Successful treatment with ustekinumab and vacuum-assisted closure therapy in recalcitrant myelodysplastic syndrome-associated pyoderma gangrenosum: case report and literature review

Nieto, D.; Sendagorta, Elena; Rueda, José M.; Pinto, Pedro Herranz

doi:10.1111/ced.13679

Cited by 17 publications

(17 citation statements)

References 10 publications

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“…The positive clinical response documented here is congruent with a growing number of cases also demonstrating improvements in recalcitrant PG with the use of ustekinumab. 5–7 Therefore, this case further supports the use of ustekinumab in therapy for PG and also draws attention to the probable contribution of IL-23 in disease pathogenesis.…”

Section: Discussionsupporting

confidence: 69%

“…4 Correspondingly, cases have emerged suggesting ustekinumab, a humanized monoclonal antibody targeting the shared p40 subunit of IL-12 and IL-23, may hold promise for inducing remission of refractory PG. 5–7 Here, we, adding to this body of literature, demonstrate the use of ustekinumab to treat PG that developed in a medically complex patient already receiving systemic immunosuppressive therapy.…”

Section: Introductionmentioning

confidence: 80%

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Ustekinumab for the treatment of recalcitrant pyoderma gangrenosum: A case report

Vallerand

Hardin

2019

SAGE Open Medical Case Reports

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Pyoderma gangrenosum is an ulcerating disease associated with a high degree of morbidity and mortality. Currently, little is known about the pathophysiology of pyoderma gangrenosum, though it has been linked to increased levels of inflammatory cytokines including interleukin-23. As pyoderma gangrenosum is a rare disease, evidence for pyoderma gangrenosum treatment is dependent on reporting of cases with successful therapies. Here, we describe a case of pyoderma gangrenosum developing on the lateral leg of a medically complex 47-year-old male already on chronic immunosuppressive therapy, who achieved successful wound healing with the use of ustekinumab, a monoclonal antibody targeting inhibition of interleukin-12 and interleukin-23. This case lends further evidence for the role of interleukin-23 in the pathogenesis of recalcitrant pyoderma gangrenosum and also suggests that healthcare providers may consider a trial of ustekinumab in pyoderma gangrenosum that has failed previous topical treatments or systemic immunosuppression.

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Section: Discussionsupporting

confidence: 69%

Section: Introductionmentioning

confidence: 80%

Ustekinumab for the treatment of recalcitrant pyoderma gangrenosum: A case report

Vallerand

Hardin

2019

SAGE Open Medical Case Reports

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“…When the disease is mild or the lesions are superficial, topical medications could be used as monotherapy 15 . Otherwise, oral GCSs (0.5‐1 mg/kg/day) remain the first‐line treatment in the majority of cases 9,16 . In our case series oral GCSs were very efficacious in various types of PG (pustular, vegetative, bullous, ulcerative).…”

Section: Discussionmentioning

confidence: 70%

“…New evidences showed that surgical approaches with NPWT and STSG are safe treatment options if performed under adequate immunosuppressive therapy 25 . NPWT associated with ustekinumab was shown to be successful in a PG‐associated myelodysplastic syndrome 16 …”

Section: Discussionmentioning

confidence: 99%

PG‐TIME : A practical approach to the clinical management of pyoderma gangrenosum

Janowska

Oranges

Fissi

et al. 2020

Dermatologic Therapy

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Pyoderma gangrenosum (PG) is a neutrophilic dermatosis which may be rapidly progressive. Standard guidelines for local treatment are lacking. Through our experience, we suggested a local treatment algorithm based on the tissue, infection, moisture balance, and epithelization (TIME) concept. The clinical and histopathological features of 52 patients with PG, the duration, and the evolution of the lesions were retrospectively evaluated. Systemic therapies, local treatments, and standard wound treatments were reported. We observed ulcerative PG in the majority of the patients (82.6%), followed by the pustular form (9.6%), the peristomal type (3.8%), the vegetative form (1.9%), and the bullous type (1.9%). The lower leg was the most commonly area affected (90.4% of cases). Pathergy was reported in 15.3% of cases. The first‐line treatment was the use of oral glucocorticosteroids (GCSs). We observed healing after 3 weeks of GCS in 17.3% of cases. In 25% of all cases, we obtained complete healing with long‐term low doses (<0.5 mg/kg) of GCS in the range of 2 to 6 months. We used second‐line treatments in 57.6% of patients. Local, systemic treatment, and correct wound management can be associated with better clinical results. We suggest a new local therapeutic algorithm in both the inflammatory and noninflammatory healing phases.

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“…In a systematic review published in 2015 including 823 cases of PG, HMs have been found to be associated with this condition in 12.5% of patients, following inflammatory bowel diseases ( n = 537; 65.2%) and polyarthritis ( n = 133; 16.1%) [ 66 ]. Haematological malignancies associated with PG, which are a major cause of death in this disease [ 41 , 67 ], are mainly represented by acute and chronic myeloid leukaemia [ 68 , 69 ], myelodysplastic syndrome [ 70 ], multiple myeloma [ 71 ], monoclonal gammopathy of undetermined significance [ 72 ] and lymphoma [ 73 ]. Although IgG isotype gammopathies are overall more frequent, most monoclonal gammopathies associated with PG are of the IgA isotype [ 47 , 74 ].…”

Section: Neutrophilic Dermatosesmentioning

confidence: 99%

Immune-Mediated Dermatoses in Patients with Haematological Malignancies: A Comprehensive Review

et al. 2020

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Haematological malignancies induce important alterations of the immune system, which account for the high frequency of autoimmune complications observed in patients. Cutaneous immune-mediated diseases associated with haematological malignancies encompass a heterogeneous group of dermatoses, including, among others, neutrophilic and eosinophilic dermatoses, autoantibody-mediated skin diseases, vasculitis and granulomatous dermatoses. Some of these diseases, such as paraneoplastic pemphigus, are associated with an increased risk of death; others, such as eosinophilic dermatoses of haematological malignancies, run a benign clinical course but portend a significant negative impairment on a patient's quality of life. In rare cases, the skin eruption reflects immunological alterations associated with an unfavourable prognosis of the associated haematological disorder. Therapeutic management of immune-mediated skin diseases in patients with haematological malignancies is often challenging. Systemic corticosteroids and immunosuppressive drugs are considered frontline therapies but may considerably augment the risk of serious infections. Indeed, developing a specific targeted therapeutic approach is of crucial importance for this particularly fragile patient population. This review provides an up-to-date overview on the immune-mediated skin diseases most frequently encountered by patients with onco-haematological disorders, discussing new pathogenic advances and therapeutic options on the horizon.

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Successful treatment with ustekinumab and vacuum-assisted closure therapy in recalcitrant myelodysplastic syndrome-associated pyoderma gangrenosum: case report and literature review

Abstract: Click https://www.wileyhealthlearning.com/ced.aspx for the corresponding questions to this CME article.

Cited by 17 publications

References 10 publications

Ustekinumab for the treatment of recalcitrant pyoderma gangrenosum: A case report

Ustekinumab for the treatment of recalcitrant pyoderma gangrenosum: A case report

PG‐TIME : A practical approach to the clinical management of pyoderma gangrenosum

Immune-Mediated Dermatoses in Patients with Haematological Malignancies: A Comprehensive Review

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