Extensive Pyoderma Gangrenosum: A Challenging Diagnosis and Literature Review of Management

Abstract

Pyoderma gangrenosum is a very rare, non-infectious leukocytic dermatosis, which is often associated with an underlying systemic disease. It is usually diagnosed based on the apparent clinical findings and by excluding other causes of ulcerative skin diseases. Treatment modality includes the use of systemic steroids and oral steroids. Immunosuppressive agents, such as cyclosporine and mycophenolate mofetil, can also be added if it fails to respond to steroids. We report a case of pyoderma gangrenosum in an 85-year-old female who presented in the inpatient facility with rapidly enlarging necrotic, ulcerative lesions with accompanying fever. She was managed with systemic steroids to which she responded well. This led to the arrest of the initially progressive lesions with some residual scarring.

Post-Operative Pyoderma Gangrenosum: A Long Journey for a Patient with Myelodysplastic Syndrome

“…It is characterized by painful and erythematous papules, pustules or vesicles that rapidly progress to ulcerations with a violaceous and necrotic border [ 1 ]. Typically seen in female patients, there are around 3-10 million cases of PG per year [ 2 , 3 ]. Approximately 50% of those patients have underlying diseases, such as inflammatory bowel disease (IBD), inflammatory arthritis, and hematological disorders or malignancy.…”

“…Pathergy, a phenomenon in which skin subjected to minor or major trauma will rapidly develop PG, is the underlying principle in post-operative PG [ 5 ]. Clinically, patients develop fevers and severe pain around the incision sites [ 2 , 3 , 5 ]. Other case reports have discussed post-operative PG after mastectomy, vein grafting, skin grafting, colostomies, cesarean sections, cholecystectomy, excisional basal cell carcinoma, orthopedic procedures, appendectomies and cardiothoracic procedures [ 6 ].…”

“…The time from surgery to development will vary depending on the sub-type of PG: generally, post-operative PG has been observed from four days to six weeks, while peristomal PG occurs an average of 18.4+/- 7.5 months after stoma creation [ 5 , 7 ]. The major diagnostic criteria include a rapidly progressive ulcerative skin lesion with irregular edges, purple-blue undermined borders with necrosis, and exclusion of other diseases [ 2 , 8 ]. Additionally, two minor criteria must be met, including the presence of underlying systemic diseases as mentioned above, response to corticosteroids, histopathology confirming neutrophilic infiltrates with or without lymphocytic vasculitis, or presence of pathergy [ 2 , 8 ].…”

“…The major diagnostic criteria include a rapidly progressive ulcerative skin lesion with irregular edges, purple-blue undermined borders with necrosis, and exclusion of other diseases [ 2 , 8 ]. Additionally, two minor criteria must be met, including the presence of underlying systemic diseases as mentioned above, response to corticosteroids, histopathology confirming neutrophilic infiltrates with or without lymphocytic vasculitis, or presence of pathergy [ 2 , 8 ].…”

Pyoderma Gangrenosum Overlying Venous Insufficiency: An Inevitable Misdiagnosis

“…The etiology and pathogenesis of PG are poorly understood [1]. The pathergy phenomenon, a hallmark of PG, is a condition in which trivial trauma leads to the development of similar lesions that may be resistant to healing [2]. This phenomenon implies that uncontrolled inflammatory responses to nonspecific stimuli may trigger PG [3].…”