Idiopathic Pyoderma Gangrenosum: Successful Resolution with Infliximab Therapy and Pro-inflammatory Cytokines Assessment

Abstract

Pyoderma gangrenosum (PG) is a rare neutrophilic dermatosis\ud of unknown aetiology, characterized by primarily\ud sterile, painful ulcers with typical violaceous undermined\ud borders and mucopurulent or haemorrhagic exudate. PG is\ud commonly associated with other systemic disorders. Diagnosis\ud of primary idiopathic forms is reached following the\ud exclusion of inflammatory bowel disease (IBD), rheumatic\ud disorders, monoclonal gammopathy and solid tumours\ud (1, 2). Treatment is generally based on corticosteroid and\ud immunosuppressive therapy; however, the recent use of\ud biological drugs has shown their effectiveness

Pyoderma gangrenosum and tumour necrosis factor alpha inhibitors: A semi‐systematic review

“…A total of 3212 unique citations were found. 1286 and 1704 citations were excluded by abstract and full‐text read, respectively, yielding 222 articles…”

Pyoderma gangrenosum: A systematic review of the molecular characteristics of disease

“…IL‐8 (also known as CXCL8) 13,15–19 and TNF‐a 13,15,16,18–23 were consistently found to be overexpressed in the wound bed of PG lesions, with the latter also being elevated in the serum of PG patients (Table 2). IL‐8 is a potent chemoattractant for neutrophils and, along with inflammatory effects of TNF‐a, may contribute to an ongoing neutrophil presence in PG tissue.…”

“…TNF‐a antagonism through infliximab or etanercept has also proven effective, although the time to complete resolution appears longer when compared to IL‐23 or IL‐17R inhibition, with complete healing occurring by 12 months 23,44 . A larger study examining adalimumab in 22 patients with PG demonstrated an average change of ulcer size of −63.8% from baseline to week 26, and a mean target healing time of 114.9 days.…”

“…Of the 236 patients, 59 had a recorded associated underlying inflammatory disease, with 30 of these being inflammatory bowel disease (50.85%), 7 rheumatoid arthritis (11.86%), 4 cystic fibrosis (6.78%), 10 with haematological abnormalities (16.9%), 2 hidradenitis suppurativa, 1 SLE, 1 autoimmune enteropathy, 1 IgA nephropathy, 1 autoimmune cholangiopathy, 1 vasculitis and 1 with a lung carcinoid tumour. Twenty-seven patients were recorded to have PG in association with a known syndrome; 16 of these were PASH syndrome, 5 PAPA syndrome, 3 with IL-8 (also known as CXCL8) 13,[15][16][17][18][19] and TNF-a 13,15,16,[18][19][20][21][22][23] were consistently found to be overexpressed in the wound bed of PG lesions, with the latter also being elevated in the serum of PG patients (Table 2). IL-8 is a potent chemoattractant for neutrophils and, along with inflammatory effects of TNF-a, may contribute to an ongoing neutrophil presence in PG tissue.…”

Recurrent Pyoderma Gangrenosum and Cystic Acne Associated with Leucocyte Adhesion Deficiency due to Novel Mutations in ITGB2: Successful Treatment with Infliximab and Adalimumab

“…Our treatment experience points to infliximab, adalimumab as the most valuable remedies for this disease. Inflixi- mab has been previously described for idiosyncratic PG (14). Any effect of high-dose IVIG therapy (15) was unnoticeable in our patient after 6 weeks.…”