Related Experiment Video
Updated: May 11, 2026
12:23
Granulocyte-dependent Autoantibody-induced Skin Blistering
Published on: October 12, 2012
Neutrophilic urticaria with systemic inflammation: a case series
Hrishikesh Belani1, Lianne Gensler, Urmila Bajpai
1Department of Dermatology, University of California, San Francisco, San Francisco, CA 94115, USA.
JAMA Dermatology
|May 30, 2013
Summary
Neutrophilic urticaria with systemic inflammation (NUSI) presents as difficult-to-treat hives with systemic symptoms. Interleukin-1 (IL-1) blockade with anakinra offers a promising therapeutic option for this condition.
Area of Science:
- Immunology
- Dermatology
- Rheumatology
Background:
- Neutrophilic infiltrates characterize a subset of urticaria patients.
- These urticarial lesions are often less itchy and resistant to standard treatments like antihistamines.
Observation:
- Two patients presented with urticaria, systemic inflammation (fever, night sweats, arthritis), and elevated acute-phase reactants.
- Conventional therapies including corticosteroids and immunosuppressants were ineffective.
Findings:
- Anakinra, an interleukin-1 (IL-1) receptor antagonist, achieved 100% clinical resolution in both patients.
- Patient 1 experienced complete symptom resolution with anakinra.
- Patient 2 achieved 100% symptom control with daily anakinra therapy.
Implications:
- Neutrophilic urticaria with systemic inflammation (NUSI) is a distinct clinical entity.
- IL-1 blockade represents a promising therapeutic strategy for NUSI.
- Identifying NUSI is crucial for patients with antihistamine-resistant urticaria and systemic inflammatory symptoms.
Related Concept Videos
01:19Hypersensitivity Reactions: Immune-Complex Reactions
Type III hypersensitivity reactions occur when antigen–antibody complexes form and activate the complement system. Normally, these complexes help the clearance of antigens by phagocytes and red blood cells. However, when large numbers of immune complexes are present, they can deposit in tissues—particularly in the walls of blood vessels—leading to inflammation and tissue injury. These deposits trigger complement activation and neutrophil recruitment, resulting in serum sickness, a systemic...
01:01Hypersensitivity Reactions: Cytolytic Reactions
Type II hypersensitivity involves IgG and IgM antibodies targeting cell surface antigens, leading to cell destruction. This can occur through complement activation, antibody-dependent cell-mediated cytotoxicity (ADCC), or acting as opsonins for phagocytosis. When excessive, these reactions cause significant tissue damage.Drug-induced hemolytic anemia is a common example, where drugs like penicillin or cephalosporins bind to red blood cells, forming drug-protein complexes. These complexes...
01:24Nephrotic Syndrome I : Introduction
Nephrotic Syndrome is a chronic kidney disorder defined by clinical findings such as severe proteinuria, hypoalbuminemia, hyperlipidemia, and edema. These symptoms result from damage to the glomeruli, the kidney’s filtering units, increasing their permeability to proteins.Definition and Meaning:Proteinuria, defined as the loss of more than 3.5 grams of protein per day in adults, is a crucial feature of nephrotic syndrome. This condition is often accompanied by edema, the accumulation of fluid...
01:30Drug Toxicity: Allergic Reactions
Drug-related allergies are immune-mediated responses triggered by the administration of pharmacological agents. These hypersensitivity reactions are classified based on the immune mechanisms involved. The four primary types—Type I, II, III, and IV—are mediated by different immunological pathways and exhibit distinct clinical manifestations.Type I Hypersensitivity/ IgE-Mediated Reactions: Immunoglobulin E (IgE) immediately mediates Type I hypersensitivity reactions. Upon initial exposure to a...
01:30Hypersensitivities
Hypersensitivity, also known as a hypersensitivity reaction or allergic reaction, is a condition where the body's immune system reacts abnormally to a foreign substance. Such substances, that cause hypersensitivity are referred to as an allergen, could be something typically harmless to most people, like pollen or certain foods.
Types of Hypersensitivities
Hypersensitivity reactions are categorized into four types: Type 1, Type 2, Type 3, and Type 4. Each type has a distinct mechanism...
Types of Hypersensitivities
Hypersensitivity reactions are categorized into four types: Type 1, Type 2, Type 3, and Type 4. Each type has a distinct mechanism...
01:30Allergic Reactions: Anaphylaxis
Anaphylaxis is a severe, life-threatening hypersensitivity reaction mediated by Immunoglobulin E (IgE) antibodies. When IgE binds to allergens, it triggers the release of mediators– histamine, leukotrienes, and prostaglandins from mast cells and basophils. These mediators cause vasodilation, edema, and inflammation, leading to various symptoms.The primary allergens causing anaphylaxis include food items (e.g., peanuts, shellfish), drugs (e.g., penicillin, asparaginase, corticotropin, heparin),...