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Severe Bilateral Lower Extremity Pyoderma Gangrenosum

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After excluding other possible diagnoses through various testing and biopsy, the presumptive diagnosis of pyoderma gangrenosum (PG) was suspected despite the patient's history of failing prednisone and infliximab therapy... Pyoderma gangrenosum is a rare, poorly understood disease characterized by expansive ulcerating cutaneous lesions... Occurring in approximately 1:100,000, it is mainly observed in young to middle-aged individuals, with a female predominance; however, it may affect people of all ages., Pyoderma gangrenosum is associated with autoimmune disorders such as inflammatory bowel disease (IBD), rheumatoid arthritis, as well as neutrophilic dysregulation disorders such as Sweet's or Behcet's syndrome 50% to 70% of the time. ,, Of note, PG can also appear as a paraneoplastic process in those with myeloproliferative malignancies... Pyoderma gangrenosum usually starts as a small, sterile inflammatory nodule or pustule at the site of minor trauma, which transforms into an ulcerative lesion... Workup should include a rheumatologic panel including an antinuclear antibody and antineutrophil cytoplasmic antibodies to screen for IBD or autoimmune conditions, and a colonoscopy may be useful to evaluate for IBD... Topical agents such as corticosteroids, tacrolimus, and cyclosporine are important, especially around the inflamed borders of the ulcer; however, they are highly absorbed and tacrolimus levels, especially, should be monitored... Topical antimicrobials such as mupirocin or silver sulfadiazine can be used to prevent superinfection... Wet to dry or adherent dressings should absolutely be avoided, as they may aggravate the pathergy associated with PG. - Because of the process of pathergy, surgical intervention is only indicated if the disease is controlled or profound necrosis is present (Figs 2 and 3)... Skin grafting should only be attempted in patients currently undergoing immunosuppressive therapy, as ulcers may even develop at the skin graft donor site... Total colectomy may cause remission if IBD is the underlying disorder; however, recurrence may develop at the stoma... Ultimately, even with effective treatment, PG has a chronic and relapsing course... Pyoderma gangrenosum can be a devastating disease of expanding cutaneous ulcerations clinically diagnosed once others are excluded... Clinical history can be useful if there is a known concomitant autoimmune disease.

No MeSH data available.


Related in: MedlinePlus

Severe bilateral lower extremity wounds that had progressed over months despite aggressive wound care and medical management with immunomodulators and steroids.
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Figure 1: Severe bilateral lower extremity wounds that had progressed over months despite aggressive wound care and medical management with immunomodulators and steroids.

Mentions: Pyoderma gangrenosum usually starts as a small, sterile inflammatory nodule or pustule at the site of minor trauma, which transforms into an ulcerative lesion.3 The most common location is in the pretibial region, but it may occur anywhere.1 Pyoderma gangrenosum may also be associated with systemic constitutional symptoms due to the elevation of IL-1B and the promotion of the inflammatory cascade.1 It can extend in a symmetric or asymmetric manner or by developing satellite lesions at sites of trauma (Fig 1).3 This process, known as pathergy, is one of the reasons why immune system dysregulation, specifically regarding neutrophils, is believed to be part of the pathophysiology.


Severe Bilateral Lower Extremity Pyoderma Gangrenosum
Severe bilateral lower extremity wounds that had progressed over months despite aggressive wound care and medical management with immunomodulators and steroids.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC5120372&req=5

Figure 1: Severe bilateral lower extremity wounds that had progressed over months despite aggressive wound care and medical management with immunomodulators and steroids.
Mentions: Pyoderma gangrenosum usually starts as a small, sterile inflammatory nodule or pustule at the site of minor trauma, which transforms into an ulcerative lesion.3 The most common location is in the pretibial region, but it may occur anywhere.1 Pyoderma gangrenosum may also be associated with systemic constitutional symptoms due to the elevation of IL-1B and the promotion of the inflammatory cascade.1 It can extend in a symmetric or asymmetric manner or by developing satellite lesions at sites of trauma (Fig 1).3 This process, known as pathergy, is one of the reasons why immune system dysregulation, specifically regarding neutrophils, is believed to be part of the pathophysiology.

View Article: PubMed Central - PubMed

AUTOMATICALLY GENERATED EXCERPT
Please rate it.

After excluding other possible diagnoses through various testing and biopsy, the presumptive diagnosis of pyoderma gangrenosum (PG) was suspected despite the patient's history of failing prednisone and infliximab therapy... Pyoderma gangrenosum is a rare, poorly understood disease characterized by expansive ulcerating cutaneous lesions... Occurring in approximately 1:100,000, it is mainly observed in young to middle-aged individuals, with a female predominance; however, it may affect people of all ages., Pyoderma gangrenosum is associated with autoimmune disorders such as inflammatory bowel disease (IBD), rheumatoid arthritis, as well as neutrophilic dysregulation disorders such as Sweet's or Behcet's syndrome 50% to 70% of the time. ,, Of note, PG can also appear as a paraneoplastic process in those with myeloproliferative malignancies... Pyoderma gangrenosum usually starts as a small, sterile inflammatory nodule or pustule at the site of minor trauma, which transforms into an ulcerative lesion... Workup should include a rheumatologic panel including an antinuclear antibody and antineutrophil cytoplasmic antibodies to screen for IBD or autoimmune conditions, and a colonoscopy may be useful to evaluate for IBD... Topical agents such as corticosteroids, tacrolimus, and cyclosporine are important, especially around the inflamed borders of the ulcer; however, they are highly absorbed and tacrolimus levels, especially, should be monitored... Topical antimicrobials such as mupirocin or silver sulfadiazine can be used to prevent superinfection... Wet to dry or adherent dressings should absolutely be avoided, as they may aggravate the pathergy associated with PG. - Because of the process of pathergy, surgical intervention is only indicated if the disease is controlled or profound necrosis is present (Figs 2 and 3)... Skin grafting should only be attempted in patients currently undergoing immunosuppressive therapy, as ulcers may even develop at the skin graft donor site... Total colectomy may cause remission if IBD is the underlying disorder; however, recurrence may develop at the stoma... Ultimately, even with effective treatment, PG has a chronic and relapsing course... Pyoderma gangrenosum can be a devastating disease of expanding cutaneous ulcerations clinically diagnosed once others are excluded... Clinical history can be useful if there is a known concomitant autoimmune disease.

No MeSH data available.


Related in: MedlinePlus