficial variant known as atypical PG that tends to occur on the hands. Patients with PG may it find it causing other organs including the heart, the central nervous system, the GI tract, the eyes, the liver, the spleen, bones, and lymph nodes. Generally PG responds well to treatment; however, recurrences may occur and residual scarring is common. Therapy of PG involves the use of anti-inflammatory agents, such as steroids, and immunosuppressive medications.

The cause is not really understood but changes to the immune system seem to be at the root of the cause. Death from PG is rare, the main complaint is severe pain and it can cause problems with healing where other breaks in the skin occur such as cuts or surgery. PG mainly occurs in the fourth and fifth decades of life, but can effect all ages.

Clinical

History:

• Patients with PG usually describe the initial lesion as a bite reaction, with a small, red papule or pustule that changes into a larger ulcerative lesion. 
• Pain is the predominant historical complaint especially in the joints.

• A complete history should be taken with special focus on the organ systems such as inflammatory bowel disease, ulcerative colitis, Crohn disease, and blood disorders, such as leukemia. Less common associated diseases include other forms of arthritis, hepatitis and immunologic diseases, such as lupus and Sjögren syndrome.

Physical:

• Two main variants of PG exist: classic and atypical. Several other variants may exist.
  

• Classic PG is characterized by a deep ulceration with a violet border that overhangs the ulcer bed, generally on the legs, but they may occur anywhere on the body.

• Atypical PG has a "juicy" wound. This is usually only at the border of small ulcerations that most often occur on the surface of the hands and forearms, or the face.

• Classic PG may occur around stoma sites; this type is known as peristomal PG and is often mistaken for a wound infection.

• PG may occur on the genitalia; this form is termed vulvar or penile PG but is different from other sexually transmitted diseases.

Workup

A skin biopsy should be performed in almost all instances because it is useful in the exclusion of other diseases. This will not confirm PG as there is no medical test currently known, but will rule out other similar causes.

Treatment

Medical Care: No specific therapy is uniformly effective for patients with PG. Generally medication such as Steroids and immunosuppressants are effective. In patients with an associated underlying disease, the effective therapy of the associated condition may be associated with a control of the cutaneous process as well. Hyperbaric Oxygen Therapy can also be used.

Surgical Care: Surgery should be avoided, if possible, because of the high risk of it breaking out at the site of the surgery.

Activity: Patients should maintain their range of motion and perform all activities that they are able to tolerate.
   

Credit: http://www.emedicine.com/DERM/topic367.htm

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