Thesis (doctoral)--Rijksuniversiteit te Groningen.

published_or_final_version / Pathology / Master / Master of Medical Sciences

No description available.

Hypocomplementemic urticarial vasculitis syndrome (HUVS) is a rare type III hypersensitivity disorder characterized by urticarial vasculitis and prolonged hypocomplementemia. Individuals with HUVS may also have joint involvement, pulmonary manifestations, ocular disease, kidney inflammation, or any other form of organ involvement. Hypocomplementemia, the presence of C1q antibody in the serum, and urticarial vasculitis are the keys to the diagnosis of HUVS. It has been reported to accompany certain infections such as hepatitis B, hepatitis C, infectious mononucleosis, and coxsackie group A. However, it has never been reported to be linked to histoplasmosis in the literature. To the best of our knowledge, we report the first case of HUVS presenting concurrently with pulmonary histoplasmosis.

No description available.

Urticarial vasculitis is a relatively rare diagnosis in a patient presenting with urticaria. The process is classically described as a generalized eruption, painful more so than pruritic, lasting longer than 24 hours. Two forms of urticarial vasculitis have been described: ahypocomplementemic form more commonly associated with systemic disease, and a normocomplementemic form that is generally limited to the skin. We report on a uniquely distributed vasculitic eruption restricted mainly to the anterior belt line area in a patient presenting with urticaria and intense pruritus. Urticarial vasculitis as a unique entity is reviewed along with its clinical and histopathologic presentation and the pharmacologic agents used for treatment.

Described for the first time approximately 30 years ago, exercise-induced anaphylaxis is a rare disorder characterized by development of a severe allergic response occurring after mild-to-strenuous physical activity. This disorder is especially important to recognize with the recent increase in physical activity and health fitness fads. A number of predisposing factors (eg, prior ingestion of particular food groups) linked to exercise-induced anaphylaxis has been outlined over the years. Mechanisms govern big the condition are still being unveiled, and it is likely that one mechanism involves mast cell degranulation and inflammatory mediator generation resulting from the biochemical effects of exercise, sometimes in the presence of an ingested allergen such that wheat or shell fish. Clinical manifestations usually occur after around 10 minutes of exercise, and follow a specific sequence, starting with pruritis and widespread urticarial lesions, evolving into a more typical anaphylactic picture with respiratory distress and vascular collapse. Fatality is exceedingly rare, with only one documented casein the literature. There is an overlap of symptoms with other syndromes (such as systemic mastocytosis and cholinergic urticaria), and these should be remembered when establishing a differential. Treatment of exercise-induced anaphylaxis consists of immediate stabilization geared toward the anaphylactic response with epinephrine and antihistamines. The patient needs to be educated on preventive measures and equipped with an epinephrine autoinjector in the event of an emergency. Exercise-induced anaphylaxis remains a potentially serious disorder, and the health care provider should be aware of its clinical features and effective management strategies.

The prevalence of childhood allergic diseases has been increasing in Taiwan. In Taipei area, according to the surveillance of Hsieh KH, the prevalence rate of childhood asthma increased from 1.3% in 1974 to 10.79% in 1994. The other allergic diseases, such as allergic rhinitis, atopic dermatitis and urticaria , have been also increasing in Taipei, Taichung and Tainan city by a serious studies in Western Taiwan. There is no any report of childhood allergic diseases in Eastern Taiwan till now. So, we want to analyze the prevalence of childhood allergic diseases and the common allergens in Eastern Taiwan. From Nov. 2002 to Oct. 2004, all of the primary school children in Yu-Li town in Hualien county were included in this study. (total 2058 children of 12 school). All of them were surveyed with pediatric allergic diseases questionnaire (asthma, allergic rhinitis, atopic dermatitis and urticaria). After the survey, the suspected allergic cases were analyzed for their hypersensitivity to the common allergens by allergen screening test and specific IgE exam of Pharmacia CAP system. 1816 children completed the questionnaire (completive rate 88.24%) and 688 (37.88%) children were suspected to have allergic diseases. 151 (8.87%) asthma¡F448 (24.67%) allergic rhinitis¡F70 (3.85%) atopic dermatitis¡F65 (3.58%) urticaria. A total of 623 suspected allergic cases were analyzed for their hypersensitivity to the common allergens. The common allergens were also analyzed. 87.7% allergic to D. pteronyssinus (Dp) ; 83.9% Blomia tropicalis (Bt) ; 35.0% German cockroach; 16.0% Dog dander; 7.5% Cat dander; 8.0% Candida albicans; 11.2% Bermuda grass; 38.6% shrimp; 33.1% crab; 26.6% milk; 14.3% egg white; 6.7% peanuts; 6.1% Cod fish; 5.4% Wheat; 4.7% Soya bean. The prevalence of childhood allergic diseases in Eastern Taiwan is lower than Western Taiwan. The different envelopment and living type may be the major reasons. From this study, we also suggest that Blomia tropicalis (Bt) should be included in the common aeroallergy analysis in Eastern Taiwan.

Urticaria can be a chronic and debilitating affliction and is a relatively common disorder affecting between 10- 20% of the population. Common causes include reactions to medication, food allergen, physical stimuli and venoms. Urticaria can be acute or chronic. Chronic urticaria lasts for more than 6 weeks and is commonly difficult to treat. The use of immunosuppressive agents for this disorder when antihistamines fail can result in significant morbidity. Recent advances in the pathogenesis, etiology, diagnosis and management of chronic urticaria have led to new paradigms in treatment of this disorder. Cyclosporine is often the most effective but has some unique adverse effects that may prevent it from being used in some patients. The use of intravenous immunoglobulin (IVIG) has proven effective in a variety of reports and we will review the mechanisms likely involved in the successful control of urticarial symptoms by immunomodulating therapy using IVIG. In this review, we will discuss mechanisms and pathogenesis of urticaria and the specific role of intravenous immunoglobulin (IVIG) in this disorder, especially in refractory or steroid-dependent cases.

INTRODUÇÃO: A urticária crônica é caracterizada pelo aparecimento de placas eritêmato-edematosas, pruriginosas que perduram por mais de seis semanas. A investigação da imunidade inata é de grande importância para a compreensão da imunopatologia de doenças dermatológicas crônicas como a Urticária Crônica Idiopática (UCI). Entre os componentes da resposta inata, a avaliação das células dendríticas plasmocitóides (pDCs) e sua capacidade de ativação por agonistas de Toll Like receptor 9 (TLR9) pode contribuir para avaliar o desequilíbrio imunológico encontrado na UCI. OBJETIVOS: Investigar a imunidade inata em pacientes com UCI e os mecanismos envolvidos na modulação da produção de IFN- por pDCs induzida pela ativação do TLR9. MÉTODOS: A secreção de IFN- e IL-10 por leucócitos de pacientes UCI (n=26) e indivíduos normais (Co, n=50) induzida pelo agonista de TLR9, CpGA (1.0 e 2.0M), ou IL12p40 induzida pelo ODN inibitório (ODN S) foi realizado por ensaio imunoenzimático. Quantificação de pDCs em sangue periférico, expressão de moléculas coestimulatórias, expressão intracelular de IFN- por pDCs e fosforilação de STAT (1 e 4) foram analisados por citometria de fluxo. Expressão do mRNA de TLR9 e fator regulador de interferon-7 foi realizado por real time PCR. A presença da pDC (CD123+) em lesões de pele de pacientes UCI foi avaliada por imunoistoquímica. RESULTADOS: Os resultados mostraram uma diminuição na produção de IFN- e IL-10 por leucócitos de pacientes UCI após estímulo com CpGA. O estímulo com ODN S induziu a produção de IL12p40 e suprimiu ainda mais a produção de IL-10 e IFN- induzida pelo CpGA, até 98% de inibição. O número de pDCs no sangue periférico e a capacidade de ativação da pDC nos pacientes, avaliada pela expressão de moléculas co-estimuladoras foi semelhante aos indivíduos normais. Além disto, foram detectadas raras pDCs nas lesões de pele dos pacientes. Um aumento constitutivo da fosforilação de STAT1 foi detectado em linfócitos não estimulados de pacientes UCI associado a uma diminuição da expressão de mRNA de TLR9 após estímulo com CpGA e alterada expressão de mRNA de IRF-7, que podem contribuir para a baixa produção de IFN-. CONCLUSÃO: Os achados mostram uma inibição na produção de IFN- via TLR9, tendo como alvo a pDC e sua participação na resposta inata na Urticária Crônica / INTRODUCTION: Chronic urticaria is a skin disorder characterized by recurrent and transitory itchy weals occurring regularly more than 6 weeks. The investigation of the innate immunity is an important parameter to understand the immunopathology of chronic dermatological diseases, such as Chronic Idiopathic Urticaria (CIU). Among the innate immune components, such as plasmacytoid dendritic cells (pDC) and their functional study activation thorough TLR-9 ligand could contribute to evaluate the immunologic disequilibrium founded in the CIU. OBJECTIVES: We decided to investigate innate immunity in CIU and the mechanisms implicated in the modulation of IFN- production by pDCs upon TLR9 activation. METHODS: The secretion of IFN- and IL-10 secretion by leucocytes of patients with CIU (n=26) and healthy control (HC, n=50) induced by TLR9 ligand, CpG type A (1.0 and 2.0M), or IL12p40 secretion induced by inhibitory ODN (ODN S) was assessed by enzyme-linked immunosorbent assay. Enumeration of pDC, coestimulatory molecules expression, intracellular IFN- expression, and STAT (1 and 4) phosphorylation were assessed by flow cytometry. TLR9 and interferon regulatory factor-7 mRNA transcripts were evaluated by real-time PCR. The presence of pDCs (CD123+) in the skin lesion of patients with CIU were assessed by means of immunohistochemistry staining. RESULTS: The findings showed a decreased IFN- and IL-10 secretion by leucocytes of CIU patients induced by CpGA. Inhibitory ODN was able to induce IL12p40 and further inhibited the IFN- secretion in both HC and patients achieving up 98% inhibition. A normal pDC percentage and degree of activation by costimulatory molecules expression was observed in CIU in patients was similar to control group. Moreover, rare presence of pDCs was detected in the skin lesion of patients. An increased constitutive STAT1 phosphorylation on non-stimulated lymphocytes and CpGA activation induced a down-regulation of TLR9 mRNA transcripts associated with altered IRF-7 along the time of CpG activation which may contribute to the decreased IFN- secretion. CONCLUSIONS: Altogether, the findings showed an impaired IFN- secretion via TLR-9, implicating innate immunity through pDCs as target in Chronic Urticaria