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SARS-CoV-2 infection can lead to a variety of clinical manifestations. The occurrence of tongue swelling has recently reported in severe cases of COVID-19, and angioedema has suggested as the causative mechanism. Several factors, such as genetic predisposing factor and angiotensin-converting enzyme inhibitors (ACEI) therapies, have proposed to induce angioedema, especially as concerns patients requiring ICU treatments. Nevertheless, the question is still debated and other causes not yet recognized should be considered. Here we present a case of macroglossia occurred in a patient deceased for COVID-19 disease, who had no family history of angioedema and did not receive ACEI as antihypertensive drug. Histological and immune-histochemical analysis revealed tongue muscle atrophy with infiltrating macrophages suggesting repair mechanisms, as seen in nerve injury recovery. These new pathological findings may open new fields of study on the pathogenesis of SARS-CoV-2., (Copyright © 2021 The Authors. Published by Elsevier Ltd.. All rights reserved.)

Acquired angioedema with C1-inhibitor deficiency is a rare and peculiar entity belonging to the spectrum of bradykinin angioedemas. It usually occurs in subjects over 60 years old, and is mostly associated with a B-cell lymphoid hemopathy or a monoclonal gammopathy. The diagnosis relies on at least one angioedema episode, lasting more than 24 h, and on the decrease of functional C1-inhibitor. Low C1q is observed in 90% of patients, and an anti C1-inhibitor antibody is found in 50% of patients. The treatment of severe attacks relies on icatibant or C1-inhibitor perfusions. Long term prophylaxis in patients with frequent attacks requires treatment of the associated hemopathy if so. In case of idiopathic angioedema, tranexamic acid and danazol may be used, provided that there is-no thrombophilia; as well as rituximab as second-line treatment. Inhibitors of kallikrein still need to be evaluated in this therapeutic indication., (Copyright © 2020. Published by Elsevier Masson SAS.)

A red swollen face can be a skin sign of a potentially life-threatening condition. We present in detail the main clinical presentations, diagnostic tests, and management of some of the most severe conditions that can frequently present as a red and swollen face: acute or recurrent angioedema, mast cell-driven or bradykinin-mediated angioedema, nonhereditary and hereditary angioedema, allergic or photoallergic facial contact dermatitis, contact urticaria, severe adverse drug reactions (particularly drug reaction with eosinophilia and systemic symptoms [DRESS]), skin infections (erysipelas, cellulitis, necrotizing fasciitis), and autoimmune diseases (dermatomyositis). There are many other conditions that also have to be considered in the differential diagnosis of a red swollen face., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Angioedema is a clinical entity defined as self-limiting edema localized in the deeper layers of the skin and mucosa and lasting for several days. Angioedema can be provoked by bradykinin and/or mast cell mediators, including histamine. Four types of acquired and three types of hereditary angioedema have been identified. The most obvious form of angioedema associated with other systemic disease is acquired angioedema due to C1-inhibitor deficiency. It is characterized by acquired consumption of C1 inhibitor and various underlying disorders, such as multiple myeloma, chronic lymphocytic leukemia, rectal carcinoma, and non-Hodgkin lymphoma. Suspected cases need an accurate differential diagnosis to exclude all other types of acquired and hereditary angioedema., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Background: Visceral angioedema is a rarely reported side effect of angiotensin-converting-enzyme inhibitors (ACEI). Because signs and symptoms tend to be nonspecific, the diagnosis is difficult to make, especially in the emergency department (ED)., Case Report: We describe 2 patients presenting with signs of hypovolemic shock, in which the diagnosis of ACEI-induced visceral angioedema was made in the ED. We surmise that patients with abdominal pain, who present with hypovolemic shock and are taking medications that can predispose to angioedema, may have this complication if their hemoglobin level is elevated compared with their previous levels. An abdominal computed tomography scan, if it does not identify any other significant etiology, will increase the probability that ACEI-induced visceral angioedema is the diagnosis when there is nonspecific bowel wall thickening or edema. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: Identification of ACEI-induced visceral angioedema in the ED will avoid prolonged admissions, unnecessary procedures, and future recurrences., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Background: Angiotensin-converting enzyme inhibitor (ACEI)-induced angioedema can occur at any point during therapy and, when severe, can require mechanical ventilation. Standard agents for anaphylactic reactions have limited efficacy for bradykinin-mediated angioedema and, therefore, agents approved for hereditary angioedema are increasingly prescribed for these patients., Objective of the Review: This systematic review critically evaluates evidence describing the off-label use of fresh frozen plasma (FFP), prothrombin complex concentrate (PCC), complement 1 esterase inhibitor (C1-INH), icatibant, and ecallantide for treatment of ACEI-induced angioedema., Discussion: A PubMed search was conducted and articles were cross-referenced for additional citations. All full-text clinical trials, case series, and case reports published in the English language describing pharmacologic treatment of ACEI-induced angioedema were included. Thirty-seven publications detailing FFP, PCC, and regimens approved for hereditary angioedema, including icatibant, ecallantide, and C1-INH, are reviewed extensively., Conclusions: While findings of decreased time to symptom resolution or a cessation in symptom progression have been reported with each of these therapies, additional data showing clinically relevant implications, such as reduced intensive care unit length of stay or avoidance of mechanical ventilation, are warranted, especially when taking cost into consideration. FFP has limited evidence demonstrating a benefit for treatment of ACEI-induced angioedema without consistent dosing strategies. However, given its wide availability and low potential for adverse reactions, FFP therapy may be reasonable. Of the novel agents traditionally used for hereditary angioedema, icatibant has the highest level of evidence and has been reported to be successful in limiting the progression of angioedema., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Angioedema is a condition in which an increase in vascular permeability leads to the swelling of body tissues. There are both hereditary and acquired forms of the disease, with the latter often associated with the administration of angiotensin-converting enzyme inhibitor medication. Involvement of the intestinal tract is a rare manifestation of angioedema, and can present with abdominal pain, nausea, and vomiting. It is critical for radiologists to be aware of this entity, as they may have the only opportunity to make the diagnosis. We present three cases of intestinal angioedema diagnosed on MRI with discussion of the imaging findings., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Purpose: Two types of bradykinin-mediated angioedema, hereditary angioedema (HAE) and acquired angioedema (AAE), result from deficiency or dysfunction of C1 esterase inhibitor, leading to an overproduction of bradykinin, which can lead to vascular permeability and life-threatening angioedema of the airway. The objective of this study was to review perioperative outcomes in a series of patients with HAE and AAE and to review current knowledge about anesthetic complications in patients with HAE or AAE., Methods: Medical records were retrospectively reviewed for perioperative complications in patients with HAE or AAE who underwent general anesthesia from January 1, 2000, to December 31, 2014, at our institution., Results: Twenty-four patients (13 with HAE, 10 with AAE, and 1 with unspecified angioedema) underwent 38 instances of general anesthesia with airway manipulation. All except 4 received prophylactic therapy. One patient, a 67-year-old woman who was pretreated with stanozolol and fresh frozen plasma required reintubation after postoperative airway edema developed., Conclusion: Life-threatening episodes of angioedema of the airway occur infrequently, but they can occur in patients who received pretreatment and in patients who have previously undergone anesthesia uneventfully. Anesthesiologists must be ready to emergently manage a difficult airway and must be familiar with recommendations provided in consensus guidelines for the treatment of HAE and AAE patients., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Background: Angioedema (AE) is characterized by nonpitting edema of the dermis and subcutaneous layers. The most common sites of involvement are the tongue, lips, face, and throat; however, swelling can also occur in the extremities, genitalia, and viscera. Life-threatening airway swelling can also occur. AE may be allergic or nonallergic. The overall lifetime incidence of AE is reported to be as high as 15%., Objective: This article summarizes the etiology, pathophysiology, and current treatment of several forms of nonallergic AE (including hereditary, acquired, and idiopathic AE) and focuses on angiotensin-converting enzyme inhibitor-induced angioedema (ACEi-AE), which is responsible for 30%-40% of all AE seen in United States emergency departments., Discussion: Although the triggers, which are primary biologic mechanisms, and treatments for ACEi-AE may differ from those of the hereditary and acquired forms of AE, the clinical effects of ACEi-AE are mediated through a shared pathway, the kallikrein-kinin system. Thus, although current therapeutic options for ACEi-AE are limited, recent advances in the treatment of hereditary AE (HAE) appear promising for improving the outcomes of patients with ACEi-AE., Conclusions: New HAE medications that correct imbalances in the kallikrein-kinin system may prove safe and efficacious in the treatment of ACEi-AE., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Angioedema is a potentially life-threatening condition that may present at any point in the perioperative care of patients. It requires prompt recognition and diagnosis; the primary concern during acute attacks is airway management. The pathophysiology, various causes of angioedema, and treatment strategies according to underlying etiology are presented., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Angioedema due to acquired C1 esterase deficiency is a rare condition and a non-inflammatory disease characterized by episodes of edema of the mucosa of the upper airway or gastrointestinal tract. The purpose of this case report is to heighten awareness among emergency physicians of a peritonitis-like condition that can develop into angioedema due to acquired C1 esterase inhibitor deficiency, and thereby help to prevent false diagnosis resulting in unnecessary surgical intervention. We report the case of a 21-year-old man who presented to the Emergency Department (ED) with abdominal pain. He was later diagnosed with angioedema of the gastrointestinal tract due to acquired C1 esterase deficiency that was initially suspected as peritonitis. Careful evaluation of the acute abdomen in acquired C1 esterase deficiency is very important in the ED to distinguish between medical and surgical causes of an acute abdomen., (Copyright © 2011 Elsevier Inc. All rights reserved.)

We are reporting the case of a bradykinin-mediated angioedema, secondary to the angiotensin converting enzyme inhibitors, which delayed treatment could have unfavorably influence the vital prognostic of the patient. Initially, the patient had an isolated edema of the superior lip. Prehospital treatment included methylprednisolone, hydroxyzine and epinephrine. The patient was subsequently taken to the emergency department. His situation deteriorated. An edema of the cheeks and the tongue appeared. The transfer of the patient to an emergency department, specializing in kinin angioedema was organized, in order for the patient to receive specific treatments. After a subcutaneous injection of icatibant, the situation improved very rapidly, with a regression of the edema. This observation is consistent with the early use of the specific therapeutic in bradykinin-mediated angioedema. Any delay in administering the treatment can negatively impact the prognostic. The availability of such treatments should therefore be organized during the prehospital phase., (Copyright © 2011 Elsevier Masson SAS. All rights reserved.)

Objectives: Present the clinical signs of bradykinin-mediated angioedema, a disease little known to intensive care anaesthesiologists, and develop their scientific basis with recent data on management in emergency and perioperative care., Data Sources: International recommendations and recent general reviews. Data collection was performed using the Medline database with the keyword: angioedema., Study Selection and Data Extraction: Research studies published during the last 10 years were reviewed. Relevant clinical information was extracted and discussed., Data Synthesis: Angioedema is a clinical syndrome characterized by episodes of transitory recurrent submucosal and subcutaneous oedema, called attacks. During an attack, the oedema may be localized at the level of the skin and/or ENT and digestive tract mucosa. This syndrome is not due to an allergic reaction. It is related to a C1 complement inhibitor deficiency or an increase in factor XII resulting in the excessive release of bradykinin, which leads to capillary permeability. There are hereditary and acquired forms, notably associated with the use of ACE inhibitors and sartans. This rare disease should be recognized by anaesthesiologists and intensive care and emergency physicians because, in the absence of specific treatment, it can be life-threatening due to the appearance of laryngeal oedema. In addition, there is a risk that the patient may have an attack during the perioperatory period, due to surgical trauma. International recommendations exist, and there are new molecules available in France. For moderate attacks, treatment is based on tranexamic acid. For hereditary forms, according to the localization and gravity of the attacks, emergency treatment is based on the use of Icatibant, a bradykinin B2 receptor inhibitor, and C1 inhibitor concentrate. For pregnant women and acquired forms, C1 inhibitor concentrate is the treatment of reference. Antalgic and perfusion treatments should not be neglected, and should be modified as a function of clinical signs. High-risk situations (perioperatory period, birthing, dental care) should be identified and short-term prophylaxis put in place before any procedure that may trigger an attack. Algorithms are proposed for the diagnosis, treatment and prevention of attacks. Recommendations exist for during childbirth, in which case C1 inhibitor concentrate should be used., Conclusion: Bradykinin-mediated angioedema should be evoked in the case of recurrent and transitory oedema. Emergency management has evolved thanks to the commercialization of new molecules. Prevention of attacks during surgery and for during childbirth is important. The availability of C1 inhibitor concentrate in sufficient doses should be verified prior to the procedure. A multi-site reference centre (CREAK) has been created to help clinicians manage this disease. Patients with this disease should be identified in emergency departments. Health establishments, which cannot all have emergency stocks, should set up procedures for rapid provision or the transfer of patients to reference sites., (Copyright © 2011 Elsevier Masson SAS. All rights reserved.)

The Gleich's syndrome is a rare disease that causes recurrent angioedema associated with major eosinophilia with good response to corticosteroids. We describe a 53-year-old man who presented with a Gleich's syndrome with a 6-year follow-up and propose a literature review. This case emphazises the favourable prognosis of this disease. In case of poor tolerance of corticosteroids, mepolizumab could be tested.

We report a case of a 50-year-old man who presented with abdominal pain and shock due to an allergic angioedema of the bowel resulting from the use of a common class of anti-hypertensive medication. After initial stabilization, a computed tomography (CT) scan was performed that documented characteristic findings and saved the patient from an unnecessary laparotomy. Although rare, this etiology of abdominal pain and hypotension should be considered as more and more individuals are taking angiotensin-converting enzyme inhibitors.

Angioedema due to an acquired deficiency in the inhibitor of the first component of human complement (CI-INH) is a rare syndrome that is usually identified as acquired angioedema (AAE). The clinical features of C1-INH deficiency, which may also be of genetic origin (hereditary angioedema, HAE), include subcutaneous, non-pruritic swelling, involvement of the upper respiratory tract, and abdominal pain due to partial obstruction of the gastrointestinal tract. Unlike those with HAE, AAE patients have no family history of angioedema and are characterised by the late onset of symptoms and various responses to treatment due to the hypercatabolism of C1-INH. The reduction in C1-INH function leads to activation of the classical complement pathway and complement consumption, as well as activation of the contact system leading to the generation of the vasoactive peptide bradykinin, increased vascular permeability, and angioedema. AAE is frequently associated with lymphoproliferative diseases ranging from monoclonal gammopathies of uncertain significance (MGUS) to non-Hodgkin's lymphoma (NHL) and/or anti-C1-INH inactivating autoantibodies. The coexistence of true B cell malignancy, non-malignant B cell proliferation and pathogenic autoimmune responses suggests that AAE patients are all affected by altered B cell proliferation control although their clinical evolution may vary.

This paper describes a middle-aged patient who developed repeated episodes of swelling of the orofacial tissues after dental treatment. On investigation, C1 inhibitor, C1q, C2, and C4 levels were all markedly reduced, and a diagnosis of acquired C1 inhibitor deficiency was made. The patient had been diagnosed with non-Hodgkin's lymphoma (NHL) 2 years previously and had undergone a successful course of chemotherapy. The development of her episodes of angioedema prompted thorough reinvestigation and a recurrence of NHL was identified. Therefore, acquired C1 inhibitor deficiency heralded a recurrence, although this had not been a manifestation when NHL was first diagnosed. The patient underwent a further course of chemotherapy and remains well, although C1 inhibitor, C1q, C2, and C4 levels remain reduced.

Angioedema is characterized by transient, episodic, nonpruritic, nonpitting and well-defined edema that involves the subcutaneous or submucosal tissue, and most commonly develops in the head and neck region. Angioedema is caused by extravasation of plasma into the interstitial space of the affected tissue. We describe a six-year-old boy who developed oral and penile angioedema subsequent to the ingestion of peanuts.

Food allergies are more and more numerous, and frequent in children at all ages. At any moment, a clinical manifestation, quickly evolutive and alarming, must need the help call at phone 15, and the intervention of a medical team of SMUR. The departmental, free, cell to 15 for all medical emergencies note a regular increase of these specific calls since ten years, as this following case report shows it.

Hereditary angioedema is characterized by recurrent skin swelling, abdominal pain attacks, and potentially life-threatening upper airway obstruction. The two classic types are both caused by mutations within the complement C1 inhibitor gene. A recently described new type does not show a deficiency of C1 inhibitor and affects almost exclusively women. We screened twenty unrelated index patients with this new type of hereditary angioedema for mutations in the coagulation factor XII gene. Two different missense mutations were identified in exactly the same position within exon 9 of the F12 gene. 'Mutation 1' (1032C-->A), encountered in five patients, predicts a threonine-to-lysine substitution (Thr309Lys). 'Mutation 2' (1032C-->G), observed in one patient, results in a threonine-to-arginine substitution (Thr309Arg). The predicted structural and functional impact of the mutations, their absence in 145 healthy controls, and their co-segregation with the phenotype in five families provide strong support that they cause disease.

Objective: The purpose of this study is to report a case of acquired angioedema and Factor V Leiden mutation in a woman who had started taking an oral contraceptive (OC), and to review the literature., Results: A 44-year-old nonobese and nonsmoking woman was admitted to our hospital because of pain and progressive erythematous lesion on her left groin. The patient had had a 6-week history of combined OC use. There was no family history of angioedema. With a diagnosis of necrotizing fasciitis, surgical debridement of all necrotic tissue was considered with fasciocutaneous flap. Histopathological analysis showed diffusely fresh bleeding areas with diffused subcutaneous necrosis. The culture of the necrotic tissue was negative. Postoperatively, she restarted combined OC, and then the painful petechial and rapidly disseminated ecchymotic lesions were seen. Oral contraceptive was stopped immediately. A venous Doppler ultrasonography of the lower extremities showed subacute thrombosis in the left popliteal vein. Complement C3 level was normal, but amounts of C4, C1 esterase inhibitor level and activity were low. Factor V Leiden was positive for the heterozygous presence of Factor V gene., Conclusions: Women with angioedema and Factor V Leiden should be counseled against using hormonal contraceptives containing estrogen, and because they may interact in a synergistic manner in the pathogenesis of cutaneous lesions and/or venous thrombosis, appropriate individualized therapy should be defined. Further studies are needed.

Urticaria, angioedema and anaphylaxis are life threatening skin diseases. Allergological emergencies are common; drugs, food, food ingredients/additives, insects, and many other factors have been reported to elicit anaphylactic reactions. The severity of symptoms ranges from pruritus to generalized skin eruptions, gastrointestinal, bronchial problems to systemic anaphylaxis and cardiovascular emergencies. The pathomechanisms and treatment of urticarias, angioedema and anaphylaxis are described. In some situations emergency kit: antihistamines, steroids, betamimetics and adrenaline is needed. Familiarity with the early diagnosis and correct management should be acknowledegable for dermatologists to recognize these allergic reactions and must be prepared to administer emergency kit.

Background: Recurrent angioedema is the hallmark of various inherited or acquired angioedema diseases. Hereditary angioedema, or HAE, due to C1 inhibitor, or C1NH, deficiency has considerable implications for dental health care providers because dental surgery may trigger distressing and even life-threatening episodes., Case Description: The authors reviewed the literature, focusing on the pathogenesis, clinical signs and treatment of HAE. They also provided case reports of four patients who died from laryngeal edema induced by tooth extraction. In patients with HAE, dental surgery--including tooth extraction--may be followed by self-limiting edema episodes, including lip swelling, facial swelling, tongue edema and laryngeal edema with upper airway obstruction. Preoperative prophylaxis has been performed with attenuated androgens, fresh frozen plasma, C1NH concentrate and antifibrinolytics. The four patients described underwent tooth extraction, which, after a symptom-free latency of four to 30 hours, provoked laryngeal edema. Three of the patients died of asphyxiation the night after surgery, and the fourth died on the second night. In three of the patients, laryngeal edema had not occurred previously., Clinical Implications: Before undergoing dental surgery, patients with a history of recurrent angioedema should be evaluated for C1NH deficiency. If it is present, they are at risk of developing life-threatening laryngeal edema.

Although frequently reported as an aetiology for chronic angioneurotic oedema or urticaria, food allergy is often a diagnosis proposed in the absence of more convincing evidence, as illustrated by the disappointing results of eviction regimens. We report a series of women with an initial diagnosis of food allergy, but in whom the role of oral contraceptives was subsequently demonstrated. Detailed medical history was obtained from 26 young women presenting with chronic angioneurotic oedema or urticaria initially attributed to food allergy, but in whom C1-esterase inhibitor (C1 INH) deficiency was demonstrated. We investigated the effects of oral contraception on C1 INH levels, C1 INH activity and clinical symptoms of these patients. Discontinuation of oral contraception induced an increase in C1 INH levels and C1 INH activity, associated with recovery or marked improvement of the clinical symptoms formerly attributed to food allergy. The relatively high frequency of women taking cyproterone acetate in this population appeared to be a remarkable finding. Replacement of the initial contraception containing ethinylestradiol by a progestogen maintained or even accentuated these good therapeutic results. Exogenous oestrogens, such as those contained in most oral contraceptives, may play an iatrogenic role in the aetiology of chronic angioneurotic oedema or urticaria.

C1 inhibitor (C1-Inh) is a protease inhibitor of the serpin family. It interacts and forms complexes with several serine proteases although not all these interactions were proved to be relevant in vivo. Based on studies in deficient patients, C1-Inh appears pivotal in regulating the activation of complement classical pathway and of contact system. The best recognized consequence of defective C1-Inh function is predisposition to episodes of self-limited, increased vascular permeability (angioedema) that is restricted to three specific sites, which include the subcutaneous space, the gut and the upper airway. Candidate mediator of angioedema is bradykinin, a potent vasoactive peptide, released upon contact system activation. Mutations in C1-Inh structural gene are the most common cause of C1-Inh deficiency and lead to hereditary angioedema. Recurrent angioedema are also seen in the acquired defect of C1-Inh that is due to autoantibodies against this protein or to an associated disease causing accelerated catabolism of C1-Inh. Apart from the profound deficiency of C1-Inh characteristic of angioedema, it has been suggested that, in specific pathologic settings, C1-Inh levels in the low normal range could still represent a significant functional deficiency. Such conditions, as extensively investigated in sepsis, are of great relevance because they open the possibility of using C1-Inh as therapeutic agent in several different diseases.

Purpose: Nonallergic isolated angioedema is an uncommon clinical syndrome raising difficult diagnosis and therapeutic problems. Occurrences linked to a C1Inh are the predominant ones and have to be examined as a priority, taking into account the specificity of the associated follow-up., Current Knowledge and Key Points: Diseases with a clinical profile close to hereditary angioneurotic edema, but without C1Inh anomaly, have been described recently. It is in fact family cases, concerning only women, where estrogens seem to play a dominant role. Angioedema's secondary aspects are gathering various pathologies (vasculitis, Gleich's syndrome, angioedema initiated by physical agents). The role played by some drugs must not be forgotten, mainly angiotensin converting enzyme inhibitors, which are at the origin of angiodema in nearly 0.5% of users., Future Prospect and Projects: Uncontrolled activation of the contact system seems to play a major role in the main part of these angiodemas. The efficiency of the tranexaminic acid (which modulates its activation) is to be taken as evident. The key to the future seems to be the development of plasmin and bradykinin inhibitors.

We report the first documented case in the literature of hereditary angioedema presenting after commencement of estrogen replacement therapy for menopausal symptoms. The late presentation of the disease and the precipitation of attacks by physiological doses of estrogen replacement therapy make this a highly unusual case. The pathophysiology of hereditary angioedema and its hormonal links are discussed.

Alpha1-antitrypsin deficiency results from point mutations that distort the structure of the protein to allow a unique protein-protein interaction that we have termed loop-sheet polymerization. Polymers of Z alpha1-antitrypsin accumulate within hepatocytes to form inclusion bodies that are associated with juvenile cirrhosis and hepatocellular carcinoma. The lack of circulating protein predisposes the Z alpha1-antitrypsin homozygote to emphysema. This polymerization process also occurs in variants of other members of the serine proteinase inhibitor (serpin) superfamily, antithrombin, C1-inhibitor and alpha1-antichymotrypsin in association with thrombosis, angiooedema and chronic obstructive pulmonary disease respectively, and we have recently shown that it underlies a novel inclusion body dementia. Understanding this mechanism of polymerization allows rational drug design to block the protein-protein linkage and so ameliorate the associated disease.

Unlabelled: Anaphylactic reactions after consumption of squid by patients sensitized to house dust mites have been reported several times., Case Report: A child allergic to dust mites developed an angioneurotic edema after eating squid. An immunoallergological assessment, including the prick test, labial test and IgE RAST revealed an allergy associated to both dust mites and squid., Conclusions: In light of the potential seriousness of anaphylactic reactions, parents of children allergic to dust mites and these children should be made aware of the increased risk of allergies to squid that they may face.

Food hypersensitivity is increasing, with clinical indications and allergens multiplying and evolving. We report our experience with clinical indications and distribution of allergens in children with food hypersensitivity. Data were established in a prospective study at medical centres in Nancy and Toulouse (France). We studied 378 children with food hypersensitivity indicated by food challenge, which account for 74.2% of food hypersensitivity. Clinical features were: atopic dermatitis (46.5%), urticaria (17.9%), oedema (14.2%), asthma (8.4%), anaphylaxis (5.2%), gastro-intestinal symptoms (2.1%), oral syndrome (1.8%) and rhino-conjunctivitis (0.5%). Five allergens accounted for 82% of confirmed food hypersensitivity: egg (51.8%), peanut (34.3%), milk (11.6%), mustard (8.9%) and codfish (7.1%). Allergens according to the symptoms showed that peanut allergies were more serious than other food allergy. Allergens according to age showed that peanut allergy is the first food allergy occurring after the age of three. There exists a modification in children's allergen distribution. Peanut allergy is increasing seriously, and diagnosis with food challenge is a necessity.

People deficient in C1-INH present recurrent angioedema localized to subcutaneous or mucous tissues. The defect can be caused by impaired synthesis, due to a genetic defect (hereditary angioedema), or by increased catabolism (acquired angioedema). In our experience the majority of patients with acquired angioedema (16 of 18) have autoantibodies to C1-INH in their serum. These autoantibodies bind to C1-INH with different and generally low affinity. The vasopermeability mediator responsible for attacks is still undefined: bradykinin (derived from cleavage of high molecular weight kininogen) and a kinin-like peptide (derived from the second component of complement) still remain the two primary candidates. We examined the systems controlled by C1-INH (complement, contact system, fibrinolysis and coagulation) and found that all of them are activated during angioedema attacks. Activation of the coagulation leads to generation of thrombin whose vasoactive effect can thus influence edema formation. Treatment of severe angioedema attacks is satisfactorily performed with C1-INH plasma concentrate although patients with an acquired defect frequently need very high doses. Attenuated androgens effectively prevent attacks in hereditary angioedema, but their safety, on the very long-term, needs to be further assessed. Acquired angioedema generally fail to respond to these drugs, but can be treated prophylactically with antifibrinolytic agents.

Background: Anaphylactic reactions after consumption of snails by patients sensitized to house-dust mites have been reported several times., Case Report: Two 8- and 10-year old children sensitized to house-dust mites developed Quincke's oedema after eating snails. Immunoallergologic investigations including pricks-test, labial test, IgE Rast confirmed associated allergy between snails and house dust mites., Conclusion: Considering the potential severity of anaphylactic reactions, it is necessary to warn children allergic to house dust mites and their parents about the high risk of associated allergy with snails.

A postulated role of the contact system in anaphylactic reactions to insect stings was investigated. During prospective, in-hospital sting challenge, we collected serial blood samples from five normal volunteers and 16 patients with a history of insect-sting anaphylaxis. Activation of the contact system was assessed by measuring plasma levels of factor XIIa-C1-inhibitor and kallikrein-C1-inhibitor complexes as well as those of cleaved high molecular weight kininogen (HK). In addition, antigenic levels of (pre)kallikrein, factor XII, and HK were measured. No significant changes in contact system parameters were observed in any of the five volunteers or the four patients who did not develop an anaphylactic reaction after sting challenge. In contrast, significant changes in contact system parameters occurred in 7 of the 12 patients with anaphylactic symptoms after challenge. Peak levels of either C1-inhibitor complex were found 5 minutes after the onset of anaphylactic symptoms. The increase in C1-inhibitor was most pronounced in the 4 patients with angioedema, 2 of which also developed shock. However, activation of HK was observed in all four patients with angioedema, the two patients with shock but no angioedema, as well as in 1 of the remaining 6 patients with anaphylactic symptoms other than angioedema or shock. Thus, activation products of the contact system may be involved in the pathogenesis of angioedema and shock in insect-sting anaphylaxis.