Diagnostic Criteria of Ulcerative Pyoderma Gangrenosum

Maverakis, Emanual; Ma, Chao; Shinkai, Kanade; Fiorentino, David; Callen, Jeffrey P.; Wollina, Uwe; Marzano, Angelo Valerio; Wallach, D.; Kim, Kyoungmi; Schadt, Courtney R.; Ormerod, Anthony; Fung, Maxwell A.; Steel, Andrea; Patel, Forum; Qin, Rosie; Craig, Fiona E.; Williams, Hywel C; Powell, Frank C.; Merleev, Alexander A.; Cheng, Michelle Y.

doi:10.1001/jamadermatol.2017.5980

Cited by 354 publications

(375 citation statements)

References 49 publications

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“…In addition, patients had two of the following five characteristics: history of pathergy, clinical evidence of cribriform scarring, systemic diseases associated with PG, compatible biopsy findings, and demonstrated responsiveness to immunosuppression. These criteria are consistent with prior proposed diagnostic criteria (13) as well as the most up-to-date diagnostic criteria (12). …”

Section: Methodssupporting

confidence: 85%

“…Diagnosis of PG was verified by more than one board-certified dermatologist based on clinical history, physical examination findings, and biopsy findings. Specifically, in all cases, the diagnosis of classic ulcerative PG was made and confirmed based on clinical evidence of rapidly progressing painful, necrolytic, and cutaneous ulcer(s) with an undermining violaceous border, as well as exclusion of other causes of cutaneous ulceration (12). In addition, patients had two of the following five characteristics: history of pathergy, clinical evidence of cribriform scarring, systemic diseases associated with PG, compatible biopsy findings, and demonstrated responsiveness to immunosuppression.…”

Section: Methodsmentioning

confidence: 99%

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Classic Ulcerative Pyoderma Gangrenosum Is a T Cell-Mediated Disease Targeting Follicular Adnexal Structures: A Hypothesis Based on Molecular and Clinicopathologic Studies

et al. 2018

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Background: Pyoderma gangrenosum (PG) is a debilitating ulcerative skin disease that is one of the most common associated diseases seen in patients with inflammatory bowel disease and rheumatoid arthritis. Although PG is classified as a neutrophilic dermatosis, its pathophysiology is poorly understood.objective: Use data obtained from patient-reported histories, immunohistochemistry, and gene expression analysis to formulate a hypothesis on PG pathophysiology.Methods: Ten PG patients participated and answered questions about new ulcer formation. Skin biopsies of healed prior ulcers and adjacent normal skin were obtained from four patients for immunohistochemistry. Scars from healthy patients and patients with discoid lupus were used as additional controls. New onset PG papules were analyzed using immunohistochemistry and gene expression analysis via quantitative real-time PCR.results: All PG patients reported that healed sites of previous ulceration are refractory to re-ulceration. Simultaneous biopsies of healed and uninvolved skin triggered ulceration only in the latter. On immunohistochemistry, healed PG scars showed complete loss of pilosebaceous units, which were present in normal skin, and to a lesser extent in control scars, and discoid scars. Early PG papules showed perivascular and peripilosebaceous T cell infiltrates, rather than neutrophils. These early inflammatory events were dominated by increased gene expression of CXCL9, CXCL10, CXCL11, IFNG, and transcription factors consistent with Th1 phenotype.Limitations: Small sample size was the main limitation. Conclusion:We put forth the hypothesis that PG is a T cell response resulting in the destruction of pilosebaceous units.

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Section: Methodssupporting

confidence: 85%

Section: Methodsmentioning

confidence: 99%

Classic Ulcerative Pyoderma Gangrenosum Is a T Cell-Mediated Disease Targeting Follicular Adnexal Structures: A Hypothesis Based on Molecular and Clinicopathologic Studies

et al. 2018

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“…By mid April, some of the lesions showed cribriform scarring while others developed overlying pustules. She met diagnostic criteria for PG based on recently proposed diagnostic model, with neutrophilic infiltrate on biopsy, rapidly progressing ulcers with peripheral edema, undermining border, and tenderness, no clear infectious cause, response to intralesional corticosteroid injections, and a history suggestive of pathergy – ulcers located in the central and lateral anterior abdomen near dulaglutide injection sites . An extensive work‐up for malignancy and inflammatory bowel disease, including pan‐intestinal visualization, was negative despite highly elevated anti‐saccharomyces cerevisiae titers.…”

Section: Report Of a Casementioning

confidence: 99%

Pyoderma gangrenosum associated with dulaglutide therapy

2018

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“…The microbiologic culture identified Citrobacter koseri.Duplex sonography, abdominal sonography, and chest X-ray remained unremarkable.Histopathology from an ulcer margin demonstrated a chronic ulceration with involvement of deep subcutaneous adipose tissue, a combination of small vessel vasculitis capillary proliferations, and neutrophilic infiltrate(Figure 2).Diagnostic criteria for ulcerative pyoderma gangrenosum were fulfilled(Maverakis et al, 2018; Table 1). She was treated with secukinumab for about 12 months and achieved a PASI 100.…”

mentioning

confidence: 99%