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Indian Journal of Dermatology 2024We report a case of a 54-year-old female diagnosed with HIV and antiretroviral therapy (ART) for the same. Seven years ago, she suffered from fever, cough and weight...
We report a case of a 54-year-old female diagnosed with HIV and antiretroviral therapy (ART) for the same. Seven years ago, she suffered from fever, cough and weight loss, was diagnosed with pulmonary tuberculosis and also seropositive for HIV. She suffered from Herpes Zoster infection, after which her ART regimen was changed to TLD (tenofovir, lamivudine and dolutegravir). The patient presented with two episodes of pyoderma gangrenosum (PG), which were biopsy-proven, corresponding to a rise in CD4 counts above 500. She responded to glucocorticoids, both systemic and topical.
PubMed: 38841245
DOI: 10.4103/ijd.ijd_680_23 -
Journal of Investigative Medicine High... 2024Skin lesions in chronic lymphocytic leukemia (CLL) have been reported in between 4% and 20% of patients with CLL and are a rare entity compared with T-cell leukemia....
Skin lesions in chronic lymphocytic leukemia (CLL) have been reported in between 4% and 20% of patients with CLL and are a rare entity compared with T-cell leukemia. They can present mainly as leukemic cutis or, frequently, as secondary lesions such like urticaria, itching, pyoderma gangrenosum, cutaneous vasculitis, Sweet's syndrome, and erythroderma. We report on an adult patient who developed a skin lesion of forearms and hands, leading to the discovery of isolated cutaneous CLL after two biopsies. Isolated CLL cutaneous location is very rare and may be diagnosed late, as in the case of our patient. A better knowledge of the course of the illness and rapid diagnosis of this CLL cutis leukemia will enhance the therapeutic efficacy of the disease.
Topics: Humans; Leukemia, Lymphocytic, Chronic, B-Cell; Skin Neoplasms; Male; Biopsy; Skin; Aged; Middle Aged
PubMed: 38813875
DOI: 10.1177/23247096231204736 -
Dermatology Practical & Conceptual Apr 2024
Disparities in Financial Burden, Outcomes, and Comorbidities Among Pediatric Patients With Pyoderma Gangrenosum With and Without Mental Health Disorders in a Multivariate Analysis of the 2016 Kids' Inpatient Database.
PubMed: 38810044
DOI: 10.5826/dpc.1402a57 -
Journal of Translational Medicine May 2024
Topics: Humans; Bibliometrics; Pyoderma Gangrenosum; History, 20th Century; History, 21st Century; Biomedical Research
PubMed: 38802936
DOI: 10.1186/s12967-024-05306-4 -
Archives of Dermatological Research May 2024Pyoderma gangrenosum (PG) is a neutrophilic dermatosis characterized by ulcerative painful lesions with violaceous undermined borders. Up to 75% of PG cases develop in...
Pyoderma gangrenosum (PG) is a neutrophilic dermatosis characterized by ulcerative painful lesions with violaceous undermined borders. Up to 75% of PG cases develop in association with an underlying systemic disease. Monoclonal gammopathy is reportedly a concomitant condition with PG, with studies indicating immunoglobulin (Ig) A gammopathy as the most common. Whether gammopathy is associated with PG or is an incidental finding has been debated. We sought to investigate the association and characteristics of gammopathy in patients with PG. We retrospectively identified PG patients at our institution from 2010 to 2022 who were screened for plasma cell dyscrasia. Of 106 patients identified, 29 (27%) had a gammopathy; subtypes included IgA (41%), IgG (28%), and biclonal (IgA and IgG) (14%). Mean age was similar between those with and without gammopathy (60.7 vs. 55.9 years; P = .26). In addition, hematologic or solid organ cancer developed in significantly more patients with vs. without gammopathy (8/29 [28%] vs. 5/77 [6%]; P = .003). Among the subtypes of gammopathy, IgG monoclonal gammopathy had the highest proportion of patients with subsequent cancer development (4 of 8 patients, 50%). Study limitations include a retrospective, single-institution design with a limited number of patients. Overall, our data show a high prevalence of gammopathy in patients with PG; those patients additionally had an increased incidence of cancer, especially hematologic cancer.
Topics: Humans; Pyoderma Gangrenosum; Retrospective Studies; Middle Aged; Female; Male; Paraproteinemias; Aged; Immunoglobulin A; Adult; Immunoglobulin G
PubMed: 38795175
DOI: 10.1007/s00403-024-03098-7 -
Archives of Dermatological Research May 2024Pyoderma gangrenosum (PG) is a rare inflammatory dermatologic condition with neutrophilic infiltration of the skin that causes pustules and ulcerations. Janus kinase... (Review)
Review
Pyoderma gangrenosum (PG) is a rare inflammatory dermatologic condition with neutrophilic infiltration of the skin that causes pustules and ulcerations. Janus kinase (JAK) inhibitors are immunomodulating agents that have been recently described in the literature as an effective treatment for PG. We describe a patient with PG on the lower extremities successfully treated with baricitinib. We also conducted a narrative review of the literature of PG patients treated with JAK inhibitors who were refractory to other treatments.
Topics: Humans; Pyoderma Gangrenosum; Janus Kinase Inhibitors; Pyrazoles; Sulfonamides; Azetidines; Purines; Female; Treatment Outcome; Skin; Middle Aged; Male
PubMed: 38795155
DOI: 10.1007/s00403-024-02958-6 -
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