Adult ; Female ; Humans ; Lymphadenitis ; complications ; diagnosis ; pathology ; Positron-Emission Tomography ; Urticaria ; etiology ; Vitiligo ; etiology

Familial Mediterranean fever (FMF) is an autosomal recessive disease characterized by recurrent episodes of fever and serosal, synovial, or cutaneous inflammation, caused by a dysfunction of pyrin as a result of mutation within the MEFV gene. It occurs mainly among Mediterranean and Middle Eastern populations, including Jews, Arabs, and Turks. However, FMF cases have been reported outside the Mediterranean and Middle Eastern countries in recent years. Although FMF has been relatively rare in Korea until now, proper recognition of FMF might lead to more frequent diagnoses of FMF. We experienced an interesting case, a 31-year-old Korean man who presented with recurrent abdominal pain with fever and urticarial eruption for 10 years. DNA analysis showed complex mutations (p.Leu110Pro, p.Glu148Gln) in the MEFV gene. To date, three cases have been reported, and this case of FMF with skin conditions is the first case in Korea.
Abdominal Pain/*etiology ; Adult ; Base Sequence ; Cytoskeletal Proteins/genetics ; Familial Mediterranean Fever/complications/*diagnosis/genetics ; Humans ; Male ; Polymorphism, Single Nucleotide ; Recurrence ; Sequence Analysis, DNA ; Urticaria/*diagnosis

Restless legs syndrome (RLS) is a disease characterized by the urge to move the legs and sleep disturbances. Similarly, chronic spontaneous urticaria (CSU) is a dermatological disease characterized by pruritus and sleep disorders. In this study, we aimed to determine the prevalence and severity of RLS in patients with chronic spontaneous urticaria (CSU) and to compare the quality of sleep of patients with and without RLS in the CSU group using the Pittsburgh Sleep Quality Index. A total of 130 patients with CSU and 100 healthy controls were included in this study. The frequency of RLS, frequency of sleep disturbances, and average score of RLS in patients with CSU were statistically significantly higher than control groups (respectively P = 0.008, P = 0.009, P = 0.004). Subjective sleep quality, sleep latency and habitual sleep efficiency scores in patients with RLS were statistically significantly higher than patients without RLS (respectively P = 0.016, P = 0.007, P = 0.035). We claimed that pruritus of urticaria may decrease the quality of sleep in patients with RLS and it may trigger and worsen the restless legs syndrome. Furthermore, RLS and CSU may share a common etiology.
Adult ; Case-Control Studies ; Cross-Sectional Studies ; Female ; Humans ; Male ; Middle Aged ; Prevalence ; Restless Legs Syndrome/*complications/epidemiology/pathology ; Severity of Illness Index ; Sleep Wake Disorders/etiology ; Urticaria/*complications/diagnosis

Urticarial vasculitis is characterized clinically by urticarial skin lesions and histologically by leukocytoclastic vasculitis. Hypocomplementemic urticarial vasculitis is associated with connective tissue diseases such as systemic lupus erythematosus (SLE). We report a case of urticarial vasculitis that preceded manifestations of SLE.
Anti-Infective Agents/therapeutic use ; Anti-Inflammatory Agents/therapeutic use ; Diagnosis, Differential ; Female ; Humans ; Lupus Erythematosus, Systemic/*diagnosis/etiology/pathology ; Middle Aged ; Recurrence ; Skin/pathology ; Urticaria/complications/*diagnosis ; Vasculitis, Hypersensitivity/complications/*diagnosis/pathology

Chronic urticaria may often be associated with interleukin (IL)-1-mediated autoinflammatory disease, which should be suspected if systemic inflammation signs are present. Here, we report a case of Schnitzler's syndrome without monoclonal gammopathy treated successfully with the IL-1 receptor antagonist anakinra. A 69-year-old man suffered from a pruritic urticarial rash for 12 years. It became aggravated episodically and was accompanied by high fever, arthralgia, leukocytosis, and an elevated C-reactive protein and erythrocyte sedimentation rate. The episodes each lasted for over one week. Neutrophilic and eosinophilic inflammation was found on skin biopsy. However, serum and urine electrophoresis showed no evidence of monoclonal gammopathy. The cutaneous lesions were unresponsive to various kinds of anti-histamines, systemic glucocorticoids, colchicine, cyclosporine, dapsone, and methotrexate, which were administered over a span of 3 years immediately preceding successful treatment. A dramatic response, however, was observed after a daily administration of anakinra. This observation suggests that the correct diagnosis of this case is Schnitzler's syndrome without monoclonal gammopathy. For an adult patient with refractory chronic urticaria and systemic inflammation, Schnitzler's syndrome could be considered as a possible differential diagnosis. Although the typical form of Schnitzler's syndrome exhibits the presence of monoclonal gammopathy as a diagnostic criterion, monoclonal gammopathy may be absent in an atypical form. In such a situation, an IL-1 antagonist should be effective for the management of chronic urticaria.
Aged ; Blood Sedimentation ; C-Reactive Protein/metabolism ; Chronic Disease ; Humans ; Interleukin 1 Receptor Antagonist Protein/therapeutic use ; Leukocytes/metabolism ; Male ; Paraproteinemias/complications ; Schnitzler Syndrome/blood ; Schnitzler Syndrome/drug therapy ; Urticaria/complications

OBJECTIVENeonatal-onset multisystem inflammatory disease (NOMID) is not widely recognized in China. This study aimed to investigate the diagnosis and treatment of NOMID.

METHODTo analyze the clinical characteristics and laboratory results including skin biopsy, gene analysis and serum interleukin 1β of a boy admitted to Peking University First Hospital in November of 2013. Reports on NOMID were searched and the clinical and laboratory characteristics of reported cases were summarized.

RESULTThe patient was a 1-year-old boy. He had urticaria since 2 days after birth, and presented with episodes of fever, aseptic meningitis, symptoms of joints, short statue, hearing loss, abnormal fundus findings, and leucocytosis, high level of c-reactive protein (CRP) and abnormal findings of head MRI including ventriculomegaly and white matter dysplasia. Urticaria was confirmed by skin biopsy. Gene analysis showed T1702T/A in exon 4 of NLRP3 gene, which causes Phe568lle. Serum interleukin 1β increased dramatically. The boy was diagnosed as NOMID. He did not respond to antibiotic therapy and anti-allergy therapy. Corticosteroid therapy induced normalization of body temperature, and alleviation of rash, but not improvement in cerebrospinal fluid cell numbers. After searching reports of NOMID at PubMed, and Chinese literature published before November 2013, we summarized cases from 8 reports and reviewed 148 cases. The results showed that fever, urticaria, meningitis and arthropathy are the most common manifestations of NOMID, only 57% (69/122) of patients had mutation of NLRP3.

CONCLUSIONThis is a rare report of NOMID in children in China. Fever, urticaria, aseptic meningitis and persistently high level of CRP are characteristics of NOMID. Gene analysis and serum interleukin-1β detection can aid in diagnosis.

C-Reactive Protein ; analysis ; Carrier Proteins ; genetics ; China ; Cryopyrin-Associated Periodic Syndromes ; complications ; diagnosis ; therapy ; Fever ; etiology ; Humans ; Infant ; Interleukin-1beta ; blood ; Joint Diseases ; etiology ; Male ; Meningitis, Aseptic ; etiology ; Mutation ; NLR Family, Pyrin Domain-Containing 3 Protein ; Urticaria ; etiology

Anisakidae larvae can cause anisakiasis when ingested by humans. Although several groups have reported a gastrointestinal Anisakis allergy among people in Spain and Japan, our report is the first to summarize the clinical features of 10 Anisakis allergy cases in Korea. We enrolled 10 Korean patients (6 men and 4 women) who complained of aggravated allergic symptoms after ingesting raw fish or seafood. Sensitization to Anisakis was confirmed by detecting serum specific IgE to Anisakis simplex. The most common manifestation of anisakiasis was urticaria (100%), followed by abdominal pain (30%) and anaphylaxis (30%). All patients presenting with these symptoms also exhibited high serum specific IgE (0.45 to 100 kU/L) to A. simplex. Nine patients (90%) exhibited atopy and increased total serum IgE levels. The fish species suspected of carrying the Anisakis parasite were flatfish (40%), congers (40%), squid (30%), whelk (10%), and tuna (10%). Anisakis simplex should be considered as a possible causative food allergen in adult patients presenting with urticaria, angioedema, and anaphylaxis following the consumption of raw fish or seafood.
Abdominal Pain/immunology ; Adult ; Aged ; Anaphylaxis/immunology ; Angioedema/immunology ; Animals ; Anisakiasis/complications/ethnology/*immunology ; Anisakis/*immunology ; Antibodies, Helminth/*blood ; Asian Continental Ancestry Group ; Female ; Food Hypersensitivity/complications/ethnology/*immunology ; Humans ; Immunoglobulin E/*blood ; Korea/epidemiology ; Male ; Middle Aged ; Seafood/*adverse effects/parasitology ; Urticaria/immunology ; Young Adult

OBJECTIVETo compare the activity of antihistamines by the index of area under dose-response curve (AUDRC) obtained from histamine-induced wheal and flare reactions.

METHODSMizolastine 10 mg, loratadine 10 mg, and placebo were given to 90 healthy volunteers and 60 allergic patients in a double-blind and randomized manner. Histamine titration tests (histamine concentrations 54.3, 20.0, 7.3, and 2.7 mmol/L) were performed for each one before dosing and 2, 4, and 24 hours after dosing. The reactivity was evaluated by histamine-induced wheal and flare areas. The AUDRC values of the wheal and flare areas as a function of the natural logarithm transformed histamine concentration were calculated for each subject, and compared.

RESULTSThere was no significant difference of the wheal and flare areas between health volunteers and allergic patients. The AUDRC(27-54.3 mmol/l.) for wheal and flare of mizolastine was 115.7, 23.4, 7.7, 49.8 and 902.1, 40.9, 2.6, 46.9 ln (mmol/L) x mm2 at each time (before dosing and 2, 4, 24 hours after dosing) respectively. Compared with loratadine [116.2, 80.2, 49.7, 71.9 and 957.6, 495.3, 153.5, 205.9 ln (mmol/L) x mm2], mizolastine decreased AUDRC(2.7 - 54.3 mmol/L significantly (P < 0.01).

CONCLUSIONHistamine-induced wheal and flare inhibition test is a reliable pharmacodynamic model for antihistamines, and AUDRC may be an useful index to predict antihistamines pharmacodynamic activity.

Adolescent ; Adult ; Aged ; Anti-Allergic Agents ; therapeutic use ; Area Under Curve ; Benzimidazoles ; therapeutic use ; Double-Blind Method ; Female ; Histamine ; Histamine H1 Antagonists ; therapeutic use ; Humans ; Loratadine ; therapeutic use ; Male ; Middle Aged ; Rhinitis, Allergic, Perennial ; complications ; drug therapy ; Skin Tests ; Time Factors ; Urticaria ; chemically induced ; drug therapy

: Anaphylaxis is a predominantly childhood disease. Most of the literature on anaphylaxis has emerged from Western countries. This study aimed to describe the incidence, triggers and clinical presentation of anaphylaxis among children in Singapore, look for predictors for anaphylaxis with severe outcomes, and study the incidence of biphasic reactions.: We retrospectively reviewed records of children presenting with anaphylaxis to our paediatric emergency department from 1 January 2007 to 31 December 2014.: We identified 485 cases of anaphylaxis in 445 patients. Cutaneous symptoms (urticarial/angio-oedema) were the most common across all age groups (481 cases, 99%), followed by respiratory (412, 85%), gastrointestinal (118, 24%) and cardiovascular (35, 7.2%) symptoms. Central nervous system symptoms (drowsiness/ irritability) were rare across all age groups (11, 2.2%). Food was identified as the most common trigger across all age groups (45% to 63%). Seafood was the most common food trigger (57, 25%). A total of 420 (86.6%) children were treated with adrenaline, 451 (93%) received steroids and 411 (85%) received antihistamines. Sixty-three (13%) children fulfilled the criteria of severe anaphylaxis. There was no statistically significant association between severe anaphylaxis and the type of trigger (= 0.851), nor an overall past history of atopy (= 0.428). The only independent predictor for severe anaphylaxis was a previous drug allergy (= 0.016). A very low prevalence of biphasic reactions (0.6% of study population) was noted in our study.: We described the presentation and management of anaphylaxis in the Singapore population. A history of drug allergy is associated with severe presentation. Biphasic reactions are rare in our population.
Adolescent ; Adrenal Cortex Hormones ; therapeutic use ; Anaphylaxis ; drug therapy ; epidemiology ; etiology ; physiopathology ; Angioedema ; epidemiology ; etiology ; physiopathology ; Child ; Child, Preschool ; Drug Hypersensitivity ; epidemiology ; Emergency Service, Hospital ; Epinephrine ; therapeutic use ; Female ; Food Hypersensitivity ; complications ; epidemiology ; Gastrointestinal Diseases ; epidemiology ; etiology ; physiopathology ; Histamine Antagonists ; therapeutic use ; Humans ; Hypotension ; etiology ; physiopathology ; Incidence ; Infant ; Male ; Pediatrics ; Prevalence ; Respiratory Tract Diseases ; epidemiology ; etiology ; physiopathology ; Retrospective Studies ; Risk Factors ; Seafood ; Severity of Illness Index ; Singapore ; epidemiology ; Sympathomimetics ; therapeutic use ; Tertiary Care Centers ; Urticaria ; epidemiology ; etiology ; physiopathology