Pyoderma gangrenosum (PG) is a rare, non-infectious dermatologic disease confined mainly to the lower limbs and presents with rapidly progressing painful ulceration. The cause of PG is still unknown, although it occurs more frequently in patients with autoimmune and systemic inflammatory diseases such as inflammatory bowel diseases (Crohn’s disease, ulcerative colitis), rheumatoid arthritis, and certain hematologic diseases. The disease may start from an erythematous papule, nodule including a pustular lesion that may increase rapidly to form mucocutaneous gangrene with undermined sloughing margins. The ulcers may increase in size if not treated early and are often sensitive to minor tissue injury (pathergy phenomenon) and, therefore difficult to manage.
Epidemiology
Fewer than 5% of all cases occur in children and the incidence of Pyoderma Gangrenosum Increases with age. The development of the disease occurs between 11 and 89 years of age.
Anatomy
Pathophysiology
Neutrophil Dysfunction: The two major mechanisms of the continuous activation and dysregulated chemotaxis of neutrophils are inflammation and tissue injury.
Cytokine Imbalance: Pro-inflammatory cytokines such TNF-α, IL-1, IL-6 and IL-8 increase the immune cell infiltrate in the skin thus increasing inflammation.
Genetic Susceptibility: Links to the syndrome symptoms in individuals with PSTPIP1 mutation are indicative of genetic factors in some cases.
Association with Systemic Diseases: PG is known to be associated with systemic inflammatory and autoimmune disorders, such as IBD, RA, and hematologic disorders suggesting common immunologic mechanisms.
Etiology
Immune Dysregulation:
PG is considered an autoinflammatory disease with an abnormality of the pattern of neutrophil inflammation. The studies have suggested that high activity of neutrophils is detrimental to tissue and causes ulceration.
Association with Systemic Diseases:
About 30% to 50% of the patients diagnosed with PG also have other associated systemic diseases, including:
Inflammatory Bowel Disease (IBD):
Crohn’s disease and ulcerative colitis.
Arthritis: Like rheumatoid arthritis
Hematologic Diseases:
Trauma or Skin Injury (Pathergy): Pathergy can occur, where even minor skin injuries such as cuts, injections, or surgical wounds can worsen or initiate the development of PG lesions.
Genetics
Prognostic Factors
Size and depth of ulcers:
Preliminary analysis of data suggests that the size and depth of the ulcers are important as it defines the prognosis and length of healing time.
Number of lesions: In this case multiple lesions may suggest a more aggressive disease process.
Location: Skin lesions in the lower limbs heal comparatively slower than lesions in other areas of the body because of inadequate blood supply.
Clinical History
Age Group:
PG in rare but can affect patients of any age, and it is most common in adults in their third to fifth decade of life. They are not so frequent in children and elderly patients. The condition may appear in both sexes, although it seems to present at a more frequent rate in women than in men.
Physical Examination
Lesion Appearance
Surrounding Skin
Distribution
Associated Symptoms
Age group
Associated comorbidity
Inflammatory Bowel Disease (IBD)
Rheumatologic and Autoimmune Disorders
Hematologic Disorders
Metabolic and Endocrine Conditions
Associated activity
Acuity of presentation
Acute Presentation:
Rapid Onset: Ulcers may develop suddenly, often within days, and can expand rapidly.
Pain: Acute presentation symptoms are usually very severe.
Signs of Infection: Like Inflammation: They may develop erythema, heat and edema around the ulcer.
Necrosis: Skin necrosis may be present and most striking, in the central area of the ulcer.
Chronic Presentation:
Slow Onset: They emerge because of factors which cause gradual deterioration and take longer to reach even their initial stage.
Pain and Inflammation: It may be less severe, or it may not be particularly severe, but pain can endure throughout the years.
Stable Ulceration: Unpredictably of acute PG, ulcers can remain in a stable stage without aggression.
Differential Diagnoses
Infectious Ulcers
Vasculitis Disorders
Autoimmune and Inflammatory Diseases
Neoplastic Conditions
Laboratory Studies
Imaging Studies
Procedures
Histologic Findings
Staging
Treatment Paradigm
Diagnosis and Assessment
Clinical Examination: Diagnosis is mainly clinical, owing to the clinical appearance of the ulcers and the patient history.
Exclusion of Other Conditions: It is essential to first exclude infections, vasculitis, other skin diseases.
Initial Treatment
Wound Care: The management of the ulcers is vital in this case. This may involve washing of the wounds, proper dressing and sometimes debridement.
Pain Management:
Pain control may be needed for the ulcers, and this will need the use of analgesics.
Medical Therapy
Corticosteroids: The most common are the systemic corticosteroids because they decrease inflammation and facilitate healing among patients.
Immunosuppressants: In case corticosteroids are not effective other drugs include azathioprine, mycophenolate mofetil and cyclosporine.
Biologics: For unresponsive cases, the use of biologic agents including Infliximab or adalimumab is viable where the patient also suffers from inflammatory diseases including rheumatoid arthritis or inflammatory bowel diseases.
Antibiotics: As with many illnesses, although PG is not caused by an infection, antibiotics may be necessary in cases where there is an addition bacterial infection.
Adjunctive Therapies
Intralesional Corticosteroids: Some of the patients may, however, benefit from injections into the lesions.
Compression Therapy: May be used to promote healing in some types of ulcers.
Hyperbaric Oxygen Therapy: It has been found to be beneficial in certain cases easing the process of healing in some ways.
Stress can exacerbate PG. Stress could be eased by such activities as mindfulness, meditation, or yoga.
Allergen Control: If certain allergens such as dust mites, pet dander, or certain kinds of foods cause inflammation, avoid them.
Moisturizing: Make sure the skin is well moisturized using specially purchased hypoallergenic moisturizer to avoid the issue of dryness developing cracks.
Infection Prevention:
Wound Care: Develop and maintain healthy practices that cover wound care of any injury or ulcer.
Temperature Regulation:
Climate Control: Hot, humid conditions can worsen PG. Maintain a comfortable indoor temperature and humidity level.
Effectiveness of Corticosteroids in treating pyoderma gangrenosum
Corticosteroids: They are usually the initial intervention given to the patient. They can be applied directly to the affected skin, taken orally or given up through injection to decrease inflammation.
Effectiveness of Immunosuppressants in treating pyoderma gangrenosum
The medications used may include azathioprine, cyclosporine, and mycophenolate mofetil to lower the immune system as well as decrease inflammation.
Diagnosis:
Essential assessment involves taking a clinical history and performing clinical examination to the patient. Exclusion of other pathology (such as infections or vasculitis).
Initial Management:
Wound Care: Care for ulcers to avoid accumulation of bacteria and other pathogens.
Pain Management: Relieving pain using pain relievers.
Medical Therapy:
Corticosteroids: Anti-inflammatory drugs such as systemic corticosteroids are the first-line treatment for a person with the condition.
Immunosuppressants: Some of the drugs used may include azathioprine, cyclosporine or mycophenolate mofetil when corticosteroids are ineffective.
Adjunctive Therapies:
Antibiotics: If secondary infection occurs.
Topical Treatments: Occlusive dressings, silver sulfadiazine or any other topical preparations can be used.
Surgical Intervention: Skin lesions may require debridement of necrotic tissue, but other extensive surgical procedures only exacerbate the situation.
Long-term Management: Symptom observational for the flare up of the disease and further medication alteration.
Treating coexisting disorders (e.g., inflammatory bowel disease) or conditions commonly seen in these patients.
Patient Education: Counselling on the PG patients.
Pyoderma gangrenosum (PG) is a rare, non-infectious dermatologic disease confined mainly to the lower limbs and presents with rapidly progressing painful ulceration. The cause of PG is still unknown, although it occurs more frequently in patients with autoimmune and systemic inflammatory diseases such as inflammatory bowel diseases (Crohn’s disease, ulcerative colitis), rheumatoid arthritis, and certain hematologic diseases. The disease may start from an erythematous papule, nodule including a pustular lesion that may increase rapidly to form mucocutaneous gangrene with undermined sloughing margins. The ulcers may increase in size if not treated early and are often sensitive to minor tissue injury (pathergy phenomenon) and, therefore difficult to manage.
Fewer than 5% of all cases occur in children and the incidence of Pyoderma Gangrenosum Increases with age. The development of the disease occurs between 11 and 89 years of age.
Neutrophil Dysfunction: The two major mechanisms of the continuous activation and dysregulated chemotaxis of neutrophils are inflammation and tissue injury.
Cytokine Imbalance: Pro-inflammatory cytokines such TNF-α, IL-1, IL-6 and IL-8 increase the immune cell infiltrate in the skin thus increasing inflammation.
Genetic Susceptibility: Links to the syndrome symptoms in individuals with PSTPIP1 mutation are indicative of genetic factors in some cases.
Association with Systemic Diseases: PG is known to be associated with systemic inflammatory and autoimmune disorders, such as IBD, RA, and hematologic disorders suggesting common immunologic mechanisms.
Immune Dysregulation:
PG is considered an autoinflammatory disease with an abnormality of the pattern of neutrophil inflammation. The studies have suggested that high activity of neutrophils is detrimental to tissue and causes ulceration.
Association with Systemic Diseases:
About 30% to 50% of the patients diagnosed with PG also have other associated systemic diseases, including:
Inflammatory Bowel Disease (IBD):
Crohn’s disease and ulcerative colitis.
Arthritis: Like rheumatoid arthritis
Hematologic Diseases:
Trauma or Skin Injury (Pathergy): Pathergy can occur, where even minor skin injuries such as cuts, injections, or surgical wounds can worsen or initiate the development of PG lesions.
Size and depth of ulcers:
Preliminary analysis of data suggests that the size and depth of the ulcers are important as it defines the prognosis and length of healing time.
Number of lesions: In this case multiple lesions may suggest a more aggressive disease process.
Location: Skin lesions in the lower limbs heal comparatively slower than lesions in other areas of the body because of inadequate blood supply.
Age Group:
PG in rare but can affect patients of any age, and it is most common in adults in their third to fifth decade of life. They are not so frequent in children and elderly patients. The condition may appear in both sexes, although it seems to present at a more frequent rate in women than in men.
Lesion Appearance
Surrounding Skin
Distribution
Associated Symptoms
Inflammatory Bowel Disease (IBD)
Rheumatologic and Autoimmune Disorders
Hematologic Disorders
Metabolic and Endocrine Conditions
Acute Presentation:
Rapid Onset: Ulcers may develop suddenly, often within days, and can expand rapidly.
Pain: Acute presentation symptoms are usually very severe.
Signs of Infection: Like Inflammation: They may develop erythema, heat and edema around the ulcer.
Necrosis: Skin necrosis may be present and most striking, in the central area of the ulcer.
Chronic Presentation:
Slow Onset: They emerge because of factors which cause gradual deterioration and take longer to reach even their initial stage.
Pain and Inflammation: It may be less severe, or it may not be particularly severe, but pain can endure throughout the years.
Stable Ulceration: Unpredictably of acute PG, ulcers can remain in a stable stage without aggression.
Infectious Ulcers
Vasculitis Disorders
Autoimmune and Inflammatory Diseases
Neoplastic Conditions
Diagnosis and Assessment
Clinical Examination: Diagnosis is mainly clinical, owing to the clinical appearance of the ulcers and the patient history.
Exclusion of Other Conditions: It is essential to first exclude infections, vasculitis, other skin diseases.
Initial Treatment
Wound Care: The management of the ulcers is vital in this case. This may involve washing of the wounds, proper dressing and sometimes debridement.
Pain Management:
Pain control may be needed for the ulcers, and this will need the use of analgesics.
Medical Therapy
Corticosteroids: The most common are the systemic corticosteroids because they decrease inflammation and facilitate healing among patients.
Immunosuppressants: In case corticosteroids are not effective other drugs include azathioprine, mycophenolate mofetil and cyclosporine.
Biologics: For unresponsive cases, the use of biologic agents including Infliximab or adalimumab is viable where the patient also suffers from inflammatory diseases including rheumatoid arthritis or inflammatory bowel diseases.
Antibiotics: As with many illnesses, although PG is not caused by an infection, antibiotics may be necessary in cases where there is an addition bacterial infection.
Adjunctive Therapies
Intralesional Corticosteroids: Some of the patients may, however, benefit from injections into the lesions.
Compression Therapy: May be used to promote healing in some types of ulcers.
Hyperbaric Oxygen Therapy: It has been found to be beneficial in certain cases easing the process of healing in some ways.
Avoiding Triggers:
Identify and Avoid Stressors:
Stress can exacerbate PG. Stress could be eased by such activities as mindfulness, meditation, or yoga.
Allergen Control: If certain allergens such as dust mites, pet dander, or certain kinds of foods cause inflammation, avoid them.
Moisturizing: Make sure the skin is well moisturized using specially purchased hypoallergenic moisturizer to avoid the issue of dryness developing cracks.
Infection Prevention:
Wound Care: Develop and maintain healthy practices that cover wound care of any injury or ulcer.
Temperature Regulation:
Climate Control: Hot, humid conditions can worsen PG. Maintain a comfortable indoor temperature and humidity level.
Corticosteroids: They are usually the initial intervention given to the patient. They can be applied directly to the affected skin, taken orally or given up through injection to decrease inflammation.
The medications used may include azathioprine, cyclosporine, and mycophenolate mofetil to lower the immune system as well as decrease inflammation.
Pyoderma gangrenosum (PG) is a rare, non-infectious dermatologic disease confined mainly to the lower limbs and presents with rapidly progressing painful ulceration. The cause of PG is still unknown, although it occurs more frequently in patients with autoimmune and systemic inflammatory diseases such as inflammatory bowel diseases (Crohn’s disease, ulcerative colitis), rheumatoid arthritis, and certain hematologic diseases. The disease may start from an erythematous papule, nodule including a pustular lesion that may increase rapidly to form mucocutaneous gangrene with undermined sloughing margins. The ulcers may increase in size if not treated early and are often sensitive to minor tissue injury (pathergy phenomenon) and, therefore difficult to manage.
Fewer than 5% of all cases occur in children and the incidence of Pyoderma Gangrenosum Increases with age. The development of the disease occurs between 11 and 89 years of age.
Neutrophil Dysfunction: The two major mechanisms of the continuous activation and dysregulated chemotaxis of neutrophils are inflammation and tissue injury.
Cytokine Imbalance: Pro-inflammatory cytokines such TNF-α, IL-1, IL-6 and IL-8 increase the immune cell infiltrate in the skin thus increasing inflammation.
Genetic Susceptibility: Links to the syndrome symptoms in individuals with PSTPIP1 mutation are indicative of genetic factors in some cases.
Association with Systemic Diseases: PG is known to be associated with systemic inflammatory and autoimmune disorders, such as IBD, RA, and hematologic disorders suggesting common immunologic mechanisms.
Immune Dysregulation:
PG is considered an autoinflammatory disease with an abnormality of the pattern of neutrophil inflammation. The studies have suggested that high activity of neutrophils is detrimental to tissue and causes ulceration.
Association with Systemic Diseases:
About 30% to 50% of the patients diagnosed with PG also have other associated systemic diseases, including:
Inflammatory Bowel Disease (IBD):
Crohn’s disease and ulcerative colitis.
Arthritis: Like rheumatoid arthritis
Hematologic Diseases:
Trauma or Skin Injury (Pathergy): Pathergy can occur, where even minor skin injuries such as cuts, injections, or surgical wounds can worsen or initiate the development of PG lesions.
Size and depth of ulcers:
Preliminary analysis of data suggests that the size and depth of the ulcers are important as it defines the prognosis and length of healing time.
Number of lesions: In this case multiple lesions may suggest a more aggressive disease process.
Location: Skin lesions in the lower limbs heal comparatively slower than lesions in other areas of the body because of inadequate blood supply.
Age Group:
PG in rare but can affect patients of any age, and it is most common in adults in their third to fifth decade of life. They are not so frequent in children and elderly patients. The condition may appear in both sexes, although it seems to present at a more frequent rate in women than in men.
Lesion Appearance
Surrounding Skin
Distribution
Associated Symptoms
Inflammatory Bowel Disease (IBD)
Rheumatologic and Autoimmune Disorders
Hematologic Disorders
Metabolic and Endocrine Conditions
Acute Presentation:
Rapid Onset: Ulcers may develop suddenly, often within days, and can expand rapidly.
Pain: Acute presentation symptoms are usually very severe.
Signs of Infection: Like Inflammation: They may develop erythema, heat and edema around the ulcer.
Necrosis: Skin necrosis may be present and most striking, in the central area of the ulcer.
Chronic Presentation:
Slow Onset: They emerge because of factors which cause gradual deterioration and take longer to reach even their initial stage.
Pain and Inflammation: It may be less severe, or it may not be particularly severe, but pain can endure throughout the years.
Stable Ulceration: Unpredictably of acute PG, ulcers can remain in a stable stage without aggression.
Infectious Ulcers
Vasculitis Disorders
Autoimmune and Inflammatory Diseases
Neoplastic Conditions
Diagnosis and Assessment
Clinical Examination: Diagnosis is mainly clinical, owing to the clinical appearance of the ulcers and the patient history.
Exclusion of Other Conditions: It is essential to first exclude infections, vasculitis, other skin diseases.
Initial Treatment
Wound Care: The management of the ulcers is vital in this case. This may involve washing of the wounds, proper dressing and sometimes debridement.
Pain Management:
Pain control may be needed for the ulcers, and this will need the use of analgesics.
Medical Therapy
Corticosteroids: The most common are the systemic corticosteroids because they decrease inflammation and facilitate healing among patients.
Immunosuppressants: In case corticosteroids are not effective other drugs include azathioprine, mycophenolate mofetil and cyclosporine.
Biologics: For unresponsive cases, the use of biologic agents including Infliximab or adalimumab is viable where the patient also suffers from inflammatory diseases including rheumatoid arthritis or inflammatory bowel diseases.
Antibiotics: As with many illnesses, although PG is not caused by an infection, antibiotics may be necessary in cases where there is an addition bacterial infection.
Adjunctive Therapies
Intralesional Corticosteroids: Some of the patients may, however, benefit from injections into the lesions.
Compression Therapy: May be used to promote healing in some types of ulcers.
Hyperbaric Oxygen Therapy: It has been found to be beneficial in certain cases easing the process of healing in some ways.
Avoiding Triggers:
Identify and Avoid Stressors:
Stress can exacerbate PG. Stress could be eased by such activities as mindfulness, meditation, or yoga.
Allergen Control: If certain allergens such as dust mites, pet dander, or certain kinds of foods cause inflammation, avoid them.
Moisturizing: Make sure the skin is well moisturized using specially purchased hypoallergenic moisturizer to avoid the issue of dryness developing cracks.
Infection Prevention:
Wound Care: Develop and maintain healthy practices that cover wound care of any injury or ulcer.
Temperature Regulation:
Climate Control: Hot, humid conditions can worsen PG. Maintain a comfortable indoor temperature and humidity level.
Corticosteroids: They are usually the initial intervention given to the patient. They can be applied directly to the affected skin, taken orally or given up through injection to decrease inflammation.
The medications used may include azathioprine, cyclosporine, and mycophenolate mofetil to lower the immune system as well as decrease inflammation.
Diagnosis:
Essential assessment involves taking a clinical history and performing clinical examination to the patient. Exclusion of other pathology (such as infections or vasculitis).
Initial Management:
Wound Care: Care for ulcers to avoid accumulation of bacteria and other pathogens.
Pain Management: Relieving pain using pain relievers.
Medical Therapy:
Corticosteroids: Anti-inflammatory drugs such as systemic corticosteroids are the first-line treatment for a person with the condition.
Immunosuppressants: Some of the drugs used may include azathioprine, cyclosporine or mycophenolate mofetil when corticosteroids are ineffective.
Adjunctive Therapies:
Antibiotics: If secondary infection occurs.
Topical Treatments: Occlusive dressings, silver sulfadiazine or any other topical preparations can be used.
Surgical Intervention: Skin lesions may require debridement of necrotic tissue, but other extensive surgical procedures only exacerbate the situation.
Long-term Management: Symptom observational for the flare up of the disease and further medication alteration.
Treating coexisting disorders (e.g., inflammatory bowel disease) or conditions commonly seen in these patients.
Patient Education: Counselling on the PG patients.
Both our subscription plans include Free CME/CPD AMA PRA Category 1 credits.
Digital Certificate PDF
On course completion, you will receive a full-sized presentation quality digital certificate.
medtigo Simulation
A dynamic medical simulation platform designed to train healthcare professionals and students to effectively run code situations through an immersive hands-on experience in a live, interactive 3D environment.
medtigo Points
medtigo points is our unique point redemption system created to award users for interacting on our site. These points can be redeemed for special discounts on the medtigo marketplace as well as towards the membership cost itself.
Community Forum post/reply = 5 points
*Redemption of points can occur only through the medtigo marketplace, courses, or simulation system. Money will not be credited to your bank account. 10 points = $1.
All Your Certificates in One Place
When you have your licenses, certificates and CMEs in one place, it's easier to track your career growth. You can easily share these with hospitals as well, using your medtigo app.
