Neutrophilic dermatosis of the dorsal hands (NDDH) is a localized variant of Sweet syndrome, a neutrophilic dermatosis commonly associated with underlying malignancy or systemic inflammatory disorders. NDDH can be mistaken for infection due to its rarity, clinical appearance, and common association with elevated inflammatory markers. A high index of suspicion for this diagnosis should be maintained with symmetrical hand lesions, as approximately 78% of patients with NDDH have bilateral disease. Patients respond rapidly to the initiation of systemic steroids; however, like with generalized cutaneous Sweet syndrome, diagnosis should prompt further work-up for underlying disease.
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http://dx.doi.org/10.56305/001c.115811 | DOI Listing |
Front Immunol
March 2025
Semmelweis University, Department of Physiology, Budapest, Hungary.
Objective: Contact hypersensitivity (CHS), or allergic contact dermatitis (ACD), is an inflammatory skin disorder characterized by an exaggerated allergic reaction to specific haptens. During this delayed-type allergic reaction, the first contact with the allergen initiates the sensitization phase, forming memory T cells. Upon repeated contact with the hapten, the elicitation phase develops, activating mostly macrophages, cytotoxic T cells, and neutrophilic granulocytes.
View Article and Find Full Text PDFClin Rev Allergy Immunol
March 2025
Department of Rare Diseases, Peking Union Medical College Hospital (PUMCH), Chinese Academy of Medical Sciences & Peking Union Medical College; State Key Laboratory of Complex Severe and Rare Diseases, PUMCH; Department of Rheumatology and Clinical Immunology, PUMCH; National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Ministry of Science & Technology; Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, 100730, China.
Interleukin (IL)-17, a pro-inflammatory cytokine, plays a pivotal role in immune regulation by bridging innate and adaptive responses. Beyond its canonical involvement in T helper-17 cells-mediated immunity, IL-17 contributes significantly to the pathogenesis of systemic autoinflammatory diseases (SAIDs) including Familial Mediterranean Fever (FMF), nucleotide-binding oligomerization domain-like receptor protein 3 (NLRP3)-associated autoinflammatory diseases, and synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome. Dysregulated IL-17 signaling drives inflammasome activation, neutrophil recruitment, and chronic tissue inflammation.
View Article and Find Full Text PDFJAMA Dermatol
March 2025
Service de Dermatologie et Allergologie, Faculté de Médecine, Sorbonne Université, Hôpital Tenon, Assistance Publique-Hôpitaux de Paris, Paris, France.
Importance: VEXAS syndrome (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) is a monogenic disease caused by UBA1 somatic variants in hematopoietic progenitor cells, mostly involving adult men. It is associated with inflammatory-related symptoms, frequently involving the skin and hematological disorders. Recently described myelodysplasia cutis (MDS-cutis) is a cutaneous manifestation of myelodysplasia in which clonal myelodysplastic cells infiltrate the skin.
View Article and Find Full Text PDFRheumatology (Oxford)
March 2025
Department of Medicine, McMaster University, Hamilton, ON, Canada.
ANCA-associated vasculitis (AAV) is a heterogeneous autoimmune disease marked by varying organ involvement and outcomes. Plasma exchange, a method of removing native plasma and replacing it with crystalloid, albumin or donor plasma, can deplete autoantibodies and may help control autoimmune diseases rapidly. In AAV, several randomized controlled trials have been performed but, individually, had mixed results.
View Article and Find Full Text PDFRheumatology (Oxford)
March 2025
Department of Medicine, University of Cambridge, Cambridge, UK.
Drug development in ANCA-associated vasculitis has aimed to improve on the success of the B cell depleting monoclonal antibody rituximab and exploit better understanding of inflammatory pathways. More potent B cell depletion strategies are being tested as are B cell cytokine inhibitors. The involvement of the complement system in pathogenesis is more complicated than previously thought and extends beyond C5a dysregulation and its inhibition with avacopan, broader complement inhibitors and complement regulatory agonists are potential newer therapies.
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