Pyoderma gangrenosum: a rare scary lesion

Pyoderma gangrenosum is a rare disease and difficult to diagnose. This is a rapidly progressive disease, the later it is resistant to treatment. What is pyoderma gangrenosum, symptoms and treatment? Join SignsSymptomsList to find out information about this scary disease!

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• 1. Introduction:
• 2. Types of pyoderma gangrene
• 3. Stages of development of pyoderma gangrenosum
• 4. Differential diagnosis of pyoderma gangrenosum
• 5. Diagnosis of pyoderma gangrenosum
• 5. Treatment of pyoderma gangrene

1. Introduction:

1.1 Definition:

Pyoderma gangreneosum "Pyoderma Gangreneosum" (referred to as PG) has a nature that is not what its name suggests. This is a non-infectious (pus-producing) lesion. Pyoderma gangrenosum is a chronic inflammatory skin disease caused by many causes.

1.2 Be more specific about this disease

Due to a decrease in the function of neutrophils (a type of cell involved in the body's fight against germs). In patients with pyoderma gangrenosum, neutrophils are abundant in the affected tissue, even though no bacteria are present.

The gene mutation stimulates neutrophils and macrophages (cells that eat bacteria) to increase secretion of inflammatory substances. Inflammation is increased in an abnormal, pathological way that has no explanation.

This is a very complex disease, the causes and mechanisms are not understood clearly and thoroughly.

Read more Learn about white blood cells - the warriors that protect the body

1.3 Frequency of illness

 For every 1,000,000 people, there are 1-3 cases of pyoderma gangrenosum

Pyoderma gangrenosum can appear in all subjects, at any age, but is common in 20-50 years old.

The male:female ratio is almost equal. There is some literature that women are more common.

This presence was not evident in the Asian population. However, it can still be confirmed that this is a rare disease. One study in the Japanese population found only 62 cases of PG over the course of 30 years.

2. Types of pyoderma gangrene

Typical (ulcerative): The site of manifestation is in 2 lower extremities. The progression of the ulcer is very rapid, the border of the lesion is blue-violet. Often accompanied by diseases: inflammatory bowel disease, arthritis, blood cancer

Bullous: Usually appears on the face, can also be on the back of the hands, forearms, blue - gray. Often accompanied by blood cancer (acute myeloid leukemia).

Pustular: Usually appears on the legs or upper half of the body (from the waist up). These blisters are painful and have a red border. Occurs with inflammatory bowel disease

Pustular PG lesions

Vegetative form: Appears in the trunk. Ulcers have raised buds, no purple border. This is the most benign lesion, usually not associated with other diseases.

Ulcers around the navel: Often accompanied by inflammatory bowel disease or bowel cancer.

Postoperative (post-operative) ulcers: Appears after surgery in the chest area. Usually occurs 7-11 days after surgery.

3. Stages of development of pyoderma gangrenosum

Advanced ulcer stage:

Happens often very quickly, from a small ulcer with a red border, then turning red - purple, then a large ulcer that is difficult to define the border. Pain is very intense during this stage, especially with rapidly progressing ulcers.

Recovery phase:

The wound margin healed, the ulcer began to disappear

PG after 6 weeks of treatment

4. Differential diagnosis of pyoderma gangrenosum

There is no one specific test to diagnose PG. Therefore, usually to make a diagnosis, the doctor will rule out all other possible, more common causes. Including:

• Infection: The necessary tests are: pus culture of the ulcer, chest X-ray.
• Vasculitis and other autoimmune diseases: Vasculitis, systemic lupus erythematosus . Other immunological and hemostatic tests should be performed.
• Neutrophils disorders: Sweet's syndrome: Sudden fever and rash, pain. Examination and questioning are enough to rule it out.
• Vascular lesions: Doppler vascular ultrasound, angiography to exclude.
• Due to external damage: insect bites, trauma. Exclude by examination and questioning of the patient.

5. Diagnosis of pyoderma gangrenosum

Traditionally, a rapidly progressive ulcerative lesion with indistinct margins and all other causes ruled out would aid the physician in the diagnosis of PG.

However, today there are a number of studies that suggest other diagnostic modalities to help doctors not miss this pathology.

Marverakis Criteria:

The patient must meet the criteria: “Biopsy for wound 

The lesion showed a large infiltration of neutrophils” along with 4/6 minor criteria for the diagnosis of PG. Sub-criteria, including:

• Rule out infection.
• Previous trauma (the sore can be much more extensive than the original injury).
• Have inflammatory bowel disease or rheumatoid arthritis.
• Nodular lesions were present before the ulceration, or cysts progressed to ulceration rapidly (within 4 days).
• There is a lesion as shown with pain at the ulcer site.
• The ulcer heals, leaving a "wrinkled paper" image.

• Sign of "wrinkled paper"

Trial treatment noted a reduction in ulcer size within 1 month.

This criterion helps to accurately diagnose 90% of PG cases.

5. Treatment of pyoderma gangrene

Surgery to remove severely damaged tissue may be considered. Do not perform extensive excision of the ulcer tissue, as this may cause the ulcer to become larger. Skin grafting should be performed when PG has stopped progressing. The goal is to help the injury reduce the likelihood of an external injury.

Antibiotics should be considered before a diagnosis of PG has been established. Because PG is a non-infectious disease. Some cases of PG sores have bacteria present, this may be due to the loss of the skin covering, allowing bacteria to enter after the PG has formed. Antibiotics have no role in common PG disease

5.1 Small diameter necrotizing pyoderma

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• Topical corticosteroids
• Tacrolimus Oil
• Topical Cyclosporin
• Antibiotics with anti-inflammatory effects such as: Doxycylin or Minocyline
• The average duration of treatment is 6 weeks

5.1 Large diameter necrotizing pyoderma

Treatment with systemic immunosuppressive drugs such as: Prednisone orally or Methylprednisolone when given intravenously.

Cyclosporin is more potent than prednisone. This drug is prescribed for treatment-resistant PG. The main side effects of this drug are kidney failure and increased blood pressure.

And some other uncommon types of treatment such as biological therapy

After treatment with high dose, attack, the patient will be treated with a gradual decrease in dose over the next few months.

PG is a disease that is not caused by an infection. But an infection can be present in an infected patient. This condition is rare, and difficult to diagnose and treat. More medical research is needed to find out the causes, mechanisms, and treatments for pyoderma gangrenosum. 

Doctor  Nguyen Doan Trong Nhan