Bradykinin mediated gastrointestinal edema as a cause of abdominal attacks in patients with hereditary angioedema due to C1-inhibitor deficiency.

Hereditary angioedema (HAE) due to C1-inhibitor (C1-INH) deficiency is a rare hereditary disease characterized by recurrent subcutaneous or submucosal angioedema due to uncontrolled bradykinin production caused by C1-INH dysfunction. Submucosal gastrointestinal swellings provoking abdominal attacks are common and mimic acute abdomen, thus constituting a diagnostic challenge. We aimed to investigate the difficulties in diagnosing abdominal attacks in patients with C1-INH-HAE and to assess the diagnostic value of medical history, the course of the attack, abdominal imaging, and treatment efficacy. The retrospective analysis of diagnostic problems and treatment complications of abdominal attacks in 274 patients with C1-INH-HAE were performed. The value of history, laboratory findings, prodromal symptoms and course of attacks and imaging were assessed. Abdominal attacks were confirmed in 274 of the 322 patients (85%; 190 women and 84 men; age, 4-70 years). In 49% of cases, the abdominal attack was the first and the only symptom for years. The simultaneous presence of marginal erythema (45% of cases), subcutaneous edema (30%), and pharyngo-laryngeal edema (10%) facilitated the diagnosis of an abdominal attack due to C1-INH-HAE. Abdominal attacks manifested with recurrent acute abdominal symptoms lasting 2 to 5 days. The disease course was characterized by the phase of progressive prodromal symptoms followed by peak symptoms and spontaneous symptom resolution. Abdominal imaging often revealed abundant ascites and limited bowel edema. In 60 cases (22%), the diagnostic difficulties resulted in exploratory laparotomy, which was inconclusive in 48 patients (80%). The attacks usually subsided within 2 hours from the administration of recommended drugs (plasma-derived C1-INH, recombinant C1-INH or icatibant). We conclude that recurrent abdominal attacks lasting a few days and resolving spontaneously were common symptoms of C1-INH-HAE. Abdominal imaging revealed transitional fluid or bowel edema. The effectiveness of recommended drugs as plasma-derived C1-INH, recombinant C1-INH or icatibant confirmed the diagnosis.

A half of schoolchildren with 'ISAAC eczema' are ill with allergic contact dermatitis.

BACKGROUND: Similarity in clinical symptoms between atopic eczema (AE) and allergic contact dermatitis (ACD) may lead to misdiagnoses in both clinical practice and epidemiological studies. As patch testing for contact allergy does not seem popular among paediatric allergists, the resulting bias leads mainly to under diagnosing of ACD and over diagnosing of AE in children and adolescents. OBJECTIVES: To assess the frequency of AE and ACD among children and adolescents who answered affirmatively the eczema module of ISAAC questionnaire. METHODS: Of 9320 schoolchildren involved in an allergy screening programme, 143 consecutive participants were recruited for the present study. The inclusion criterion was affirmative answers to questions from the eczema module of the International Study of Asthma and Allergies in Childhood (ISAAC) questionnaire. The children were examined by two allergists: a paediatrician and a dermatologist, and the children underwent patch testing. RESULTS: We diagnosed AE in 46 (55.4%) children and 18 (30.0%) adolescents, whereas 32 (38.6%) children and 31 (51.7%) adolescents were diagnosed with ACD, with a considerable overlap of both diseases. Nine of 46 (19.6%) children and 13 of 25 (52.0%) adolescents with affirmative answers to the question about flexural eczema were diagnosed with ACD, while lacking features sufficient for the diagnosis of AE according to Hanifin and Rajka. Based on the indices from the whole population tested (9320 pupils), a rough estimate of the general ACD prevalence was 5.8% for adolescents, and 8.5% for children, which is close to the figure of 7.2% observed previously in Danish schoolchildren. CONCLUSIONS: Our data demonstrate that 'ISAAC eczema' is an epidemiological entity that embraces comparable portions of cases of atopic eczema and allergic contact dermatitis, and possibly also other less frequent pruritic dermatoses. Each case of chronic recurrent dermatitis in children requires differential diagnosis aimed at allergic contact dermatitis and inflammatory dermatoses other than atopic eczema, even when predominantly localized in flexural areas.