The dilemma of treating pyoderma gangrenosum associated with monoclonal gammopathy of undetermined significance

Machan, Achraf; Azendour, Hasna; Frikh, Rachid; Hjira, Naoufal; Boui, Mohammed

doi:10.5070/d3265048791

Cited by 5 publications

(3 citation statements)

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“…This could potentially reflect a hesitancy for physicians to treat PG in these patients more aggressively due to concern for cancerous conversion leading to PG progression and recurrence. 11 While the risks and benefits for each individual patient may alter treatment protocols, earlier, more aggressive PG treatment in patients with a history of MGUS might prevent PG progression thereby decreasing overall morbidity. It is also possible the increased relative incidence of MGUS among patients presented in this review points to yet undiscovered changes to the immune system with MGUS which predispose patients to PG.…”

Section: Discussionmentioning

confidence: 99%

Recalcitrant Pyoderma Gangrenosum: Clinical Burden and Unmet Needs

Becker¹,

Velasco²,

Ortega‐Loayza³

2023

CCID

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Pyoderma gangrenosum (PG) is a rare, autoinflammatory disease leading to aseptic ulcers which carries a significant disease burden and is often difficult to treat, with many patients failing first-line treatment and requiring additional therapies. Such cases are typically referred to in the literature as "recalcitrant", "refractory", or "resistant", though little is known about the clinical characteristics of such cases. We performed a narrative literature review to characterize patient demographics and clinical course associated with difficult to treat pyoderma gangrenosum cases in order to identify trends to guide future clinical management and therapeutic innovation. We identified 148 cases with clinical manifestations and associated patient demographics stratified by ulcer and patient features. Consistent with previous work, a greater prevalence of PG was observed among female patients and those with a history of inflammatory bowel disease, however interestingly despite an aggressive course to their PG, few patients had comorbidities complicating their disease course. Additionally, despite the requirement of three or more treatments for most patients' disease to resolve, the majority healed within the typical window observed in previous clinical studies with low rates of recurrence. Biologics were the most common medication patients were on at time of remission. Collectively, our results suggest a potential benefit for a reduced threshold for biologic initiation in PG patients and a need for standardization of language in the field to facilitate treatment outcomes comparisons and interventions.

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Section: Discussionmentioning

confidence: 99%

Recalcitrant Pyoderma Gangrenosum: Clinical Burden and Unmet Needs

Becker¹,

Velasco²,

Ortega‐Loayza³

2023

CCID

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“…Strikingly, a complete response was achieved in just a few weeks since initiation, paralleling the resolution of MGUS (76). Indeed, an aggressive management of HM aimed at the resolution of concomitant IgA MGUS has been advocated for to achieve PG remission (77).…”

Section: Dermal/hypodermal 231 Pyoderma Gangrenosum (Level Of Evidenc...mentioning

confidence: 99%

Neutrophilic and eosinophilic dermatoses associated with hematological malignancy

Maronese,

Derlino,

Moltrasio

et al. 2024

Front. Med.

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Cutaneous manifestations of hematologic malignancy represent both a clinical challenge for the treating physician and a pathophysiological model for advancing the knowledge on individual neoplasms. Indeed, a growing body of evidence supports the concept of recurrent molecular defects associating with specific clinical features, as best exemplified by VEXAS. Herein neutrophilic and eosinophilic dermatoses of potential interest for both hematologists and dermatologists will be reviewed, including subcorneal pustular dermatosis-type IgA pemphigus, neutrophilic eccrine hidradenitis, Sweet’s syndrome as well as myelodysplasia cutis and VEXAS, pyoderma gangrenosum, eosinophilic annular erythema, eosinophilic dermatosis of hematological malignancy, Wells syndrome and cutaneous involvement in hypereosinophilic syndromes. Possible management approaches are discussed for each, emphasizing scenarios that require treatment of the underlying condition to achieve remission at the skin level.

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“…Tumor necrosis factor inhibitors, such as infliximab, have been studied in other inflammatory diseases with good response and may be an option to consider. Other steroid-sparing drugs, such as oral dapsone, cyclosporine A, mycophenolate, and tacrolimus, have been tried [37,39].…”

Section: Pyoderma Gangrenosummentioning

confidence: 99%

Treatment of Patients with Monoclonal Gammopathy of Clinical Significance

Moreno

Rosiñol

Cibeira

et al. 2021

Cancers

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Monoclonal gammopathy of undetermined significance (MGUS) is defined as the presence of a monoclonal protein (M-protein) produced by a small amount of plasma cells. The majority of patients remain asymptomatic; however, a fraction of them develop clinical manifestations related to the monoclonal gammopathy despite not fulfilling criteria of multiple myeloma or other lymphoproliferative disorder. These patients constitute an emerging clinical issue coined as monoclonal gammopathy of clinical significance (MGCS). The mechanisms involved are poorly understood, and literature is scarce regarding management. The clinical spectrum involves symptoms related to renal, neurologic, skin, ocular, or bleeding manifestations, requiring a multidisciplinary approach. Treatment strategies rely on the basis of symptomatic disease and the M-protein isotype. In this review, we focus on MGCS other than renal, as the latter was earliest recognized and better known. We review the literature and discuss management from diagnosis to treatment based on illustrative cases from daily practice.

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The dilemma of treating pyoderma gangrenosum associated with monoclonal gammopathy of undetermined significance

Cited by 5 publications

References 0 publications

Recalcitrant Pyoderma Gangrenosum: Clinical Burden and Unmet Needs

Recalcitrant Pyoderma Gangrenosum: Clinical Burden and Unmet Needs

Neutrophilic and eosinophilic dermatoses associated with hematological malignancy

Treatment of Patients with Monoclonal Gammopathy of Clinical Significance

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