Pyoderma gangrenosum is characterized by the formation of ulcers on the skin and the development of skin necrosis. In most cases, it is not a disease in its own right, but a symptom of another underlying disorder. In severe cases, entire sections of skin tissue die off.
What is pyoderma gangrenosum?
Pyoderma gangrenosum is characterized by the formation of ulcers and the death of entire areas of the skin. The ulcer is called an ulcer, and the widespread death of the skin is called gangrene. Pyoderma gangrenosum is not an infection but an autoimmune disease in which the skin is attacked by its own immune system. The white blood cells are activated. See topbbacolleges for Definitions of NTIS.
Only immunosuppressants such as glucocorticoids, cyclosporine A or dapsone can sufficiently weaken the immune system and contribute to remission of the symptoms. Pyoderma gangrenosum often occurs in the context of another underlying disease such as ulcerative colitis, Krohn’s disease, rheumatoid arthritis, vasculitis, chronic hepatitis or leukemia.
However, it can also develop after skin injuries or after operations from the surgical wound. The exact mechanism of formation is not yet known. Ulcerations usually develop very slowly. However, there are also cases associated with massive outbreaks.
Causes
Pyoderma gangrenosum is an autoimmune disease whose cause has not yet been fully clarified. It is often misinterpreted as a complication of an existing autoimmune disease. This is also shown by the associations of pyoderma gangrenosum with other autoimmune diseases.
Around ten percent of all patients with pyoderma gangrenosum suffer from ulcerative colitis or Krohn’s disease. In up to 50 percent of all cases, a connection with rheumatoid arthritis, vasculitis, chronic arthritis or leukemia can be found. A causal relationship with the metabolic syndrome is also suspected. Overall, it is assumed that pyoderma gangrenosum is not an isolated skin disease, but rather an expression of a skin reaction in a generalized systemic disease process on an autoimmunological basis.
Symptoms, Ailments & Signs
Pyoderma gangrenosum often begins with the formation of pustules and papules on the skin, which quickly enlarge and coalesce. After all, they later decay and form a painful ulcer. There is a central zone of necrosis in the center of the ulcer. A bluish-livid discoloration appears at the border area to the intact skin. No infections are found in the ulcer.
In most cases, the disease develops very slowly. However, there are also cases with rapid development, sometimes even necessitating amputation of the affected limb. 80 percent of the lower legs are affected. However, all other skin areas can also be affected by pyoderma gangrenosum.
If the affected areas become infected, severe disease progressions are to be expected. Very often other symptoms also appear. Depending on the underlying disease, severe digestive problems, rheumatic symptoms or chronic respiratory problems often occur.
Diagnosis & course of disease
For the most part, the diagnosis of pyoderma gangrenosum is based on the typical clinical presentations of the disease. Vasculitis (inflammation of the blood vessels) often occurs in the early stages of the disease. Therefore, a test excision in the diseased area is also useful at this stage. Later, only inflammatory reactions can be detected.
No specific changes can be detected serologically. Sometimes pathologically elevated concentrations of monoclonal antibodies or variable autoantibodies are detected. As part of a differential diagnosis, specific skin diseases such as erythema, skin tuberculosis, Buruli ulcer, erysipelas, leg ulcers or lues must be ruled out.
Complications
With this disease, patients suffer from a number of unpleasant skin ailments. This primarily leads to skin necrosis and also to the formation of ulcers under the skin. For this reason, those affected are also dependent on regular examinations in order to avoid various complications. However, the further course of the disease depends very much on the exact cause, so that a general prediction of the complications is usually not possible.
In the worst case, however, the skin layers can die off. Pustules and papules form on the skin itself. The ulcers are usually painful and the skin may take on an unnatural coloration. If the disease is not treated, in some cases the amputation of the affected limb is necessary.
The disease can be treated with medication. However, the primary focus is on treating the underlying disease. As a rule, those affected also need psychological treatment. Life expectancy is not negatively affected by successful treatment. However, due to the treatment, it cannot be ruled out that the symptoms will not recur in the patient’s life.
Treatment & Therapy
Non-adherent wound dressings and wound dressings that promote the formation of granulation tissue are used to treat pyoderma gangrenosum. Furthermore, wound healing is also accelerated by regular scraping (curettage) on the affected areas. However, removal of necrosis by surgical means is contraindicated because it can enlarge the lesions.
This effect is also referred to as a pathergy phenomenon. Overall, good results are obtained with the systemic use of immunosuppressants. High-dose glucocorticoids in combination with cytostatics such as azathioprine or cyclophosphamide are mainly used as immunosuppressants. Therapy with glucocorticoids alone often leads to a recurrence after its discontinuation. Therefore, better results occur when combining different processes leading to immunosuppression.
Bacterial colonization of the wounds can be prevented by compresses with Rivanol and, in the case of milder forms, by baths with common salt and chlorhexidine. The movement of the patient should not be restricted during the treatment, because regular walks promote the flow of lymph. As a result, any swelling that occurs can be easily reduced. An accompanying pain treatment is carried out by the administration of analgesics.
An important component of the therapy is the psychological treatment. Psychological side effects are also to be expected, particularly in the case of such an extreme course of disease as pyoderma gangrenosum. Methods such as autogenic training, progressive muscle relaxation or individual deep relaxation help to reduce stress. Psychotherapeutic treatment is also recommended.
Prevention
Since the exact cause of pyoderma gangrenosum is not known, there are no specific recommendations for its prevention. Existing autoimmune diseases increase the likelihood of developing symptoms of this disease. It is likely that ongoing treatment of the underlying condition can also reduce the risk of pyoderma gangrenosum.
General recommendations for a healthy lifestyle with a balanced diet, plenty of exercise, and avoiding alcohol and cigarettes are always good for the well-being of the body. In individual cases, they may also contribute to reducing the risk of pyoderma gangrenosum.
Aftercare
In most cases, those affected by pyoderma gangrenosum only have a few measures and options for direct aftercare. For this reason, a doctor should be consulted at an early stage in order to prevent further complications or symptoms from this disease. Self-healing cannot occur, so a doctor should be contacted at the first signs or symptoms.
Many of those affected are dependent on taking various medications. It is always important to ensure that it is taken regularly and that the dosage is correct in order to counteract the symptoms properly and permanently. Furthermore, those affected should also wear compression stockings to completely heal the pyoderma gangrenosum. If anything is unclear, you have questions or if you have any side effects, you should always consult a doctor first.
Many of those affected also depend on psychological help during treatment, with the support of their own family in particular having a very positive effect on the further course of the disease. However, the further course of pyoderma gangrenosum depends heavily on the time of diagnosis and the severity of the disease, so that a general prediction is not possible.
