A case of extracutaneous pyoderma gangrenosum in a patient with persistent cutaneous and systemic symptoms: Implications for differential diagnosis and treatment

Lucia, Gabriella Santa; DeMaio, Alexa; Karlin, Samantha; Elston, Dirk M.

doi:10.1016/j.jdcr.2021.07.019

Cited by 4 publications

(5 citation statements)

References 10 publications

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“…She also had numerous abscesses in the lungs and in the pericardial, hepatic, splenic, and pancreatic regions. The lesions resolved after the use of prednisone, azathioprine, and canakinumab [ 8 ].…”

Section: Discussionmentioning

confidence: 99%

“…In our viewpoint, the results indicate physiological transient autoimmunity as a defensive function of the immune system, the aim of which is to eliminate apoptotic residues during the breakdown of neutrophils [ 14 , 15 ]. However, as regards other described cases, apart from high inflammatory index rates, anti-neutrophil cytoplasmic antibodies (ANCA) were negative [ 6 , 8 ]. Considering the above-mentioned immunological test results, the patient is still monitored for the possible development of (ANCA)-associated vasculitis or connective tissue diseases.…”

Section: Discussionmentioning

confidence: 99%

“…It is advisable to include other immunosuppressants (azathioprine, cyclophosphamide, methotrexate, cyclosporine) or anti-TNF-alpha agents (adalimumab, infliximab) to enhance treatment or reduce corticosteroid doses [ 1 , 4 ]. Some research described the cases of the efficacy of anakinra, an IL-1 receptor antagonist; canakinumab, an IL-1β antagonist; and colchicine [ 1 , 6 , 8 ]. Treatment involving splenectomy did not meet expectations because a relapse usually occurred after surgery [ 1 ].…”

Section: Discussionmentioning

confidence: 99%

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Aseptic Abscess Syndrome in Rheumatoid Arthritis Patient

Owczarczyk‐Saczonek

Kasprowicz-Furmańczyk

Kuna

et al. 2022

Medicina

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Aseptic abscess syndrome (AAS) is a rare, potentially life-threatening disorder, with numerous features of neutrophilic dermatoses. The main symptoms include aseptic abscess-like collections in internal organs (spleen, liver, lungs), lack of microbes (bacteria, viruses, or parasites) after an exhaustive search, ineffectiveness of antibiotics, and high sensitivity to corticosteroid therapy. AAS is characterized by the development of deep, inflammatory abscesses and systemic symptoms (weight loss, abdominal pain, fever, and leukocytosis). They may be associated with inflammatory bowel disease (IBD) and autoimmune diseases. The patient in this study is a 67-year-old man, suffering from rheumatoid arthritis (RA), with numerous purulent abscesses in the mediastinum, within the subcutaneous tissue above the extension surfaces of the joints, and on the dorsum of the hands. The lesions are accompanied by bone destruction. The patient was treated with prednisone 40 mg and adalimumab, which resulted in a quick reduction of inflammatory markers and clinical improvement, as well as the healing and absorption of abscesses. Despite COVID-19 infection, treatment with remdesivir, prednisone, and adalimumab was continued, with the complete resolution of the lesions. AAS is difficult to recognize, so practitioners have to be aware of this condition, especially in patients with RA.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Aseptic Abscess Syndrome in Rheumatoid Arthritis Patient

Owczarczyk‐Saczonek

Kasprowicz-Furmańczyk

Kuna

et al. 2022

Medicina

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“…Extracutaneous lesions tend to be misdiagnosed for an infectious cause, as the infiltrates are not temporally related to typical cutaneous lesions of PG. A case study reported by Santa Lucia et al examined a 56-year-old woman already diagnosed with cutaneous PG maintained on immunosuppressive therapy, returning to the hospital multiple times with pulmonary, pericardial, hepatic, splenic, and pancreatic involvement [ 37 ]. Suspicion of infection directed the internal medicine team to discontinue immunosuppressants and start the patient on broad-spectrum antibiotics and antifungals.…”

Section: Reviewmentioning

confidence: 99%

Narrative Review: Pyoderma Gangrenosum

Park,

Raj,

Bajda

et al. 2024

Cureus

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Pyoderma gangrenosum (PG) is a skin lesion, characteristically a neutrophilic dermatosis, that can be complicated by rapid progression, necrosis, and ulceration. This is an important pathology to be discussed given that there are no established criteria for diagnosis or treatment. This review aims to elucidate characteristics and variations of PG that distinguish it from other ulcerative skin lesions. Variability in presentation can lead to missed or incorrect diagnosis, and some of the currently proposed criteria for categorizing and diagnosing PG have been included here. These criteria distinguish PG in terms of the nature of the lesion, the location, etiology, responsiveness to immunosuppressive therapy, and patient history. The etiology and pathogenesis of PG remain unknown, but we summarize prominent theories and explanations. Furthermore, recent research indicates that the incidence of PG has a strong correlation with autoimmune conditions, particularly inflammatory bowel disease. Major treatments for PG coincide with these findings, as the majority involve targeted anti-inflammatories, immunosuppressants, and surgical interventions. These treatments are addressed in this review, with added context for local versus systemic disease.

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“…Given the rarity of systemic PG, the evidence base for treatment options is derived from case reports. [2][3][4][5] Successful splenic PG treatments include monotherapy high-dose corticosteroids (n = 3), 2 combined corticosteroid and dapsone (n = 2), 2 combined corticosteroid and cyclosporin (n = 5), 2,5 infliximab (n = 1), 3 adalimumab 3 (n = 2) 3,4 or combined corticosteroid, azathioprine and canakinumab (n = 1). 5 This case of systemic PG responding well to monotherapy corticosteroids highlights the value of a diagnostic and therapeutic trial of such treatment in patients with a history of PG and unusual aseptic systemic collections.…”

mentioning

confidence: 99%