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Dermatologic Clinics Apr 2024The term neutrophilic dermatosis encompasses a heterogeneous group of diseases, often associated with an underlying internal noninfectious disease, with an overlapping... (Review)
Review
The term neutrophilic dermatosis encompasses a heterogeneous group of diseases, often associated with an underlying internal noninfectious disease, with an overlapping histopathologic background characterized by perivascular and diffuse neutrophilic infiltrates in one or more layers of the skin; extracutaneous neutrophilic infiltrates may be associated. Neutrophilic dermatoses are not frequent in children and, when they appear in this age group, represent a diagnostic and therapeutic challenge. Apart from the classic neutrophilic dermatoses such as pyoderma gangrenosum, Sweet syndrome, and Behçet disease, a neutrophilic dermatosis can be the presentation of rare genetic diseases of the innate immune system, such as autoinflammatory diseases.
Topics: Humans; Child; Dermatitis; Pyoderma Gangrenosum; Skin; Sweet Syndrome; Neutrophils
PubMed: 38423686
DOI: 10.1016/j.det.2023.12.005 -
The Journal of Dermatology Sep 2023Pyoderma gangrenosum (PG) is a rare, neutrophilic skin disease. For the purpose of accurate diagnosis and proper treatment of PG, the Japanese clinical practice guidance...
Pyoderma gangrenosum (PG) is a rare, neutrophilic skin disease. For the purpose of accurate diagnosis and proper treatment of PG, the Japanese clinical practice guidance for PG developed by the Japanese Dermatological Association was published in 2022. In this guidance, clinical aspects, pathogenesis, current therapies, and clinical questions on PG are described from the viewpoints of current knowledge and evidence-based medicine. Here, the English version of the Japanese clinical practice guidelines for PG is presented and is intended to be widely referred to in the clinical examination and treatment of PG.
Topics: Humans; Pyoderma Gangrenosum
PubMed: 37311717
DOI: 10.1111/1346-8138.16845 -
Journal of Personalized Medicine Oct 2023Rheumatoid arthritis (RA) is a chronic, systemic autoimmune disorder characterized by inflammatory arthritis and periarticular structural damage. Available evidence... (Review)
Review
Rheumatoid arthritis (RA) is a chronic, systemic autoimmune disorder characterized by inflammatory arthritis and periarticular structural damage. Available evidence suggests that RA results from complex interactions between genetic susceptibility (e.g., HLA-DRB1), environmental factors (e.g., smoking), and immune dysregulation. Alongside joint-related symptoms, individuals with RA may also experience a wide array of skin issues, including the development of nodules, neutrophilic dermatoses, vasculitis, and vasculopathy. Treatment strategies for these manifestations vary but routinely involve corticosteroids, disease-modifying anti-rheumatic drugs, and biologics, with individualized approaches guided by disease severity. In this review, we provide comprehensive insights into the skin-related issues associated with RA, outlining their clinical characteristics and histopathological findings. Our aim is to facilitate early diagnosis and personalized treatment to improve the quality of life of affected individuals.
PubMed: 37888090
DOI: 10.3390/jpm13101479 -
Maedica Sep 2023Pyoderma gangrenosum (PG) is an infrequent, aseptic neutrophilic dermatosis that can be observed in patients with systemic diseases such as inflammatory bowel disease or...
Pyoderma gangrenosum (PG) is an infrequent, aseptic neutrophilic dermatosis that can be observed in patients with systemic diseases such as inflammatory bowel disease or rheumatic disorders. Due to its rare entity, PG often constitutes a diagnostic enigma, as it simulates other skin disorders. Typically, it is displayed as painful, ulcerative lesions localized to the lower extremities. In our study, we present a case of a 67-year-old woman with recently diagnosed ulcerative colitis who presented with two painful ulcers, one on the left anterior tibia and the other one on the left subclavian area. Initially, their clinical image overlapped with skin abscess. However, taking into account patient's medical history, skin examination, sterile wound cultures and skin biopsy, the diagnosis of PG was established. The patient was completely recovered with high doses of corticosteroids, daily wound changes and surgical intervention involving loose wound edge approximation. In this study, we highlight that clinicians should always be aware of patient's medical history in such cases, in order to early diagnose PG and avoid inaccurate medical approaches which might have an impact on patients' quality of life.
PubMed: 38023744
DOI: 10.26574/maedica.2023.18.3.528 -
Cureus Aug 2023Pyoderma gangrenosum (PG) is a challenging cutaneous manifestation associated with Dubowitz syndrome, a rare genetic disorder characterized by multiple congenital... (Review)
Review
Pyoderma gangrenosum (PG) is a challenging cutaneous manifestation associated with Dubowitz syndrome, a rare genetic disorder characterized by multiple congenital anomalies, developmental delay, and distinctive facial features. This review article aims to provide a comprehensive overview of the association between Dubowitz syndrome and pyoderma gangrenosum, emphasizing the clinical presentation, challenges in diagnosis and management, and potential underlying mechanisms. A comprehensive literature search was conducted to gather relevant studies, and inclusion and exclusion criteria were applied to select appropriate articles. The association between Dubowitz syndrome and pyoderma gangrenosum has been documented in reported cases and studies. Clinical characteristics of Pyoderma gangrenosum in Dubowitz syndrome include painful necrotic ulcers with undermined borders. Diagnosing pyoderma gangrenosum in the context of Dubowitz syndrome can be challenging due to the overlapping clinical features and complexities associated with the syndrome. Managing pyoderma gangrenosum involves a multidisciplinary approach, with general principles of wound care, systemic therapy, and pain management. Specific considerations for treating pyoderma gangrenosum in Dubowitz syndrome include collaboration among specialists and careful monitoring. Future directions for management include further research to understand the underlying mechanisms and develop targeted therapies. Recognizing and addressing pyoderma gangrenosum in Dubowitz syndrome is crucial for optimal patient care. This review enhances awareness among healthcare professionals and provides insights for improving diagnosis, management, and treatment outcomes for individuals with this challenging combination of conditions.
PubMed: 37706150
DOI: 10.7759/cureus.43408 -
The Journal of Dermatology Feb 2024Pyoderma gangrenosum (PG) and hidradenitis suppurativa (HS) are stubborn inflammatory skin diseases categorized as neutrophilic hypodermal dermatoses. These conditions... (Review)
Review
Pyoderma gangrenosum (PG) and hidradenitis suppurativa (HS) are stubborn inflammatory skin diseases categorized as neutrophilic hypodermal dermatoses. These conditions exhibit connections with other autoinflammatory disorders driven by immune responses. Their pathogenesis is complex, rooted in significant imbalances in both innate and adaptive immune systems, particularly featuring elevated levels of tumor necrosis factor-α (TNF-α), interleukin (IL)-1, IL-8, IL-17, and IL-23. Studies involving skin tissue pathology and serology have indicated that targeting specific cytokines can bring therapeutic benefits. Indeed, many patients in clinical settings have responded positively to such interventions. Yet, given the diverse cytokines in play, focusing on a single one with antibody therapy might not always be effective. When resistance to biologics emerges, a combined approach targeting multiple overactive cytokines with immunosuppressants, for example cyclosporine and Janus kinase inhibitors, could be an option. In the current review, we explore recent therapeutic developments for PG and HS.
Topics: Humans; Hidradenitis Suppurativa; Pyoderma Gangrenosum; Skin; Dermatitis; Cytokines
PubMed: 38009911
DOI: 10.1111/1346-8138.17031 -
Cell Death Discovery Jan 2024Neutrophils have both antimicrobial ability and pathogenic effect in the immune system, neutrophil extracellular traps (NETs) formation is one of the representative... (Review)
Review
Neutrophils have both antimicrobial ability and pathogenic effect in the immune system, neutrophil extracellular traps (NETs) formation is one of the representative behaviors of their dual role. NETs formation was triggered by pathogen-related components and pathogen non-related proteins as cytokines to exert its effector functions. Recent studies indicate that the pathogenicity of NETs contributed to several skin diseases such as psoriasis, Stevens-Johnson syndrome, toxic epidermal necrolysis, and neutrophilic dermatosis. Especially in neutrophilic dermatosis, a heterogeneous group of inflammatory skin disorders characterized with sterile neutrophilic infiltrate on dermis, NETs formation was reported as the way of participation of neutrophils in the pathogenesis of these diseases. In this review, we describe the different processes of NETs formation, then summarized the most recent updates about the pathogenesis of neutrophilic dermatosis and the participation of NETs, including pyoderma gangrenosum and PAPA syndrome, Behçet syndrome, hidradenitis suppurativa, Sweet Syndrome, pustular dermatosis and other neutrophilic dermatosis. Furthermore, we discuss the link between NETs formation and the development of neutrophilic dermatosis.
PubMed: 38195543
DOI: 10.1038/s41420-023-01787-2 -
Dermatology Reports Mar 2024Major aphthae are usually located on the dorsum of the tongue, the mucosal surface of the lips and the palate. They are large, round or oval ulcers, with a whitish-grey...
Major aphthae are usually located on the dorsum of the tongue, the mucosal surface of the lips and the palate. They are large, round or oval ulcers, with a whitish-grey bed, well-defined borders and erythematous halo. They are very often accompanied by severe pain. Major aphthae can take up to four months to heal, often with a scar. Relapses are possible. We present a case of major aphtha that was previously diagnosed as squamous cell carcinoma.
PubMed: 38623362
DOI: 10.4081/dr.2024.9646