Pyoderma gangrenosum study pilot registry: The first step to a better understanding.

The objective of this study was to develop a pilot physician driven patient pyoderma gangrenosum (PG) registry to summarise patient baseline demographics, PG-related medical history, treatments, and outcomes for patients with pyoderma gangrenosum. Standardised patient information was collected prospectively during clinical encounters between December 2019 and July 2021 at a single academic institution. Eligibility criteria for the study was a diagnosis of pyoderma gangrenosum determined by a PARACELSUS score of at least 10 for ulcerative patients. Main outcome measures included demographic data, PG related history and comorbidities, past and current treatments, healing outcomes, hospitalisations and recurrences of PG. The Pyoderma Gangrenosum Study (PYGAS) Registry currently includes 52 patients with 56 target lesions of four distinct PG subtypes (41 ulcerative, 12 peristomal, 2 vegetative and 1 bullous). For the 38 patients with 41 total ulcerative PG lesions, referrals to our institution most commonly came from dermatologists (42.1%). The median follow-up time in our initial registry was 5.5 months (95% CI = 4.1-11.5 months), with average time between follow-up visits at 1.1 months. These ulcers were most commonly treated with first-line systemic immunosuppressants (70.6%), such as corticosteroids or cyclosporine. Additional use of systemic immunomodulators at baseline visit was statistically significantly associated with healing (P = 0.048). This pilot study suggests that use of systemic immunomodulators has an impact on healing of PG patients. Wound care regimens are variable, and assessing their impact on treatment outcomes could be challenging. Standardisation of both wound care regimens and data collection in prospective clinical studies is necessary to assess their impact in PG treatment outcomes.

Gap of knowledge in diagnosis of pyoderma gangrenosum in clinical specialties education.

OBJECTIVES: Pyoderma gangrenosum (PG) is a chronic ulcerative neutrophilic dermatosis. It presents a diagnostic challenge due to the absence of disease-specific markers or histopathology, lack of universally accepted diagnostic criteria, and many mimicking diseases including necrotizing soft tissue infections (NSTI). PG cases often present first to specialties other than dermatology. We reviewed major educational resources in internal medicine, family medicine, and infectious disease for their coverage of PG. CONTENT: For each specialty, we reviewed five major textbooks, five prominent journals, and any commonly used online resources. Twelve of 15 textbooks mentioned PG, only three of which included a differential, with none including NSTI in the differential. Only two of 13 journals included review articles about PG, and none of these including NSTI in their differential. Interestingly, online resources tended to be the most complete; six of nine contained PG articles, nearly all including a differential and three listing NSTI within it. SUMMARY: We found an underrepresentation of PG among major textbooks and journals in clinical specialties, especially in differentiating PG from its mimickers. While online resources may help fill this gap in knowledge, texts and journals remain essential. Misdiagnosis and resultant mismanagement of PG can lead to disastrous outcomes. OUTLOOK: We recommend that PG be added to the differential diagnoses of chronic ulcers in educational resources. We also suggest the addition of identification and differentiation of PG to learning materials and lectures for providers in specialties who may encounter PG, NSTI, or similarly presenting diseases to address this gap.

Pyoderma gangrenosum underrepresentation in non-dermatological literature.

BACKGROUND: Pyoderma gangrenosum (PG) is a chronic, ulcerative neutrophilic dermatosis. PG presents a diagnostic challenge, largely due to the many mimicking diseases, the lack of confirmatory laboratory or biological markers, and the absence of widely accepted diagnostic criteria. In particular, PG is often mistaken for necrotizing soft tissue infections (NSTI). METHODS: We reviewed four major textbooks each in general surgery, plastic surgery, trauma surgery, vascular surgery, emergency medicine, and dermatology. We also performed a search of review articles addressing NSTI and necrotizing fasciitis (NF). RESULTS: Ten out of the 20 non-dermatology textbooks did not list PG anywhere, and only two listed a differential diagnosis for PG. None of the non-dermatology textbooks indicated PG in the NSTI differential diagnosis, while three of the dermatology textbooks included PG in the NSTI differential diagnosis. PG was listed in all of the dermatology textbooks. Only one of the NSTI and NF articles mentioned PG in the differential diagnosis. CONCLUSIONS: There is an underrepresentation in major textbooks of surgery and emergency medicine and in NSTI and NF review articles when it comes to diagnosing PG. This might be leading to trainees and advanced providers in these fields being uninstructed on PG, and likely contributes to PG misdiagnosis and mismanagement. We recommend PG be included in the differential diagnosis of chronic ulcers and NSTI in non-dermatology textbooks. We also suggest adding identification and diagnosis of inflammatory mimickers of NSTI (e.g. PG) in teaching modules in surgical and emergency specialties to address this knowledge gap.