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RE: misspelled word - PYODERMA

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The

term pyoderma gangrenosum

is a misnomer. The condition is neither infectious nor

gangrenous; rather, it is an inflammatory process of unknown etiology, leading

to painful skin ulcers.

Pyoderma gangrenosum

primarily affects adults, typically those between 40 and 60 years old. It consists of recurring destructive ulcers that begin as

pustules and resolve as cribriform scars. Several clinical variants of PG have been identified,

including ulcerative, pustular, bullous,

vegetative, and peristomal types. The typical PG lesion has inflamed,

undermined borders and is found on the lower leg. Because

no diagnostic test for PG exists and a number of other conditions resemble PG,

diagnosis relies on the clinical presentation and exclusion of other causes. Pyoderma gangrenosum

is associated with other conditions in up to 75% of patients, and the type of

clinical presentation may suggest an underlying systemic disease. Associated conditions include inflammatory bowel disease,

arthritis (rheumatoid or seronegative), monoclonal gammopathy, and hematologic

malignancies. After PG is diagnosed, the underlying

condition or conditions should be determined.

Pathophysiology

The

mechanism by which PG lesions develop is unknown; however, it is believed that pathergy plays a role. Pathergy is the development of lesions in areas of trauma. In susceptible people, even minimal trauma or irritation

to the skin can result in the production of PG lesions. Debridement is one traumatic event that may result in PG

pustules or ulcers. They may also occur at skin graft

harvest sites and stoma placement sites or at surgical wounds or scars.

It has

been suggested that interleukin-8 (IL-8), a proinflammatory

cytokine, may play a role in the pathogenesis of PG.12 Interleukin-8 is chemotactic for neutrophils and

is not normally detected in human skin. However, IL-8

is overexpressed in certain chronic skin conditions,

including PG. One laboratory study showed that PG

ulcers developed after IL-8 was injected into human skin xenografts

that were grafted onto mice.

Diagnosis

No single

diagnostic test is available for PG. Pyoderma gangrenosum is a clinical diagnosis of exclusion. Histologic and laboratory

evaluation in patients with PG are based largely on excluding other causes and

evaluating a patient for underlying systemic disease. Pyoderma gangrenosum may resemble

both infectious and inflammatory conditions, such as vasculitis,

vasculopathy, mycobacterial

or bacterial infections, or collagen vascular diseases

Because

PG is associated with many systemic diseases and malignancies, including

inflammatory bowel disease, arthritis, and hematologic

malignancies, a careful search for associated conditions should be undertaken

when evaluating a patient; these conditions may impact treatment. This can be accomplished with a variety of blood, urine,

and radiographic tests, such as complete blood counts, renal and hepatic

function, sigmoid or colonoscopy, chest radiograph, and, if necessary, bone

marrow biopsy.

Treatment

There is

no specific treatment for PG, although corticosteroids are usually helpful. The goals for managing PG are pain control, wound healing,

and prevention of infection. The treatment should be

tailored to the patient's type of lesions, extent of disease, duration of

disease, severity of disease, lifestyle, other associated systemic diseases,

age, gender, prior treatments, and the practitioner's preference.

For

limited or mild disease, topical or intralesional

steroids may be used. For more

severe or widespread disease, a variety of systemic therapies can be used. Antibiotics with anti-inflammatory properties, such as minocycline or dapsone, are often

used as first-line therapies. Systemic steroids given

in an oral, intravenous, or pulsed fashion are generally effective, although

their adverse effects limit longterm use. A variety of immunosuppressive agents have been found

effective for treating PG, including cyclosporine, mycophenylate

mofetil, methotrexate, and chlorambucil. Thalidomide,

granulocyte-macrophage colony-stimulating factor, and nicotine patches

represent other therapeutic options.

In

summary, PG is an inflammatory process resulting in ulceration of unknown

etiology. It is associated with systemic disorders in

a significant percentage of patients and treatment is aimed at the underlying

inflammatory process.

HTH - Barb in Texas - Together in the Fight, Whatever it Takes!

Son Ken (33) UC 91 - PSC 99 Listed 7/21 @ Baylor Dallas

_._,___

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