Pyoderma gangrenosum


Pyoderma gangrenosum

Description, Causes and Risk Factors:

Abbreviation: PG.

ICD-10: L88.

A chronic, noninfective eruption of spreading, undermined ulcers showing central healing, with diffuse dermal neutrophil infiltration; often associated with ulcerative colitis.

Pyoderma gangrenosum (PG) is a distinctive, rare, destructive inflammatory skin disease of unknown etiology. In about half of the cases pyoderma gangrenosum is associated with systemic diseases like inflammatory bowel disease, polyarthritis, hematologic disorders (such as leukaemia or preleukemic states), psoriatic arthritis, spondyloarthropathy, hepatitis, primary billiary cirrhosis, and immunologic diseases (such as lupus erythematosus and Sjögren's syndrome).

Generally pyoderma gangrenosum is most common on the lower extremities and the trunk. Although predominantly a condition seen in adult life, occasionally it can be seen in childhood and may be seen on the buttocks, perineum, and the head and neck.

Types:

    Pustular PG: Pustular PG develops as small painful pustules on otherwise healthy skin during acute exacerbations of in?ammatory bowel disorders.

  • Ulcerative PG: The ulcerative form of PG is the easiest to recognize and is the one most commonly associated with this diagnosis. Painful ulcers evolve from one or more tender papules or pustules, frequently around hair follicles. These characteristically have well demarcated purple (sometimes described as violaceous) undermined borders and surrounding erythema. Ulcers may be singular or multiple and occur anywhere on the body (although most commonly on the trunk and lower limbs).

  • Bullous PG: Bullous PG arises from rapidly developing hemorrhagic blisters mainly located on the arms in patients with myloproliferative disorders.

  • Vegetative PG: The vegetative form of the condition is the least typical. This presents as a non-painful super?cial ulcer generally without the classic purple edge. It is often solitary and progresses slowly. It is not usually associated with any systemic conditions.

Causes and Risk factors:

It's not certain what causes pyoderma gangrenosum, but it may be related to a disorder of the immune system. About half the people with pyoderma gangrenosum have an underlying chronic health condition. However, skin trauma, such as a cut or puncture wound, can result in new ulcers forming in people that have this skin disorder. Pyoderma gangrenosum is most common in people in their 40s and 50s but can occur at any age. It's also associated with the following conditions:

    Ulcerative colitis: This disease of the large intestine causes chronic inflammation of your large intestine.

  • Rheumatoid arthritis: Rheumatoid arthritis refers to a condition in which the thin membranes surrounding your joints become irritated and inflamed.

  • Crohn's disease: Like ulcerative colitis, Crohn's disease causes long-term inflammation in the intestine but can occur anywhere along your digestive tract.

  • Hepatitis: This condition causes your liver to become inflamed. The cause of hepatitis may be a virus or a disorder of your immune system.

Symptoms:

The first sign of pyoderma gangrenosum is one or more small, red bumps on your skin that resemble spider bites. Over time, the bumps expand to form painful, open sores with reddish-purple borders. The ulcers usually appear on your legs but may develop anywhere on your body. Other symptoms you might experience include achy joints or pain in your bones and tiredness.

Diagnosis:

Differential diagnoses may include:

    Sweet's syndrome (although thisrarely presents with perifollicularin?ammation and there is nodestruction of the capillary bed).

  • Behçet's disease.

  • Rheumatoid vasculitis.

  • Hepatic folliculitis.

  • Necrotizing cutaneous infections suchas necrotizing fasciitis.

  • Cutaneous anthrax.

  • Pustular drug reactions.

The sudden appearance of unexplainable ulcers on your skin is the first indication that you may have pyoderma gangrenosum. In order to rule out a bacterial infection or some other cause, your doctor may examine a culture sample from your skin or a sample of your skin under a microscope (biopsy).

You'll likely also have a complete physical exam, including blood tests, to determine whether you have an underlying health condition that's associated with pyoderma gangrenosum.

Blood tests may include:FBC (full blood count), inflammatory markers, LFTs, urine protein and rheumatological investigations may be appropriate to look for associated diseases (as under 'Aetiology' above).Autoantibodies - patients with PG are often p-ANCA (perinuclear) positive, particularly if inflammatory bowel disease is present. The presence of c-ANCA (cytoplasmic) may indicate Wegener's granulomatosis.

Treatment:

There have been a number of treatment regimes indicated for the management of pyoderma gangrenosum. The choice of therapy depends on the severity of the presenting symptoms and the presence or absence of underlying systemic disease. Where the condition is found in association with systemic disease, the ?rst priority should be the control of this condition

Where lesions are mild and there is an absence of systemic disease it may be possible to control the condition with topical preparations such as topical corticosteroids and local dressings. Topical tacrolimus, a drug licensed for the management of atopic eczema, has recently been shown to be effective in the management of early pyoderma gangrenosum lesions.

As your skin heals, you will likely taper off the corticosteroids or immunosuppressants. You can expect your skin to recover several months after beginning therapy. Without treatment, the ulcers may widen, remain the same or slowly heal.

Where the condition is more widespread or aggressive in nature, a systemic approach is favored. High-dose oral corticosteroids have been the mainstay of treatment for a number of years. Sulphones and other antileprotics and antimicrobials have also been found to be useful. It is believed that these drugs act predominantly as antiin?ammatory mediators, systemic immunosuppressant drugs such as cyclosporin have been used, however, these are not without risk. Secondary infection can occur and the immunocompromised individual may be at risk of catastrophic sepsis. The recent development of biological TNF-? inhibitors such as in?iximab and etanercept normally used in the management of severe rheumatoid disease and psoriatic arthritis does offer new options for pyoderma gangrenosum sufferers who fail to respond to other therapies.

In some cases, debridement has been suggested, however, in view of the potential role of pathergy in the development and acceleration of this condition, this option does not appear appropriate.

Surgery: Doctors do not commonly use surgery as a treatment option because trauma to the skin may worsen existing ulcers or stimulate new ones to develop. If the ulcers on your skin are large and need help with healing, your doctor may surgically attach a piece of real or artificial skin over the open sores. Doctors perform surgery only once all of the inflammation has improved.

NOTE: The above information is educational purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition.

DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.

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