Pyoderma gangraenosum is characterized by ulceration of the skin and formation 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.
What is pyoderma gangraenosum?
Pyoderma gangraenosum is characterized by ulceration and the death of entire sections of skin. The ulcer is called an ulcer and the extensive death of the skin is called gangrene. Pyoderma gangraenosum is not an infection, but an autoimmune disease in which the skin is attacked by its own immune system. There is an activation of the white blood cells. Only immunosuppressive drugs such as glucocorticoids, cyclosporin A or dapsone can sufficiently weaken the immune system and contribute to remission of the disease symptoms. Pyoderma gangraenosum often occurs in the setting of another underlying disease such as ulcerative colitis, Krohn’s disease, rheumatoid arthritis, vasculitis, chronic hepatitis, or even leukemia. However, it can also develop after skin injuries or after surgery from the surgical wound. The exact mechanism of its development is not yet known. Usually, ulcerations develop very slowly. However, there are also cases associated with massive outbreaks.
Causes
Pyoderma gangraenosum is an autoimmune disease, the cause of which has not been fully determined. In many cases, it is also interpreted as a complication of an existing autoimmune disease. This is also shown by the associations of pyoderma gangraenosum with other autoimmune diseases. Thus, about ten percent of all patients with pyoderma gangraenosum 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 metabolic syndrome is also suspected. Overall, it is believed that pyoderma gangraenosum is not an isolated skin disease but is an expression of a skin reaction in a generalized systemic disease process on an autoimmunologic basis.
Symptoms, complaints, and signs
Pyoderma gangraenosum often begins with the formation of pustules and papules on the skin that rapidly enlarge and coalesce. Eventually, they later deteriorate and form a painful ulcer. In the center of the ulcer there is a central zone of necrosis. A bluish-livid discoloration appears toward the border area with 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 limbs. The lower legs are affected in 80 percent of cases. However, all other skin areas can also be affected by pyoderma gangraenosum. Severe courses of the disease are to be expected when the affected areas are infected. Very often, other symptoms also occur. Depending on the underlying disease, severe digestive problems, rheumatic symptoms, or chronic respiratory problems often occur.
Diagnosis and course of the disease
For the most part, the diagnosis of pyoderma gangraenosum is based on the typical clinical manifestations of the disease. In the early stages of the disease, vasculitis (inflammation of the blood vessels) often also occurs. Therefore, a trial excision in the diseased area is also useful at this stage. Later, only inflammatory reactions can be detected. Serologically, no specific changes can be detected. Sometimes pathologically elevated concentrations of monoclonal antibodies or variable autoantibodies are detected. As part of a differential diagnosis, specific skin diseases such as erythema, cutaneous tuberculosis, Buruli ulcer, erysipelas, leg ulcers, or even syphilis must be excluded.
Complications
In this disease, patients suffer from a number of unpleasant skin complaints. This primarily involves skin necrosis and further also the formation of ulcers under the skin. For this reason, patients 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 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 associated with pain and the skin can take on an unnatural color. If the disease is not treated, amputation of the affected limbs may be necessary in some cases. The disease can be treated with the help of medications. However, the main focus is on the treatment of the underlying disease. As a rule, those affected are also dependent on psychological treatment. Life expectancy is not negatively affected if treatment is successful. However, based on the treatment, it cannot be ruled out that the complaints will not recur in the patient’s life.
Treatment and therapy
Nonadherent dressings and wound dressings that promote the formation of granulation tissue are used to treat pyoderma gangraenosum. Furthermore, wound healing is also accelerated by regular scrapings (curettage) of the affected areas. However, necrosis ablation by surgical means is contraindicated because it can further increase the size of the lesions. This effect is also known as the pathergy phenomenon. Overall, good results are obtained by systemic application of immunosuppressants. Immunosuppressants are mainly high-dose glucocorticoids in combination with cytostatics such as azathioprine or cyclophosphamide. Therapy with glucocorticoids alone often leads to relapse after discontinuation. Therefore, better results occur with the combination of different processes leading to immunosuppression. Bacterial colonization of wounds can be prevented by poultices with Rivanol and, in mild courses, by baths with saline and chlorhexidine. Patients’ movement should not be restricted during treatment, as regular walks promote lymph flow. This helps to reduce any swelling that may occur. Accompanying pain treatment is carried out by administering analgesics. Psychological treatment is an important component of the therapy. Especially in the case of such an extreme disease as pyoderma gangraenosum, psychological accompanying effects are to be expected. Methods such as autogenic training, progressive muscle relaxation or individual deep relaxation help to reduce stress. Psychotherapeutic treatment is also recommended.
Prevention
Because the exact cause of pyoderma gangraenosum is unknown, there are no specific recommendations for its prevention. Existing autoimmune diseases increase the likelihood of the appearance of symptoms of this disease. It is likely that ongoing treatment of the underlying disease can also reduce the risk of pyoderma gangraenosum. General recommendations for a healthy lifestyle with a balanced diet, plenty of exercise, and avoidance of alcohol and cigarettes are always good for the body’s well-being. They may also help reduce the risk of pyoderma gangraenosum in individual cases.
Follow-up
In most cases, only a few measures and options for direct aftercare are available to those affected by pyoderma gangraenosum. For this reason, a physician should be consulted at an early stage to prevent further complications or discomfort from this disease. Self-cure cannot occur, so a doctor should be contacted at the first signs or symptoms. Many of those affected are dependent on taking various medications. Here, it is always important to ensure that the medication is taken regularly and in the correct dosage in order to counteract the symptoms properly and permanently. Furthermore, those affected should also wear compression stockings in order to heal the pyoderma gangraenosum completely. A doctor should always be consulted first in case of any uncertainties, questions or side effects. Many of those affected are also dependent on psychological help during treatment, whereby the support of one’s own family in particular can have a very positive effect on the further course of the disease. The further course of pyoderma gangraenosum is, however, strongly dependent on the time of diagnosis and also on the manifestation of the disease, so that a general prediction is not possible.
