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Cureus Apr 2024While Pyoderma gangrenosum (PG) is commonly associated with hematological disorders such as acute myeloid leukemia (AML), it typically presents concurrently with the...
While Pyoderma gangrenosum (PG) is commonly associated with hematological disorders such as acute myeloid leukemia (AML), it typically presents concurrently with the hemopathy, mostly in its bullous form, among middle-aged individuals. Here, we report the unusual case of a young female patient who presented with PG in its ulcerative form, three weeks before the onset of AML. A 31-year-old female presented with a one-week history of painful perianal papulopustule that evolved into an irregular ulceration with violaceous borders, mucopurulent serosity, and erythematous surrounding skin. Laboratory work-up demonstrated elevated inflammatory markers and hyperleukocytosis, with no cytopenia, and normal peripheral blood smear. Two weeks later, the ulcer growth was noted with a similar ulceration at a venipuncture site. A complete blood count revealed pancytopenia, with 45% blasts on the peripheral blood smear. Skin biopsies showed an aseptic neutrophilic infiltrate in favor of PG. Intravenous methylprednisolone was administered with rapid resolution of the lesions. However, the patient died shortly after. The post-mortem results of bone marrow aspirate revealed AML, with immunohistochemistry of the skin lesions confirming the clonality of neutrophils derived from the leukemic clone. This case highlights a distinctive clinical presentation, illustrating the manifestation of PG three weeks before the onset of AML in its ulcerative rather than bullous form, in a young female patient.
PubMed: 38784334
DOI: 10.7759/cureus.58838 -
The Australasian Journal of Dermatology May 2024
PubMed: 38783769
DOI: 10.1111/ajd.14310 -
Journal of the European Academy of... May 2024The interleukin (IL)-1 superfamily upregulates immune responses and maintains homeostasis between the innate and adaptive immune systems. Within the IL-1 superfamily,... (Review)
Review
The interleukin (IL)-1 superfamily upregulates immune responses and maintains homeostasis between the innate and adaptive immune systems. Within the IL-1 superfamily, IL-36 plays a pivotal role in both innate and adaptive immune responses. Of the four IL-36 isoforms, three have agonist activity (IL-36α, IL-36β, IL-36γ) and the fourth has antagonist activity (IL-36 receptor antagonist [IL-36Ra]). All IL-36 isoforms bind to the IL-36 receptor (IL-36R). Binding of IL-36α/β/γ to the IL-36R recruits the IL-1 receptor accessory protein (IL-1RAcP) and activates downstream signalling pathways mediated by nuclear transcription factor kappa B and mitogen-activated protein kinase signalling pathways. Antagonist binding of IL-36Ra to IL-36R inhibits recruitment of IL-1RAcP, blocking downstream signalling pathways. Changes in the balance within the IL-36 cytokine family can lead to uncontrolled inflammatory responses throughout the body. As such, IL-36 has been implicated in numerous inflammatory diseases, notably a type of pustular psoriasis called generalized pustular psoriasis (GPP), a chronic, rare, potentially life-threatening, multisystemic skin disease characterised by recurrent fever and extensive sterile pustules. In GPP, IL-36 is central to disease pathogenesis, and the prevention of IL-36-mediated signalling can improve clinical outcomes. In this review, we summarize the literature describing the biological functions of the IL-36 pathway. We also consider the evidence for uncontrolled activation of the IL-36 pathway in a wide range of skin (e.g., plaque psoriasis, pustular psoriasis, hidradenitis suppurativa, acne, Netherton syndrome, atopic dermatitis and pyoderma gangrenosum), lung (e.g., idiopathic pulmonary fibrosis), gut (e.g., intestinal fibrosis, inflammatory bowel disease and Hirschsprung's disease), kidney (e.g., renal tubulointerstitial lesions) and infectious diseases caused by a variety of pathogens (e.g., COVID-19; Mycobacterium tuberculosis, Pseudomonas aeruginosa, Streptococcus pneumoniae infections), as well as in cancer. We also consider how targeting the IL-36 signalling pathway could be used in treating inflammatory disease states.
PubMed: 38779986
DOI: 10.1111/jdv.19935 -
Heliyon May 2024Pyoderma gangrenosum (PG) is an extremely rare case of sterile necrotic ulcerative disease associated with malnutrition as a predisposition factor. It is unclear,...
Pyoderma gangrenosum (PG) is an extremely rare case of sterile necrotic ulcerative disease associated with malnutrition as a predisposition factor. It is unclear, though, whether dilated cardiomyopathy, which affects blood flow and results in stenosis in the arteries, could play a role as an etiology. In this study, a case of pyoderma gangrenosum in a 10-year-old boy complicated by dilated cardiomyopathy, a previous history of cerebrovascular disease, and a malnourished condition were reported. The patient was reported to have exudative necrotic lesions in both legs. Lesions began as small, multiple, itchy lesions on both legs, which later became blisters and scuffed, and progressed into painful, peeled-off lesions with pus, bleeding, redness around lesions, and maggots within a month. A high fever was an accompanying symptom. The multidisciplinary team was involved to provide a comprehensive treatment for this patient. Antibiotics and necrotomy debridement were performed several times. Anticoagulant treatment was indicated as the coagulation markers were increased and echocardiography suggested thrombus in the left ventricle. The underlying condition that increases the risk of pyoderma gangrenosum should be corrected. The patient was discharged after a clinical improvement, although the continuation of outpatient monitoring was required. Our report suggests that a chronic condition of dilated cardiomyopathy that affects normal blood flow leads to malnutrition, the formation of thrombus, and stenosis of a peripheral artery, all of which contributed to pyoderma gangrenosum. Therefore, early surgical treatment, antibiotic administration, and anticoagulant treatment were recommended.
PubMed: 38778983
DOI: 10.1016/j.heliyon.2024.e30838 -
Dermatology Online Journal Mar 2024Superficial granulomatous pyoderma gangrenosum is a rare, superficial, vegetating form of pyoderma gangrenosum that tends to occur as a single lesion, most commonly on...
Superficial granulomatous pyoderma gangrenosum is a rare, superficial, vegetating form of pyoderma gangrenosum that tends to occur as a single lesion, most commonly on the trunk. Herein, we report a clinically confounding case of disseminated superficial granulomatous pyoderma gangrenosum in a patient with a 5-year history of painful and chronic ulcerations of the bilateral upper extremities and face in a sun exposed distribution. This was a diagnostically challenging case due to the treatment-refractory nature of our patient's skin lesions and the atypical clinical and histologic presentations encountered. We review our clinical decision process and acknowledge other entities that were considered during the clinical course of this case. Additionally, we discuss the lack of responsiveness to various treatment options with eventual successful clearance of this patient's active skin disease with initiation of adalimumab.
Topics: Humans; Pyoderma Gangrenosum; Adalimumab; Female; Male; Granuloma; Middle Aged; Suppuration; Dermatitis
PubMed: 38762855
DOI: 10.5070/D330163283 -
BioRxiv : the Preprint Server For... Apr 2024Pyoderma gangrenosum (PG) is a rare neutrophilic dermatosis causing chronic and recalcitrant painful ulcerations. Pathogenic mechanisms are yet poorly understood...
Pyoderma gangrenosum (PG) is a rare neutrophilic dermatosis causing chronic and recalcitrant painful ulcerations. Pathogenic mechanisms are yet poorly understood limiting therapeutic options, however, IL-12/IL-23 inhibition via ustekinumab has previously been associated with positive outcomes. We aimed to elucidate the dysregulated immune landscape of PG and lesional skin changes associated with IL-12/IL-23 blockade. We applied spatial transcriptomics and comparative computation analysis on lesional biopsies from two patients obtained before and after IL-12/IL-23 blockade with ustekinumab. Our data indicate lesional PG skin exhibits complex patterns of inflammation, including a not previously described major infiltration of B cells and establishment of tertiary lymphoid structures. In both patients, IL-12/IL-23 blockade led to marked clinical improvement but was associated with amelioration of contrasting inflammatory pathways. Notably, plasma cell markers and tertiary structures were recalcitrant to the treatment regime suggesting that B cells might play a role in the refractory nature of PG.
PubMed: 38746177
DOI: 10.1101/2024.04.26.591387 -
JAAD Case Reports Jun 2024
PubMed: 38741660
DOI: 10.1016/j.jdcr.2023.08.045 -
Journal of Gastroenterology and... May 2024Extraintestinal manifestations (EIMs) pose a significant threat in inflammatory bowel disease (IBD) patients. Vedolizumab (VDZ) primarily affects the gastrointestinal...
BACKGROUND AND AIM
Extraintestinal manifestations (EIMs) pose a significant threat in inflammatory bowel disease (IBD) patients. Vedolizumab (VDZ) primarily affects the gastrointestinal tract. However, its impact on EIMs remains uncertain. Therefore, we conducted this meta-analysis to examine the effects of VDZ on EIMs during treatment.
METHODS
Relevant studies were identified by conducting thorough searches across electronic databases, including PubMed, Ovid Embase, Medline, and Cochrane CENTRAL. Primary outcomes focused on the proportion of patients with resolution for pre-existing EIMs in IBD patients receiving VDZ. Secondary outcomes included the proportion of patients with EIM exacerbations and new onset EIMs during VDZ treatment.
RESULTS
Our meta-analysis encompassed 21 studies. The proportion of patients with resolution of pre-existing EIMs in VDZ-treated IBD patients was 39% (150/386; 95% confidence interval [CI] 0.31-0.48). The proportion of patients with EIM exacerbations occurred at a rate of 28% (113/376; 95% CI 0.05-0.50), while new onset EIMs had a rate of 15% (397/2541; 95% CI 0.10-0.20). Subgroup analysis revealed a 40% (136/337) proportion of patients with resolution for articular-related EIMs and a 50% (9/18) rate for erythema nodosum. Exacerbation rates for arthritis/arthralgia, erythema nodosum/pyoderma gangrenosum, and aphthous stomatitis during VDZ use were 28% (102/328), 18% (7/38), and 11% (3/28), respectively. The incidence rate of newly developed EIMs during treatment was 11% (564/4839) for articular-related EIMs, with other EIMs below 2%.
CONCLUSION
VDZ demonstrates efficacy in skin-related EIMs like erythema nodosum and joint-related EIMs including arthritis, arthralgia, spondyloarthritis, and peripheral joint diseases. Some joint and skin-related EIMs may experience exacerbation during VDZ therapy.
PubMed: 38740543
DOI: 10.1111/jgh.16612 -
Cureus Apr 2024Pyoderma gangrenosum is a rare ulcerative skin disease of uncertain etiology, which in some cases can be misdiagnosed as an infectious process. In even more unique...
Pyoderma gangrenosum is a rare ulcerative skin disease of uncertain etiology, which in some cases can be misdiagnosed as an infectious process. In even more unique cases, this can occur in the postoperative period. Termed postsurgical pyoderma gangrenosum, this type of inflammatory skin condition requires a high index of suspicion to be able to appropriately treat and reduce complications. We present a 55-year-old female who presented with multiple wounds following mastopexy and abdominoplasty. With a prompt diagnosis and a multidisciplinary approach, we could accurately care for the patient and minimize poor aesthetic sequela.
PubMed: 38738036
DOI: 10.7759/cureus.58060 -
Anais Brasileiros de Dermatologia May 2024Pyoderma Gangrenosum (PG) is a chronic disease characterized by recalcitrant skin ulcers.
BACKGROUND
Pyoderma Gangrenosum (PG) is a chronic disease characterized by recalcitrant skin ulcers.
OBJECTIVE
We aimed to evaluate the demographic, clinical characteristics, treatments and factors affecting the treatment responses of patients with PG.
METHODS
We performed a multicenter study of 12 tertiary care centers. We analyzed the data of the patients who were followed up with a diagnosis of PG between the years 2012‒2022 retrospectively.
RESULTS
We included a total of 239 patients of whom 143 were female and 96 were male, with an average age of 54.2 ± 17.4 years. The most common treatment was systemic steroids (n = 181, 75.7%). Among these patients, 50.8% (n = 92) used systemic steroids as the sole systemic agent, while 49.2% (n = 89) used at least one adjuvant immunosuppressive agent. The independent factors determined in regression analysis to influence response to systemic steroids positively were disease onset age ≥ 30-years, negative pathergy, absence of leukocytosis, negative wound culture, presence of a single lesion, and absence of upper extremity involvement. Biological agents were used in 18.4% (n = 44) of the patients in the present study. We also analyzed pathergy positive PG and early onset (onset age < 30) PG separately due to their distinct clinical features which were revealed during statistical analysis.
STUDY LIMITATIONS
Retrospective nature of the present study.
CONCLUSIONS
Analyses of the factors influencing treatment responses are addressed in this study. Also, we concluded that investigation for accompanying autoinflammatory diseases of pathergy positive PG and early onset PG is necessary and the patients in these two groups are more resistant to treatment, necessitating more complicated treatments.
PubMed: 38735817
DOI: 10.1016/j.abd.2024.02.002