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Monogenic autoinflammatory diseases are a heterogeneous emergent group of conditions that are currently under intensive study. We review the etiopathogenesis of these syndromes and their principal manifestations. Our aim is to propose a classification system based on the clinicopathologic features of typical skin lesions for routine clinical use in dermatology. Our focus is on diagnosis in pediatric practice given that this is the period when the signs and symptoms of these syndromes first appear. In Part 1 we discuss the course of urticaria-like syndromes, which include cryopyrin-associated periodic conditions and hereditary periodic fever syndromes. Pustular syndromes are also covered in this part. Finally, we review the range of therapies available as well as the genetic mutations associated with these autoinflammatory diseases., (Copyright © 2017 AEDV. Publicado por Elsevier España, S.L.U. All rights reserved.)

Chronic spontaneous urticaria is a disorder mediated by mast cells, characterized by the development of wheals, angioedema or both, lasting six weeks or more, with or without a known trigger agent. First and second line treatment are antihistamines, but some refractory cases require other alternatives, such as omalizumab. Searching in Epistemonikos database, which is maintained by screening 30 databases, we identified four systematic reviews including five pertinent randomized controlled trials overall. We combined the evidence using meta-analysis and generated a summary of findings following the GRADE approach. We concluded omalizumab reduces symptoms and improves quality of life in patients with chronic spontaneous urticaria.

Background: Chronic urticaria is characterized by swelling and itchy hives presenting remissions and exacerbations, for six weeks or more, which may or may not be accompanied by angioedema. Helicobacter pylori infection has been associated with various diseases among them chronic urticaria., Objective: To determine the association between H. pylori infection and allergic and non-allergic chronic urticaria., Material and Method: We conducted a retrospective, observational and cross-sectional study which included 82 patients of both sexes with a diagnosis of chronic urticaria, aged between 2 and 75 years. Total serum IgE and IgG anti-H. pylori were measured in all participants. Statistical analysis was performed using the chi-squared test with 0.05 significance., Results: A total of 82 patients, 33 males and 49 females, with a mean age of 36.9 years were studied. H. pylori seropositivity was in 65.2% of allergic patients and 34.8% of non-allergic patients (p = 0.148)., Conclusions: We found no association between chronic allergic urticaria and H. pylori infection.

The pine processionary caterpillar is the larval form of the Thaumetopoea pityocampa moth. Mediterranean forests regularly suffer plagues of this insect, which has been moving north as a result of global warming. When the small urticating hairs that develop during the last 3 larval stages are shed and can become airborne. If they come in contact with skin, they can cause a variety of reactions, notably contact urticaria and papular rashes. Irritation can also occur if the hairs lodge in the mucosa of the conjunctiva or in the respiratory tract. Several cases of anaphylactic reactions have been reported in recent years. Mechanical (irritative) mechanisms may be involved in the pathogenesis of lesions, or immunoglobulin E-mediated allergic hypersensitivity reactions may be implicated when the process is rapid, recurrent, and progressively more severe., (Copyright © 2011 Elsevier España, S.L. y AEDV. All rights reserved.)

Introduction: Papular urticaria caused by the bites of fleas traditionally has been defined as a chronic allergic disease. However, currently no clear relationship has been described between this pathology and common allergic diseases., Objective: The expression of IL-10, IL-4 and IFN-γ as markers of effector T cell responses was examined in skin lesions of patients with papular urticaria by flea bite., Materials and Methods: Fourteen skin lesion biopsies were sampled from children with a clinical diagnosis of papular urticaria by flea bite and were compared with 5 healthy skin biopsies of children with no history of the disease. All children were under 12 years old. RNA was extracted with trizol and the expression levels of cytokines were analyzed by real time PCR technique., Results: A wide range in the expression levels of IFN-γ and IL-10 was noted as well as constant low values of IL-4. Three distinct profiles were observed, but which did not correspond to a recognizable pattern among the patients. The samples obtained from healthy tissues showed no expression of any of the cytokines., Conclusions: This is the first characterization of cytokines that mediate the immune response at the site of the skin lesion in children with papular urticaria by flea bite. The data indicated that the local response was mixed and that a single phenotype is not predominant among the patients.

Unlabelled: We report the case of a child with diagnosis of chronic urticaria/angioedema and its evolution upon omalizumab treatment., Case Report: Our patient is a 12-years-old female who suffered for 14 months severe chronic urticaria/angioedema. She had a poor response to the highest doses of combined therapy with 3 antihistamines, steroids and anti-leukotrienes and great impairment of her quality of life. An autologous serum skin test was positive until 1:100 dilutions, leading to the diagnosis of chronic autoimmune urticaria. Due to the lack of response to treatment, therapy with omalizumab was administered. A notable reduction in symptoms toward the third dose was observed. After 12 months of this treatment, the patient is asymptomatic and has a negative autologous serum test., Conclusion: Omalizumab could be a therapeutic option for patients with autoimmune urticaria unresponsive to other treatments.

Urticaria is considered a heterogeneous group of diseases that share different patterns of skin reactions. The wide diversity in urticaria subtypes have been identified and this reflects partial understanding of the causes or factors that trigger it, as well as the molecular and cellular mechanisms that are involved in their physiopathology. The objective of this article was to make an extensive review of the literature to be able to offer the readers a complete information and updating on the basic, ethiologic and physiophatologic mechanisms and mainly to make a special emphasis on diagnosis and treatment of urticaria, promoting the continuous medical education.

Background: Chronic urticaria may be continuous or recurrent according to its form of appearance. Within the diseases associated to chronic urticaria there are mycosis, parasitism and bacterial infections where Helicobacter pylori stands out. This has been related to the allergic diseases promoting a Th2 response., Objective: To determine the frequency of infection caused by Helicobacter pylori in patients with chronic urticaria of the allergic and clinical immunology service of the Puebla University Hospital., Patients and Methods: A descriptive, cross-sectional, prolective and observational study was made in adult patients, between 18 and 60 years old, with diagnosis of chronic urticaria. Inhalated and food skin prick test were made to all the patients. The infection by Helicobacter pylori was documented by serology, fecal antigen, endoscopy with fast test of urease and histological study. Descriptive statistics was implemented such as frequency, percentage, central tendency and dispersion measures., Results: 30 patients were included; 83.3% were women. The average age was 37.8 years (SD 13.2). The most frequent type of chronic urticaria was the persistent one, representing 56.7%. The frequency of positivism of IgG antibodies against Helicobacter pylori represented the 60.0%, for IgM 33.3% and for fecal antigen 60.0%, the combined IgG, IgM, fecal antigen, fast test of ureasa and histological study was of 83%., Conclusion: The high frequency of infection caused by Helicobacter pylori in the patient with chronic urticaria suggests a possible role in its etiopathogeny, extending the therapeutic possibilities.

Objectives: To determine the incidence and nature of adverse events associated with the induction of rush Hymenoptera venom immunotherapy., Material and Methods: Between 1998 and 2003, we administered venom immunotherapy to 48 patients allergic to bee or wasp venom, by means of a rush immunotherapy protocol (3 days)., Results: We observed no severe adverse reactions in any patients. 12 patients developed only local reactions at the site of injections that did not required any pharmacological treatment. Two patients experienced mild systemic reactions consisting of diffuse urticaria on day 3. Both adverse reactions were treated with intravenous antihistamines., Conclusions: Our experience confirms that rapid venom immunotherapy is safe and should be considered in every case especially for patients during the stinging insect season when a rapid protection is required.

Chronic urticaria is a frequent pathology, characterized by the presence of hives and/or angioedema lasting longer than 6 weeks. In an important number of patients it behaves as an autoimmune illness, frequently associated with alterations in thyroid function and thyroid antibodies. We herein describe a consecutive series of 70 patients with a diagnosis of chronic urticaria. Seven (10%) had a diagnosis of thyroid illness previous to their first consultation. Thyroid function and thyroid antibodies were studied in the remaining 63 patients by measuring the level of serum thyrotropin and the titer of peroxidase antibodies. Abnormal thyrotropin levels were detected in 11 (17%) patients, who in conjunction with the 7 patients with previous thyroid illness, add up to 18 (26%) with altered thyroid function. From 61 patients who were tested for thyroid peroxidase antibodies, 22 (36%) were positive. Of 57 patients without a diagnosis of previous thyroid disease, in whom both the levels of serum thyrotropin and the presence of thyroid peroxidase antibodies had been studied, 24 (42%) presented at least one altered study. Given the high percentage of thyroid alterations in our series of patients, it seems clinically relevant to study the thyroid function and the presence of thyroid peroxidase antibodies in patients with chronic urticaria.

Background: It has been established that 27-50% of patients with idiopathic chronic urticaria have antibodies directed against the alpha chain of the high-affinity IgE receptor, which are indirectly detected by cutaneous tests with autoserum. Thus, an autoimmune urticaria diagnosis can be settled., Objective: To prove methotrexate's efficiency in patients with autoimmune urticaria., Material and Methods: Seven patients took part in the study. A series of tests was performed in order to rule out any possible infectious, metabolic, or physical etiology. Initial treatment with methotrexate with doses of 2.5 mg every 12 hours, two days a week was provided. In case there were no toxicity data, doses would increase to three days a week for a 6-week period., Results: Statistically significant improvement was observed in the itching, as well as the presence of spots, repercussion on daily activities, sleep disorders. There was no statistical difference regarding the extension of the lesions and the presence of angioedema. Adverse effects were not significant., Conclusion: We conclude that methotrexate is effective in the treatment of autoimmune urticaria.

Background: Chronic urticaria is characterized by erythematous wheals during more than 6 weeks. In 47% of the patients it is associated to Helicobacter pylori infection; in 50%, to antibodies (Abs) against the high affinity receptor of the IgE, and in 12 to 20% to antithyroid's antibodies (antithyroglobuline, mychrosomals) and, from these, 25% have alterations of the thyroid function., Objective: To determine the presence of the anti-Helicobacter pylori and anttihyroids antibodies and the high affinity anti-receptor of the IgE in healthy subjects and patients with chronic urticaria., Material and Methods: Eighty subjects were included: 40 healthy subjects and 40 patients whit diagnosis of chronic urticaria. In all of them a blood sample was extracted to the determination of the antibodies and skin test applications with autologous serum to determine the antibody of the high affinity anti-receptor of IgE. For the analysis of the results descriptive statistics was used (central tendency measures, frequencies, means, averages and percentages), as well as multiple correlations and inferential analyses., Results: Anti-Helicobacter pylori, antithyroid (antithyroglobulin, microsomal) antibodies and the skin test with autologous serum (antibody of the high affinity anti-receptor of IgE) were negative in the healthy group (mean age: 41 years, 36 women, 90%). Of the 40 patients with chronic urticaria (mean age: 42 years, 36 women, 90%), the frequency of the antibody of high affinity anti-receptor of the IgE was: 5 patients with negative skin test (12.5%), 1 (2.5%)+, 7 (17.5%)++, 9 (22.5%)+++, 18 (45%)++++. The anti-Helicobacter pylori antibodies were positive in 30 (75%) patients and negative in 10 (25%). The antithyroid's antibodies were as follows: antithyroglobulin: 4 (10%) positive, microsomal: 4 (10%) positive. The concomitant diseases found were: hypothyroidism (3, 7.5%) mixed rhinitis (2.5%) and autoimmunity 2 (5%)., Conclusion: The test skin with autologous serum to determine the IgG antibody against the high affinity receptor of IgE is a simple and low-cost procedure leading to determine the cause of the idiopathic chronic urticaria in a high percentage of patients. As reported in literature, the Helicobacter pylori infection documented by the detection of the IgM antibodies against Helicobacter pylori is frequent in patients with chronic urticaria, which is important due to it could be implied in the diagnosis and treatment.

Recent advances on the pathogenesis of chronic urticaria have defined a group of patients with autoantibodies directed to the IgE or to the alpha chain of the Fc high affinity receptor of IgE, Fc epsilon RI alpha. These antibodies are detected in vivo through the autologous serum test (AST) and in vitro with a variety of techniques. We here describe 37 patients with chronic urticaria, 28 female and 9 male, with a f/m ratio of 3.1. Mean age at onset was 36.5 years (range 16-78). AST was positive in 25 (68%) of 37 patients. Serum induced a wheal significantly larger than plasma (122 +/- 78 mm2 vs 57 +/- 66 mm2, p < 0.05). Median persistence of the chronic urticaria, estimated by Kaplan-Meyer analysis, was 437 days, with no difference between AST(+) and AST(-) patients (437 vs. 369, p = 0.18). Mean IgE concentration was 157 +/- 173 IU/mL, as expected in an unselected population. Basophil count was lower in patients compared with controls (17 +/- 12 cel/microL vs. 43 +/- 27 cel/microL, p < 0.008). Only sera from 2/7 (28.6%) patients AST (+) and very low basophil count consistently induced expression of CD63. This effect was abrogated in non-releasing basophils, confirming the presence of antibodies directed to the Fc epsilon RI alpha-IgE. We conclude that functional antibodies are present in only a minority of patients and that their identification does not predict the outcome.

Background: Cold urticaria is caused after exposition to cold air, water and food. It is the third more frequent physical urticaria in pediatric population., Methods and Results: We reviewed twelve patients, studied different characteristics and obtained following results: mean age is 12 years and 9 months and it is more frequent in female subjects, atopy is present in 67 % of patients, other physical urticaria are present in 25 % and there is not familial inheritance. 83 % of patients have localized and generalized symptoms. Cold stimulation test is positive in 92 %. Cryoglobulins and cold agglutinins are negative in 100 % of patients in which these tests were made. There is infectious disease in only two patients. Cetirizine was used in most of patients and it was successful in 70 %. Mean duration is 3 years and 6 months. Only patient with negative cold stimulation test remains without symptoms., Conclusions: Cold urticaria must be initially diagnosed by cold stimulation test and clinical history. Cetirizine is effective and cause less adverse effects than other antihistamines traditionally used.

We performed a prospective crosswise analytical study of 84 patients treated in the Allergy Service of "Calixto García" General Hospital whose skin tests to allergenic Blomia tropicalis extract were positive. These patients' sera were used in assays for determining specific IgE antibodies and in the identification of allergen proteins in this extract by means of western blotting. Forty-four patients (55%) showed specific IgE levels higher than 0.1 U and 17 patients exhibited levels over 0.3 U. Bronchial asthma and rhinitis were the pathologies with elevated specific IgE levels. However, generally speaking, the specific IgE values were lower than those reported in other mites in spite of the fact that skin reactivity behaved the same. It was determined that specific IgE antibodies are mainly directed to allergens with a molecular weight from 13 to 15 D.

Urticaria and angioedema are common diseases in children and adults. Approximately 15-25% of the population will have urticaria or angioedema at least once in their life-time. Urticaria is characterized as the appearance of erythematous, circumscribed, elevated, pruritic, edematous swelling of the upper dermal tissue. Erythematous swelling of the deeper cutaneous and subcutaneous tissue is called angioedema. In angioedema lesions are less pruritic but pain and burning are common. Urticaria may occur in any part of the body, whereas angioedema often involves face, extremities or genitalia. In contrast to other forms of edema there are not symmetric distribution. Urticaria and angioedema are often associated. Urticaria is considered acute if symptoms are present for less than 6 weeks, but usually in childhood lesions disappear in a few days. In chronic urticaria symptoms are longer than 6 weeks; if the episodes were of shorter duration than the symptoms-free periods urticaria is considered recurrent. Acute urticaria has been reported to be the common type in childhood and chronic urticaria is more frequent in adults. Acute urticaria is usually a self-limited benign disease in young children. Nevertheless it is an uncomfortable nuisance, interfering daily activities and sleep, and produces psychosocial impact in patients and parents (an altered self-image is always an alarming situation). Urticaria is a frequent cause of emergency room visit but few patients need to be admitted. Urticaria has long been believed to be an allergic disease but clinically it has rare been proved to be so. The basic mechanism involves the release of diverse vasoactive mediators that arise from the activation of cells or enzymatic pathways. Histamine is the best known of these substances, and elicits the classic triple response consisting of vasodilatation (erythema), increased vascular permeability (edema) and an axon reflex that increases reaction. In contrast to simple symptoms and easy diagnosis of urticaria, etiologic factors are often difficult to establish. Urticaria can be classified according to the eliciting factors and the different pathomechanisms. According to several works, clinical history carried out by a trained physician can be regarded as the most valuable diagnostic tool and extensive screening test do not contribute to etiologic diagnosis of urticaria. Only a few specific tests appeared to be valuable at this respect. In different studies about children urticaria, the most common etiological factors have been identified as infection, physical urticaria, food allergy, drug adverse reaction, parasitic infestation and papular urticaria. The aim of this work-shop is to define, describe and discuss these frequent problems.

Objectives: 1) To study the clinical and analytic features of infectious disease associated to urticaria in children. 2) To look into the probable etiology of the infectious disease. 3) To determine atopic predisposition and previous urticarial episodes and to rule out the involvement of antibiotics., Design: Transversal and observational study., Setting: Pediatric Allergy Outpatient Clinic of a tertiary Hospital., Patients: Forty-four children, aged 1 to 12 years with acute urticaria associated to clinically infectious or febrile illness attending an Emergency Pediatric Department., Intervention: Symptoms evaluation and physical examination in the seventh first days and follow over 3-6 weeks by the same physician., Measurements: Clinical features of urticaria (duration, angioedema associated); Clinic diagnosis of illness infectious (acute respiratory infection, gastroenteritis, febrile syndrome); white blood cells count, C-reactive protein, aminotransferases (AST, ALT), L-Y-glutamyl transferase; viral culture and antigen detection: enterovirus (EV), adenovirus, respiratory syncytial virus (RSV), parainfluenza 1, 2 and 3, influenza A y B and cytomegalovirus (CMV); serological assay: CMV, enterovirus, mycoplasma pneumoniae, Epstein-Barr, parvovirus B19., Result: 22 children (50%) are between 1-2 years old. 40 patients (90,9%) had symptoms of respiratory tract infection and only four patients had a pneumonia. The other 4 children had a gastroenteritis. The analytic was suggestive of viral infection in 35 (79.5%) and unknown on seven patients. In 20 children (45.4%) was identified a probable infection. The viral detection was positive in 3 patients: CMV, herpes simplex 1 and influenza A. Twenty microbiological findings for seventeen patients was found by serological criterion of probable infection: enterovirus (10); parvovirus B19 (4); Epstein-Barr (3) y mycoplasma (3). Evidence of a double serologic infection was found in three patients. In comparison with a serological control group encountered that acute urticaria during a infectious disease is significantly associated (p = 0.0054) to high titer to enterovirus by complement-fixation. The urticaria was associated with angioedema in 38.6% and 9 children (20.4%) related an previous similar episode. Twenty-one (47.7%) had been treated with antibiotics before development the urticaria. All patients was given the suspected antibiotic and no patient developed any adverse reaction., Conclusions: The clinically infectious associated to urticarial rash in children, usually is a viral respiratory infections. Is more frequent at infant. In spite of antibiotic therapy is often related to development the urticaria, the subsequent challenge with the same antibiotic is good tolerated.

Urticaria is characterized by the appearance of hives and pruritus. Those hives are formed by oedema and vasodilatation and they disappear when they are pressed on. The acute presentation is extremely common and affects between 10 and 20% of the population at a determined moment. In its simplest form, urticaria is envisioned to represent the same sort of wheal-and-flare reaction observed when histamine is injected into the skin. It produces erythema because of capillary vasodilatation, oedema because of increased permeability in capillary and pruritus secondary at local specific receptors stimulation. Angioedema is caused by the same pathologic alterations that occur in the deep dermis and subcutaneous tissue. Thus, an area involved with angioedema has swelling as the prominent manifestation and appearance of the skin itself may be normal. Due to reduced nerve supply in dermis, angioedema is associated with oppression and not pruritus. Immunoallergological study of urticaria and/or angioedema was requested in 133 cases from 648 from the first patient's visits to the surgery. It supposes a 20.52%. The family suspicion of etiology was food in 62 cases, chemical products in 39 cases, other factors (physical, stings, balloons and other manufactured products.) in 7 cases and 25 cases without a direct relation. Out of 100 children diagnosed of allergic urticaria-angioedema 67 was by food; the foods implicated in frequency order were: eggs and nuts, fruit, milk, vegetables, fish and shellfish. In second place, chemical products were responsible of urticaria in 12 children; five of them were positive in diagnosed proof (prick, oral challenge) for penicillin and amoxicillin, both from beta-lactamic group; two of them had and adverse reaction to anesthetic agents; other two cases were after administration of vaccination and due to tetanus toxin; and three cases were due to aspirin, confirmed by oral provocation test. In 10 children the etiological agent was latex. Other etiologies were: three cutaneous reactions after stings (two by wasps and one by mosquito) three reactions due to spices (paprika, cumin, anise, mustard) and two reactions caused by manufactures products containing additives as yellow-orange.

Aims: The chronic urticaria and the angioedema affect to the population's wide sectors and up to now their cause is ignored., Material and Method: They were studied during one year 50 mature patients with chronic urticaria coming from the ambulatory consultation of the service of alergología of the Hospital CQ Hermanos Ameijeiras. The diagnosis of chronic urticaria settled down clinically for observation of the lesions and for the time of its evolution that should exceed six weeks. It was included as group witness to 26 healthy individuals., Results: They were high levels for the IgE seric in the patients' 70%; you even normal of IgA, IgG and IgM in the great majority of the cases. The determination of circulating immunocomplexs gave negative in 88% of the sick and only positive in 12%. The tests for cutirreacción with extracts alergénicos micóticos, in reading at the 48 hours, were not negative in a significant way, among the group of sick and in the group witness.

The urticaria common sickness is a multifactorial etiology which in a lot of occasions can't be determined in spite of the exhaustive investigation of treatment doesn't solve definitely the symptomatology, because of they have protocols of investigation research that orients us to find the common causes , the diagnostic methods to follow in each case and of this trained or form offer effective treatment that gain relief in definitive the sickness. This revision make mention of the classification etiological of the urticaria, history concepts, frequency, histopathology, chemical mediator and immunologist that participate in and the evaluation diagnostic primary to realize in the patient with urticaria acute and chronic.

This research reports the unique case of manifestation of symptomatic dermographism which includes all of its twenty five members of the family. Probing a genetic transmission autosomal dominant manner and taking into account the highly probable role of HLA in this illness. The symptomatic dermographism correspond to a group of physical familiar urticarias with symptoms appearing in early childhood and evolving throughout adulthood with seldom periods of remission. Due to the fact that all patients were affected, it was not possible to realize a typification of HLA antigens with persons not affected, to establish a relationship with the data obtained. However, results show a significant association of haplotypes; HLA A2 (78.95), B16 (68.4%), A1 (47.3%), and B5 (42.5%) among the most important. This is the first research that reports the analysis of HLA within the physical familial urticarias.

A study of 100 patients with chronic urticaria by Giardia lamblia parasitic infection were reviewed. The urticaria had been resistant to antihistamine, corticosteroid and antiparasitic therapy. An urticaria evaluation included CBC, immunoglobulins M, G, A and E determinations, and stool examination, bile drainage and duodenal smear for ova and parasites. A prick test with Giardia's allergen 1, 10 and 100 UNP dilutions were realized. We found a 93% improvement in clinical symptoms using allergy immunotherapy with Giardia's antigen preparation associated to treatment.

Twelve cases of chronic urticaria with histopathologic features of lecocitoclastic allergic angitis are studied. The type of cutaneous lesion, personal and familiar atopic history and the presence of autoimmune disease are described. Light microscopy, direct immunofluorescence, anti DNA, antinuclear, antithyroid, Ro, La, Rnp and Sm antibodies, total complement levels, C3 and C4, rheumatoid factor, latex, ASTO, cryoglobulines and complete workup were investigated, taking into account natural progression and response to therapy. Two different groups are defined: 1) normocomplementemic (5 patients) and 2) hypocomplementemic (7 patients). They were all women except one. The cutaneous lesions were indistinguishable in the two groups. Only in the second group there was an associated disease (systemic lupus erythematosus, Sjogren syndrome disease, lupus-Sjogren overlap, autoimmune thyroid disease). Urticaria had been present from the onset of the disease in 4 patients, and occurred later during its course in 8 others. Five patients had thyroid disease (Hashimoto thyroiditis or Graves disease), two of them being mother and daughter. Another patient had a family history of Grave's disease and urticaria. Anti DNA antibodies were found in 7 cases, and anti Ro + La + in 3 cases. Response to treatment was variable with spontaneous cycles of worsening and remissions. One of the patients found a relationship with certain foods. Histopathologic results are related in both clinical normocomplementemic and hypocomplementemic groups. No significant differences were found between the two groups, but Ro+ and La+ patients exhibit more intense cariorexis and neutrophilic infiltrates.

12 cases of urticarial vasculitis in 6 males and 6 females of average age are studied. Cutaneous lesions are accompanied with fever (3 cases), arthralgias (5 cases), renal manifestations (2 cases) and gastrointestinal pain (2 cases). The majority presented elevated VSG and increment of the alpha-2-globulin. Histopathologically, we find two different patterns: leukocytic-classic vasculitis and lymphocytic vasculitis of small vessels. Clinical characteristics give up with dapsone better than with doses of corticoids. We consider the influence of climatological agents (fundamentally solar exposition) and we compare our cases with the literature.

The acquired form of cold induced urticarial syndrome can be found associated with serum cryoproteins, in idiopathic form (generally IgE mediated) and transitory forms associated with other factors. The viral infections, specially infectious mononucleosis and hepatitis B can cause urticaria, mostly chronic, although infrequently produces cold urticaria. We present a case of a 13 year old patient with history suggestive of cold urticaria wherein we have found the existence of a mixed polyclonal cryoglobulinemia, IgG-IgA (exceptionally associated) and serologic markers of hepatitis B, HBsAb and HBsAb (the last being suggestive of a recent infection) 3 months from the urticaria, without recent or past history of hepatitis B infection. We also observed an elevated total serum IgE and peripheral blood eosinophilia. The provocation test presented an evolution similar to the cryoglobulinemia and markers of hepatitis B (after 18 months were negative) but serum IgE and eosinophilia remain elevated until the present time. All of this make us think that the patient could have suffered a subclinical form of hepatitis B which triggered off a cryoglobulinemia, presenting as cold urticaria.

Considering the high incidence of chronic urticaria among female patients and the frequent difficulty in identifying the etiologic factor of factors the author decided to investigate the possible role of Candida albicans and other yeasts usually found as contaminants in certain foods and beverages or purposely cultivated for industrial products, as the sensitizing agents leading to the clinical picture of chronic urticaria. One hundred female patients with urticaria which had persisted for more than 6 weeks were selected and investigated, disregarding those with dermographism or cholinergic and cold urticaria. Aside from a careful history and laboratory tests to complement the physical examination that could rule out chronic bacterial infectious foci, intestinal parasitic infestation and thyroid disorders, intradermal skin tests with standard doses of Candida albicans and Saccharomyces cerevisiae and other common environmental and food allergens were done. The patients' age ranged from 4 to 70 years. The skin tests sites were examined for Type I reactions at 15 and 20 minutes; for Type III reactions at 8 and 12 hours; and for Type IV reactions at 48 and 72 hours. When tested with Candida albicans antigen, 35% had Type I/III reactions and 60% presented Type IV reaction. When Saccharomyces cerevisiae antigen was used for testing, 29% had Type I/III reactions and none presented Type IV. Forty-nine of the sixty patients who presented Type IV reaction to Candida albicans had in the past significant vaginal discharge (or vaginal symptoms: burning, itching) that obliged the patients to consult a gynecologist, but only ten had stained smears and cultures from the vaginal secretions and four were told to have a monilia vaginal infection confirmed by the microbiological tests, although forty of them received Nistatin therapy at the time of the gynecological complaints. At the time the patients were seen by the allergist, complaining about urticaria, only four had symptoms and signs of monilia infection and were confirmed by culture: one presented oral moniliasis following broad-spectrum antibiotic, two had vaginal moniliasis developing right after their menstrual period; one had intestinal and cutaneous manifestations (perineal and crural) developing also after broad-spectrum antibiotic therapy. All the four patients had exacerbation of the urticaria while undergoing the monilia infection. After 1-2 weeks of elimination diet, each patient was challenged with yeasts-containing foods (bread, buns, sausages, beer, wines, grapes, cheese, vinegar, tomato catsup). Twenty-five patients (71%) of the group who positively reacted with a Type I/III reaction when tested with Candida antigen, showed a positive provocation test (reappearance of urticaria) and twenty patients (69%) of the group who reacted with Saccharomyces had a positive challenge test...

Urticaria and angioedema are common disorders that affect approximately 1/5 of the population at some stage in their lives. Although the clinical diagnosis of this disorder is made without difficulty, its etiology and pathogenesis remain obscure. Extensive clinical studies and the application of different laboratory techniques seldom manage to detect the etiology of chronic urticaria or determine its supposed pathogenic mechanism. Thus, a large number of causal agents have been held responsible for the symptoms of chronic urticaria and/or chronic urticaria and angioedema and for the variety of immunologic and nonimmunologic factors involved in its pathogenesis. The controversial laboratory findings in this type of patients prompted us to undertake this study on 48 subjects with chronic urticaria and/or angioedema. We studied the levels of serum immunoglobulins (IgG, IgA, IgM and IgE), the haemolytic activity of the complement (CH50), the levels of the complement components (C1 inhibitor, C3 and C4), the possible presence of circulating immune complexes and the percentages of the T lymphoid subpopulations (using OKT3, OKT4 and OKT8 markers) and the B population. The results obtained showed an alteration of the complement (CH50) and of some of its components (C3 and C4) as well as of the cytotoxic/suppressor lymphocyte subpopulation (OKT8).

This is an update article in urticaria. Its content try to summarize in few words all the practical knowledge that we have of these diseases "up to now". An exhaustive reference of the bibliography is avoided although the revision has been done indeed. We feel that if such is necessary in some papers, do difficult the reading in others like this, exclusively conceived for teaching.