Pyoderma Gangrenosum as a Presenting Feature of Undifferentiated Spondyloarthropathy with Erosive Inflammatory Arthritis

Pyoderma gangrenosum is a rare inflammatory condition with varying clinical presentations and severity. It is commonly seen in association with an underlying condition, most common of which is inflammatory bowel disease. We report a case of a 26-year-old male who came to the emergency department with increasing lower extremity ulcers, intermittent hematochezia, and pain in the small joints of his hands. After excluding a broad list of differentials for lower extremity ulcers, the diagnosis of pyoderma gangrenosum was made. He was also found to have erosive changes at multiple proximal interphalangeal joints and jug-like syndesmophytes at T12 and L1 on CT scan. Although there was evidence of a spondyloarthropathy, there was no evidence of inflammatory bowel disease on colonoscopy, psoriasis, or sexually transmitted infections. After multiple failed trials of medications including azathioprine and sulfasalazine, 4 weeks of Adalimumab resulted in rapid healing of pyoderma gangrenosum lesions and improvement in his synovitis. Coupled together, this suggests a diagnosis of pyoderma gangrenosum associated with undifferentiated spondyloarthropathy and erosive inflammatory arthritis. This case is suggestive of spondyloarthropathy going underdiagnosed and untreated in other patients with pyoderma gangrenosum as lower extremity ulcerations can be the primary complaint for seeking treatment. Although rare, axial spondyloarthropathy associated with pyoderma gangrenosum should be kept as an associated differential diagnosis when faced with pyoderma gangrenosum.

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Pyoderma Gangrenosum as the Initial Presentation of Inflammatory Bowel Disease

American Journal of Clinical Pathology ◽

10.1093/ajcp/aqaa161.109 ◽

2020 ◽

Vol 154 (Supplement_1) ◽

pp. S51-S51

Author(s):

S S Karimi ◽

G Guzman

Keyword(s):

Inflammatory Bowel Disease ◽

Lower Extremity ◽

Pyoderma Gangrenosum ◽

Crohn Disease ◽

Lupus Erythematosus ◽

Bowel Disease ◽

Bacterial Colonization ◽

Neutrophilic Dermatosis ◽

Tissue Destruction ◽

Inflammatory Bowel

Abstract Introduction/Objective Pyoderma gangrenosum is a neutrophilic dermatosis, commonly associated with arthritis, psoriasis, and systemic lupus erythematosus. It is also an aggressive manifestation of extra-intestinal inflammatory bowel disease affecting less than 5% of patients with ulcerative colitis and Crohn disease. We present a case of pyoderma gangrenosum as an initial and rapidly progressive manifestation of Crohn disease. Methods A 35-year-old man presented with fecal urgency, periumbilical pain, and frequent diarrhea, with stool cultures revealing no infectious etiology. Colonoscopy demonstrated active inflammatory bowel disease consistent with Crohn disease. Concurrently, patient developed an ulcer on right lower extremity not otherwise contributed to history of trauma or injury. Biopsy of right lower extremity ulcer revealed histopathologic findings consistent with pyoderma gangrenosum. Despite medical management, and prolonged use of wound care and wound vac, patient’s non-healing ulcer continued to geographically expand, resulting in ankle contracture. A right below the knee amputation was performed and amputation specimen was sent to pathology for further diagnostic evaluation. Results Gross examination revealed an extensive cutaneous ulcer measuring 22.1cm x 11.7cm x 0.3cm and involving the anterior medial aspect of the right lower extremity with medial and posterior extension down to the dorsum of the foot with broad deep tissue destruction and exposure of fascia and tendon. Microscopic examination revealed severe ulceration, suppurative necrosis, superficial and deep vasculitis confined to the ulcer bed. Surrounding soft tissue revealed chronic myopathic changes secondary to ischemia. Foci of commensal filamentous gram-positive bacterial colonization in a nidus of necrosis were also identified. The inflammatory pattern involved predominantly neutrophils with weak recruitment of other inflammatory cells, consistent with pyoderma gangrenosum. Conclusion This case highlights the debilitating extent of Crohn disease, its detrimental effects on the patient’s quality of life, and the diagnostic and treatment challenges it poses to clinicians in managing Crohn disease and its complications.

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A156 SERUM TUMOUR NECROSIS FACTOR-α ANTAGONIST DRUG CONCENTRATIONS IN PATIENTS WITH PYODERMA GANGRENOSUM ASSOSCIATED WITH INFLAMMATORY BOWEL DISEASE

Journal of the Canadian Association of Gastroenterology ◽

10.1093/jcag/gwab002.154 ◽

2021 ◽

Vol 4 (Supplement_1) ◽

pp. 162-163

Author(s):

M Mikail ◽

A Wilson

Keyword(s):

Inflammatory Bowel Disease ◽

Pyoderma Gangrenosum ◽

Bowel Disease ◽

Complete Resolution ◽

Therapeutic Drug ◽

Drug Concentrations ◽

Median Serum ◽

Inflammatory Bowel ◽

Factor Α ◽

Necrosis Factor

Abstract Background The utility of therapeutic drug monitoring for guiding the dosing of tumor necrosis factor-α antagonists (TNFAs) in luminal inflammatory bowel disease (IBD) is well-established and well-accepted. TNFAs, specifically infliximab and adalimumab, have become integral to the management of the rare, neutrophilic dermatosis, pyoderma gangrenosum (PG) in IBD. Little is known regarding the target serum TNFA concentrations to guide dosing to achieve resolution of PG in IBD. Aims To describe the serum TNFA concentrations (infliximab or adalimumab) associated with the resolution of PG lesions in patients with IBD. Methods Patients with IBD and associated PG treated with one of infliximab or adalimumab (collectively known as TNFAs) seen at two academic hospitals affiliated with Western University were identified. Serum TNFA concentrations were assessed at the time of PG treatment. Results Nine patients were identified. All patients had IBD-associated PG. Seven patients were treated with infliximab and 2 patients were treated with adalimumab. All patients received standard dosing. Eight patients had complete resolution of their PG, while one had near complete resolution at the time of last follow-up. A median serum infliximab concentration of 3.00 (IQR, 3.52) µg/ml at week 14 and a median serum adalimumab concentration of 2.02 (IQR, 0.98) µg/ml at week 12 were seen at the time of PG treatment. Conclusions Herein, we report low serum TNFA concentrations despite PG healing in a cohort of IBD patients. This is lower than what is in patients for successful TNFA treatment in luminal and fistulising IBD. Funding Agencies NoneNone.

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Intravenous Cyclosporine in Refractory Pyoderma Gangrenosum Complicating Inflammatory Bowel Disease

Inflammatory Bowel Diseases ◽

10.1097/00054725-200102000-00001 ◽

2001 ◽

Vol 7 (1) ◽

pp. 1-7 ◽

Cited By ~ 47

Author(s):

Sonia Friedman ◽

James F. Marion ◽

Ellen Scherl ◽

Peter H. Rubin ◽

Daniel H. Present

Keyword(s):

Inflammatory Bowel Disease ◽

Pyoderma Gangrenosum ◽

Bowel Disease ◽

Inflammatory Bowel

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Mouse model of ulcerative colitis using trinitrobenzene sulfonic acid

Bangladesh Journal of Pharmacology ◽

10.3329/bjp.v10i4.25351 ◽

2015 ◽

Vol 10 (4) ◽

pp. 860

Author(s):

Irfan Ahmad Rather ◽

Vivek K. Bajpai ◽

Nam Gyeong-Jun

Keyword(s):

Ulcerative Colitis ◽

Inflammatory Bowel Disease ◽

Mouse Model ◽

Bowel Disease ◽

Sulfonic Acid ◽

Intestinal Inflammation ◽

Cost Effective ◽

Trinitrobenzene Sulfonic Acid ◽

Clinical Presentations ◽

Inflammatory Bowel

Animal model of intestinal inflammation is of paramount significance that aids in discerning the pathologies underlying ulcerative colitis and Crohn’s disease, the two clinical presentations of inflammatory bowel disease. The 2,4,6-trinitrobenzene sulfonic acid (TNBS) colitis model represents one such intestinal inflammation-prototype that is generated in susceptible strains of mice through intra-rectal instillation of compound TNBS. In this paper, we demonstrate the experimental induction of TNBS-mediated colitis in a susceptible strain of ICR mice. This can be done by the following steps: a) acclimation, b) induction and c) observation. TNBS-mouse model provides the information in shortest possible time and simultaneously represents a cost effective and highly reproducible model method of studying the pathogenesis of inflammatory bowel disease.Video Clips<a href="https://youtube.com/v/6MsuIGzH3uA">Acclimation and induction of TNBS</a>: 4.5 min<a href="https://youtube.com/v/ya66SNwoVag">Observation and drug administration</a>: 1.5 min

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Pyoderma Gangrenosum in a Patient with Essential Thrombocythemia

Journal of Cutaneous Medicine and Surgery ◽

10.1177/120347540000400213 ◽

2000 ◽

Vol 4 (2) ◽

pp. 107-109 ◽

Cited By ~ 5

Author(s):

Kim W. King ◽

Alexander Murray

Keyword(s):

Inflammatory Bowel Disease ◽

Pyoderma Gangrenosum ◽

Essential Thrombocythemia ◽

Bowel Disease ◽

Hematologic Disease ◽

Inflammatory Bowel

Background: Pyoderma gangrenosum is an uncommon ulcerative condition associated with inflammatory bowel disease, arthritis, and hematologic disease. We report a patient with essential thrombocythemia and pyoderma gangrenosum. Objective: This article is a review of the associations between pyoderma gangrenosum and other diseases. Results: There have been two previous reports of patients with pyoderma gangrenosum and essential thrombocythemia. Conclusion: There may be a possible association between pyoderma gangrenosum and essential thrombocythemia. The diagnosis of pyoderma gangrenosum should be considered in patients with essential thrombocythemia and cutaneous ulcers.

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