Related Experiment Video
Updated: Apr 28, 2026
12:23
Granulocyte-dependent Autoantibody-induced Skin Blistering
Published on: October 12, 2012
9.9K
Urticarial pathology in Schnitzler's (hyper-IgM) syndrome
F R de Castro1, I Masouyé, R K Winkelmann
1Department of Dermatology, Mayo Clinic Scottsdale, Ariz., USA.
Dermatology (Basel, Switzerland)
|January 1, 1996
Summary
Schnitzler's syndrome, a rare disorder causing chronic urticaria and IgM gammopathy, often presents with neutrophilic urticaria. However, these neutrophils are typically not linked to immune complex vasculitis, and epidermal changes require further study.
Area of Science:
- Dermatopathology
- Immunology
- Rheumatology
Background:
- Schnitzler's syndrome is a rare autoinflammatory disorder.
- Characterized by chronic urticaria and monoclonal IgM gammopathy.
- Pathogenesis of urticarial flares is not well understood.
Purpose of the Study:
- To define histopathologic changes in Schnitzler's syndrome urticarial lesions.
- Reviewing 25 biopsies from 15 patients.
- Including 11 previously reported cases.
Main Methods:
- Histopathologic examination of skin biopsies.
- Review of clinical and laboratory data.
- Immunofluorescence studies.
Main Results:
- Most biopsies (13/25) showed neutrophilic urticaria.
- Leukocytoclastic vasculitis observed in 2 biopsies from a C4-deficient patient.
- Lymphocytic urticaria (5 biopsies) and spongiotic dermatitis (4 biopsies) were also noted.
- Epidermal changes present in 17/25 specimens.
Conclusions:
- Histopathologic findings in Schnitzler's syndrome are diverse.
- Neutrophilic urticaria is common but usually not associated with immune complex vasculitis.
- Further investigation of epidermal changes is warranted.
Related Concept Videos
01:23Skin Diseases and Disorders
5.1K
Skin is the first line of defense and encounters a variety of microbes. Some pathogenic strains are often the cause of a broad range of infections of the skin and other body systems. These conditions can affect people of all ages and may have different causes, including genetic factors, infections, autoimmune reactions, environmental factors, and lifestyle choices.
Gram-positive Staphylococcus spp. and Streptococcus spp. are responsible for many of the most common skin infections. However, many...
Gram-positive Staphylococcus spp. and Streptococcus spp. are responsible for many of the most common skin infections. However, many...
5.1K
01:25Endocarditis II: Clinical Features of Infective Endocarditis
923
Endocarditis can present various clinical features depending on the causative organism and the patient's underlying health conditions. Initially, the clinical features of infective endocarditis develop gradually, presenting with nonspecific symptoms that can be easily mistaken for other illnesses.General SymptomsEarly symptoms of infective endocarditis are fever, chills, weakness, malaise, fatigue, and weight loss. These symptoms reflect the systemic nature of the infection and the body's...
923
01:23Rheumatic Heart Disease I: Introduction
1.0K
Rheumatic heart disease or RHD is a chronic condition that results from rheumatic fever, causing permanent damage to the heart valves.Etiology and Risk FactorsIt primarily arises from rheumatic fever, an inflammatory disease that can develop after untreated or inadequately treated group A streptococcal (GAS) pharyngitis. Streptococcus spreads through direct contact with oral or respiratory secretions. While the bacteria are the causative agents, factors like malnutrition, overcrowding, poor...
1.0K
01:24Nephrotic Syndrome I : Introduction
1.1K
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...
1.1K
01:19Hypersensitivity Reactions: Immune-Complex Reactions
266
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...
266