The group of neutrophilic dermatoses includes the inflammatory condition pyoderma gangrenosum. It is important to distinguish Pyoderma gangrenosum from Pyogenic Granuloma, an entirely different condition with an equally inappropriate term.
Pyoderma gangrenosum, despite its name, is not an infection or gangrenous condition. Systemic illness is frequently linked to pyrogenic granuloma. The diagnosis is made medically after ruling out other skin conditions that are comparable.
Epidemiology
Less than 5% of incidents, it is thought, involve youngsters. The disease can start to manifest in people as young as 11 years old and as old as 89.
Anatomy
Pathophysiology
Uncertainty surrounds the pathophysiology of pyoderma gangrenosum. Genetic changes, neutrophil malfunction, & inflammatory/immune deregulation are thought to be involved. Clonal T-cell proliferation has been observed in some pyoderma gangrenosum lesions. Additionally, it has been proposed that inflammasomes are involved in the neutrophil chemotaxis that takes place in these lesions.
Complexes of receptors called inflammasomes are used in the innate immune system to signal. A mutant in Janus kinase 2, which is involved in the synthesis of numerous cytokines, is linked to some occurrences of pyoderma gangrenosum. T cell & macrophage dysfunctional cytokine signaling is probably a part of the illness process.
Inflammatory mediator concentration was found to be higher in pyoderma gangrenosum lesions. For instance, it has been discovered that IL-23 levels are higher in pyoderma gangrenosum lesions. Inflammation caused by IL-17 & neutrophil activation is both facilitated by IL-23.
Etiology
Irritable bowel syndrome, rheumatoid arthritis, & other autoimmune & inflammatory illnesses are the most typical linked systemic diseases. Additionally, solid malignancies & hematologic tumors are linked to pyoderma gangrenosum. In 5% – 12% of instances, pyoderma gangrenosum is linked to ulcerative colitis, and in 1% – 2% of instances, it is linked to Crohn’s disease.
The relationship between pyoderma gangrenosum & irritable bowel disease flare-ups and severity is unclear. Additionally, treatment for the intestinal condition that it is associated with does not usually cure pyoderma gangrenosum. In a study of 103 pyoderma gangrenosum patients, 19 percent of the individuals had seronegative arthritis, and 20 percent of the individuals had hematologic abnormalities.
Genetics
Prognostic Factors
Clinical History
Physical Examination
Physical examination
As seen in the picture below, the hallmark of classic pyoderma gangrenosum is deep ulceration with an overhanging violaceous border. Although they can appear anywhere on the body, these pyoderma gangrenosum lesions are most frequently found on the legs.
On the genitalia, pyoderma gangrenosum may manifest. It is important to distinguish between this variety and sexually transmitted illnesses, often known as vulvar and penile pyoderma gangrenosum.
In one case report, a patient with Crohn’s disease had pyoderma gangrenosum of the scrotum.
The ocular test can confirm the extracutaneous neutrophilic illness, and it has also been noted in the liver, lungs, & bones.
Age group
Associated comorbidity
Associated activity
Acuity of presentation
Differential Diagnoses
Differential diagnosis
Deep fungal infections and mycobacterial infections are among the infectious disorders on the differential diagnostic list. The differential also includes non-infectious conditions like iododerma and bromoderma.
Martorell ulcers and arterial ulcers are additional ulcer causes to consider. Pulses that are weaker on the adjacent anatomical side may be related to arterial ulcers.
Laboratory Studies
Imaging Studies
Procedures
Histologic Findings
A small, red papule and pustule that develops into bigger, ulcerative lesions is the typical initial lesion described by individuals with pyoderma gangrenosum as a biting reaction. Some people may exhibit cellulitis or what they believe to be an abscess. Patients frequently report having been bitten by a brown recluse or the other spider, but they frequently lack proof that the initial incident was indeed caused by a spider. The main complaint throughout history has been a pain. Malaise and arthralgias are frequently prevalent.
In order to identify any underlining systemic diseases, a thorough history should be collected, paying particular attention to the organ systems listed below. 50% of people with pyoderma gangrenosum experience systemic diseases, which may manifest before, concurrently with, or after the diagnosis. Irritable bowel syndrome, either ulcerative colitis and regional enteritis/disease, Crohn’s, and polyarthritis that is typically symmetrical and can be either seropositive or seronegative are among the disorders that are frequently related.
Other frequently present problems are hematologic illnesses/disorders, such as preleukemic or leukemia states, which are generally myelocytic in nature, and monoclonal gammopathies (primarily IgA [immunoglobulin A]). Other types of arthritis, such as osteoarthritis, psoriatic arthritis & spondyloarthropathy, liver diseases, such as primary biliary cirrhosis & hepatitis, myeloma (primarily of the IgA type), & immunologic disorders, such as Sjögren syndrome & lupus erythematosus, are less frequently associated diseases.
Staging
Treatment Paradigm
The severity of the underlying disease must be addressed if it exists, but there is no established link between it and the aggressiveness of pyoderma gangrenosum. The degree of systemic immunosuppression required depends on how quickly the illness develops. Cyclosporine or corticosteroids may be administered as systemic drugs if the area of the lesion is expanding quickly. Prednisolone & cyclosporine were tested for the management of pyoderma gangrenosum in the STOP GAP randomized control study in 2015.
The findings revealed no discernible difference between the two medications in the rate of tumor healing. At 6 months, 47 percent of patients using cyclosporine & 47 percent of patients taking prednisolone had treated ulcers, respectively. Both recurrence and negative reactions were comparable between the 2 groups. However, those getting prednisolone experienced more severe side effects, such as infections. Some doctors will administer immunosuppressant medications prophylactically before surgery if a patient has a history of pyoderma gangrenosum, particularly aggressive pyoderma gangrenosum, in order to avoid the development of pyoderma gangrenosum.
Combining methylprednisolone with cyclosporine is one such treatment plan. Avoiding needless procedures or surgery is obviously crucial. Topical and intralesional treatment may be sufficient on its own in cases with more sluggish or constrained illness. It has been successful to utilize topical tacrolimus, intralesional steroids, and both. Additionally, topical dapsone, nicotine, & sodium cromoglycate have all been tested. The main components of pyoderma gangrenosum treatment are wound care & pain management.
To stop infection, patients & medical professionals must clean the wound. Debridement is essential, but due to the previously described relationship with pathergy, it must be done with extreme caution to assure that only nonviable tissue is evacuated. Etanercept & adalimumab, two anti-TNF-alpha medications, are other successful treatments. An IL-12/23 inhibitor called ustekinumab, which is used to treat psoriasis, has been shown to benefit pyoderma gangrenosum. Canakinumab, an anti-IL-1 beta monoclonal antibody, & tocilizumab, an anti-IL-6 monoclonal antibody, are two more medications that may be beneficial in the management of pyoderma gangrenosum.
The group of neutrophilic dermatoses includes the inflammatory condition pyoderma gangrenosum. It is important to distinguish Pyoderma gangrenosum from Pyogenic Granuloma, an entirely different condition with an equally inappropriate term.
Pyoderma gangrenosum, despite its name, is not an infection or gangrenous condition. Systemic illness is frequently linked to pyrogenic granuloma. The diagnosis is made medically after ruling out other skin conditions that are comparable.
Less than 5% of incidents, it is thought, involve youngsters. The disease can start to manifest in people as young as 11 years old and as old as 89.
Uncertainty surrounds the pathophysiology of pyoderma gangrenosum. Genetic changes, neutrophil malfunction, & inflammatory/immune deregulation are thought to be involved. Clonal T-cell proliferation has been observed in some pyoderma gangrenosum lesions. Additionally, it has been proposed that inflammasomes are involved in the neutrophil chemotaxis that takes place in these lesions.
Complexes of receptors called inflammasomes are used in the innate immune system to signal. A mutant in Janus kinase 2, which is involved in the synthesis of numerous cytokines, is linked to some occurrences of pyoderma gangrenosum. T cell & macrophage dysfunctional cytokine signaling is probably a part of the illness process.
Inflammatory mediator concentration was found to be higher in pyoderma gangrenosum lesions. For instance, it has been discovered that IL-23 levels are higher in pyoderma gangrenosum lesions. Inflammation caused by IL-17 & neutrophil activation is both facilitated by IL-23.
Irritable bowel syndrome, rheumatoid arthritis, & other autoimmune & inflammatory illnesses are the most typical linked systemic diseases. Additionally, solid malignancies & hematologic tumors are linked to pyoderma gangrenosum. In 5% – 12% of instances, pyoderma gangrenosum is linked to ulcerative colitis, and in 1% – 2% of instances, it is linked to Crohn’s disease.
The relationship between pyoderma gangrenosum & irritable bowel disease flare-ups and severity is unclear. Additionally, treatment for the intestinal condition that it is associated with does not usually cure pyoderma gangrenosum. In a study of 103 pyoderma gangrenosum patients, 19 percent of the individuals had seronegative arthritis, and 20 percent of the individuals had hematologic abnormalities.
Physical examination
As seen in the picture below, the hallmark of classic pyoderma gangrenosum is deep ulceration with an overhanging violaceous border. Although they can appear anywhere on the body, these pyoderma gangrenosum lesions are most frequently found on the legs.
On the genitalia, pyoderma gangrenosum may manifest. It is important to distinguish between this variety and sexually transmitted illnesses, often known as vulvar and penile pyoderma gangrenosum.
In one case report, a patient with Crohn’s disease had pyoderma gangrenosum of the scrotum.
The ocular test can confirm the extracutaneous neutrophilic illness, and it has also been noted in the liver, lungs, & bones.
Differential diagnosis
Deep fungal infections and mycobacterial infections are among the infectious disorders on the differential diagnostic list. The differential also includes non-infectious conditions like iododerma and bromoderma.
Martorell ulcers and arterial ulcers are additional ulcer causes to consider. Pulses that are weaker on the adjacent anatomical side may be related to arterial ulcers.
A small, red papule and pustule that develops into bigger, ulcerative lesions is the typical initial lesion described by individuals with pyoderma gangrenosum as a biting reaction. Some people may exhibit cellulitis or what they believe to be an abscess. Patients frequently report having been bitten by a brown recluse or the other spider, but they frequently lack proof that the initial incident was indeed caused by a spider. The main complaint throughout history has been a pain. Malaise and arthralgias are frequently prevalent.
In order to identify any underlining systemic diseases, a thorough history should be collected, paying particular attention to the organ systems listed below. 50% of people with pyoderma gangrenosum experience systemic diseases, which may manifest before, concurrently with, or after the diagnosis. Irritable bowel syndrome, either ulcerative colitis and regional enteritis/disease, Crohn’s, and polyarthritis that is typically symmetrical and can be either seropositive or seronegative are among the disorders that are frequently related.
Other frequently present problems are hematologic illnesses/disorders, such as preleukemic or leukemia states, which are generally myelocytic in nature, and monoclonal gammopathies (primarily IgA [immunoglobulin A]). Other types of arthritis, such as osteoarthritis, psoriatic arthritis & spondyloarthropathy, liver diseases, such as primary biliary cirrhosis & hepatitis, myeloma (primarily of the IgA type), & immunologic disorders, such as Sjögren syndrome & lupus erythematosus, are less frequently associated diseases.
The severity of the underlying disease must be addressed if it exists, but there is no established link between it and the aggressiveness of pyoderma gangrenosum. The degree of systemic immunosuppression required depends on how quickly the illness develops. Cyclosporine or corticosteroids may be administered as systemic drugs if the area of the lesion is expanding quickly. Prednisolone & cyclosporine were tested for the management of pyoderma gangrenosum in the STOP GAP randomized control study in 2015.
The findings revealed no discernible difference between the two medications in the rate of tumor healing. At 6 months, 47 percent of patients using cyclosporine & 47 percent of patients taking prednisolone had treated ulcers, respectively. Both recurrence and negative reactions were comparable between the 2 groups. However, those getting prednisolone experienced more severe side effects, such as infections. Some doctors will administer immunosuppressant medications prophylactically before surgery if a patient has a history of pyoderma gangrenosum, particularly aggressive pyoderma gangrenosum, in order to avoid the development of pyoderma gangrenosum.
Combining methylprednisolone with cyclosporine is one such treatment plan. Avoiding needless procedures or surgery is obviously crucial. Topical and intralesional treatment may be sufficient on its own in cases with more sluggish or constrained illness. It has been successful to utilize topical tacrolimus, intralesional steroids, and both. Additionally, topical dapsone, nicotine, & sodium cromoglycate have all been tested. The main components of pyoderma gangrenosum treatment are wound care & pain management.
To stop infection, patients & medical professionals must clean the wound. Debridement is essential, but due to the previously described relationship with pathergy, it must be done with extreme caution to assure that only nonviable tissue is evacuated. Etanercept & adalimumab, two anti-TNF-alpha medications, are other successful treatments. An IL-12/23 inhibitor called ustekinumab, which is used to treat psoriasis, has been shown to benefit pyoderma gangrenosum. Canakinumab, an anti-IL-1 beta monoclonal antibody, & tocilizumab, an anti-IL-6 monoclonal antibody, are two more medications that may be beneficial in the management of pyoderma gangrenosum.
The group of neutrophilic dermatoses includes the inflammatory condition pyoderma gangrenosum. It is important to distinguish Pyoderma gangrenosum from Pyogenic Granuloma, an entirely different condition with an equally inappropriate term.
Pyoderma gangrenosum, despite its name, is not an infection or gangrenous condition. Systemic illness is frequently linked to pyrogenic granuloma. The diagnosis is made medically after ruling out other skin conditions that are comparable.
Less than 5% of incidents, it is thought, involve youngsters. The disease can start to manifest in people as young as 11 years old and as old as 89.
Uncertainty surrounds the pathophysiology of pyoderma gangrenosum. Genetic changes, neutrophil malfunction, & inflammatory/immune deregulation are thought to be involved. Clonal T-cell proliferation has been observed in some pyoderma gangrenosum lesions. Additionally, it has been proposed that inflammasomes are involved in the neutrophil chemotaxis that takes place in these lesions.
Complexes of receptors called inflammasomes are used in the innate immune system to signal. A mutant in Janus kinase 2, which is involved in the synthesis of numerous cytokines, is linked to some occurrences of pyoderma gangrenosum. T cell & macrophage dysfunctional cytokine signaling is probably a part of the illness process.
Inflammatory mediator concentration was found to be higher in pyoderma gangrenosum lesions. For instance, it has been discovered that IL-23 levels are higher in pyoderma gangrenosum lesions. Inflammation caused by IL-17 & neutrophil activation is both facilitated by IL-23.
Irritable bowel syndrome, rheumatoid arthritis, & other autoimmune & inflammatory illnesses are the most typical linked systemic diseases. Additionally, solid malignancies & hematologic tumors are linked to pyoderma gangrenosum. In 5% – 12% of instances, pyoderma gangrenosum is linked to ulcerative colitis, and in 1% – 2% of instances, it is linked to Crohn’s disease.
The relationship between pyoderma gangrenosum & irritable bowel disease flare-ups and severity is unclear. Additionally, treatment for the intestinal condition that it is associated with does not usually cure pyoderma gangrenosum. In a study of 103 pyoderma gangrenosum patients, 19 percent of the individuals had seronegative arthritis, and 20 percent of the individuals had hematologic abnormalities.
Physical examination
As seen in the picture below, the hallmark of classic pyoderma gangrenosum is deep ulceration with an overhanging violaceous border. Although they can appear anywhere on the body, these pyoderma gangrenosum lesions are most frequently found on the legs.
On the genitalia, pyoderma gangrenosum may manifest. It is important to distinguish between this variety and sexually transmitted illnesses, often known as vulvar and penile pyoderma gangrenosum.
In one case report, a patient with Crohn’s disease had pyoderma gangrenosum of the scrotum.
The ocular test can confirm the extracutaneous neutrophilic illness, and it has also been noted in the liver, lungs, & bones.
Differential diagnosis
Deep fungal infections and mycobacterial infections are among the infectious disorders on the differential diagnostic list. The differential also includes non-infectious conditions like iododerma and bromoderma.
Martorell ulcers and arterial ulcers are additional ulcer causes to consider. Pulses that are weaker on the adjacent anatomical side may be related to arterial ulcers.
A small, red papule and pustule that develops into bigger, ulcerative lesions is the typical initial lesion described by individuals with pyoderma gangrenosum as a biting reaction. Some people may exhibit cellulitis or what they believe to be an abscess. Patients frequently report having been bitten by a brown recluse or the other spider, but they frequently lack proof that the initial incident was indeed caused by a spider. The main complaint throughout history has been a pain. Malaise and arthralgias are frequently prevalent.
In order to identify any underlining systemic diseases, a thorough history should be collected, paying particular attention to the organ systems listed below. 50% of people with pyoderma gangrenosum experience systemic diseases, which may manifest before, concurrently with, or after the diagnosis. Irritable bowel syndrome, either ulcerative colitis and regional enteritis/disease, Crohn’s, and polyarthritis that is typically symmetrical and can be either seropositive or seronegative are among the disorders that are frequently related.
Other frequently present problems are hematologic illnesses/disorders, such as preleukemic or leukemia states, which are generally myelocytic in nature, and monoclonal gammopathies (primarily IgA [immunoglobulin A]). Other types of arthritis, such as osteoarthritis, psoriatic arthritis & spondyloarthropathy, liver diseases, such as primary biliary cirrhosis & hepatitis, myeloma (primarily of the IgA type), & immunologic disorders, such as Sjögren syndrome & lupus erythematosus, are less frequently associated diseases.
The severity of the underlying disease must be addressed if it exists, but there is no established link between it and the aggressiveness of pyoderma gangrenosum. The degree of systemic immunosuppression required depends on how quickly the illness develops. Cyclosporine or corticosteroids may be administered as systemic drugs if the area of the lesion is expanding quickly. Prednisolone & cyclosporine were tested for the management of pyoderma gangrenosum in the STOP GAP randomized control study in 2015.
The findings revealed no discernible difference between the two medications in the rate of tumor healing. At 6 months, 47 percent of patients using cyclosporine & 47 percent of patients taking prednisolone had treated ulcers, respectively. Both recurrence and negative reactions were comparable between the 2 groups. However, those getting prednisolone experienced more severe side effects, such as infections. Some doctors will administer immunosuppressant medications prophylactically before surgery if a patient has a history of pyoderma gangrenosum, particularly aggressive pyoderma gangrenosum, in order to avoid the development of pyoderma gangrenosum.
Combining methylprednisolone with cyclosporine is one such treatment plan. Avoiding needless procedures or surgery is obviously crucial. Topical and intralesional treatment may be sufficient on its own in cases with more sluggish or constrained illness. It has been successful to utilize topical tacrolimus, intralesional steroids, and both. Additionally, topical dapsone, nicotine, & sodium cromoglycate have all been tested. The main components of pyoderma gangrenosum treatment are wound care & pain management.
To stop infection, patients & medical professionals must clean the wound. Debridement is essential, but due to the previously described relationship with pathergy, it must be done with extreme caution to assure that only nonviable tissue is evacuated. Etanercept & adalimumab, two anti-TNF-alpha medications, are other successful treatments. An IL-12/23 inhibitor called ustekinumab, which is used to treat psoriasis, has been shown to benefit pyoderma gangrenosum. Canakinumab, an anti-IL-1 beta monoclonal antibody, & tocilizumab, an anti-IL-6 monoclonal antibody, are two more medications that may be beneficial in the management of pyoderma gangrenosum.
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.
