Vibratory Angioedema Subgroups, Features, and Treatment: Results of a Systematic Review.

BACKGROUND: Vibratory angioedema (VA) is a subtype of chronic inducible urticaria that manifests with erythematous wheals or angioedema after skin exposure to vibration. Because the condition is rare, the available information is limited. OBJECTIVE: To systematically review the clinical manifestations and treatment options of VA. METHODS: Relevant literature published until August 2020 was searched using the terms "vibratory urticaria," "vibratory angioedema," "vibratory-induced angioedema," and "vibratory-induced urticaria." Preferred Reporting Items for Systematic Reviews and Meta-analysis recommendations were applied to this systematic review. RESULTS: On the basis of review of 22 studies (16 case reports, 4 case series, and 2 cohort studies) that had a combined total of 83 patients, we propose that VA be classified as hereditary VA (33.7%) and acquired VA (66.3%). Vibration-induced itching was frequent in both subgroups. Patients with hereditary VA more commonly had wheals and systemic symptoms, whereas patients with acquired VA more frequently had angioedema, burning, pain, or tingling. Although many VA treatments are used, there is little information on their efficacy. Most patients do not achieve complete control. CONCLUSIONS: The novel VA classification proposed could help clinicians with the diagnostic workup of patients with VA. Because of the paucity of reported cases, firm recommendations for the treatment of VA are currently not possible. For patients with acquired VA, we suggest second-generation H1-antihistamines as the first-line treatment. Controlled therapeutic trials are needed and should be performed.

Vibrational angioedema: considerations for oral health care providers.

Vibratory angioedema (VA) is a rare, inherited type of physical urticaria characterized by acute onset of mucosal and / or cutaneous edema that is triggered by vibration. We report a case of a 63-year-old woman with VA elicited by use of mechanized (vibratory) dental equipment. To our knowledge, this is the first report of VA induced by mechanical dental equipment.

Amitriptyline and bromazepam in the treatment of vibratory angioedema: which role for neuroinflammation?

Vibratory angioedema is a rare form of physical urticaria, hereditary or acquired, which occurs at body sites exposed to vibrations. Pathogenic mechanisms of disease are not completely clear and, consequently, current pharmacological treatment is sometimes unsatisfactory. We report the case of a horn player affected by acquired vibratory angioedema, relapsing after prolonged use of the instrument and resistant to systemic antihistamines and corticosteroids, which successfully responded to therapy with low doses of amitriptyline and bromazepam. A neuroinflammatory mechanism can be likely implicated in the pathogenesis of vibratory angioedema, in line with many different cutaneous/mucosal diseases involving a complex interplay of homeostatic/allostatic systems. Furthermore, in mucosal diseases, such as vibratory angioedema, physical/psychological stressors have a relevant role. In such cases, because of the complex interplay between nervous and immune system, the pharmacological activity of benzodiazepines and typical antidepressants may downregulate neuroinflammation.

Failure of omalizumab and successful control with ketotifen in a patient with vibratory angio-oedema.

Vibratory angio-oedema is a rare form of physical urticaria characterized by pruriginous weals and angio-oedema at the site of exposure to vibration. Severe treatment-resistant disease can occur, and is associated with significant disability. Therapy with omalizumab, a monoclonal IgG anti-IgE antibody, has been shown to be successful in several types of physical urticaria. We report a patient with vibratory angio-oedema for whom all standard treatments for urticaria, including omalizumab, failed to show a clinical benefit. Finally, ketotifen was tried, and unexpectedly reduced symptoms significantly. Ketotifen may thus represent a therapeutic option in patients with treatment-resistant vibratory angio-oedema.

Pre-eclampsia, placental abruption and increased risk of atopic sensitization in male adolescent offspring.

PROBLEM: The aim of this study was to investigate maternal pre-eclampsia (PE), placental abruption (PA) and atopy among offspring. METHOD OF STUDY: Obstetric data was recorded from 378 women who were included into the Caesarean delivery birth cohort during 1990-1992. When their children were 15-17 years old skin prick tests (SPTs) and inhalant allergen-specific (IAS) immunoglobulin E (IgE) were determined among offspring. RESULTS: Adolescents whose mothers had either PE or PA at the time of delivery had more atopy and especially more severe atopy as measured by the incidence of SPTs positive for > or = 5 allergens (RR: 4.28, 95% CI: 1.54-11.92; P < 0.005) and elevated IAS IgE levels (> or = 1.0 kU/L) (RR: 4.27, 95% CI: 1.08-16.99; P < 0.039). This increased risk was particularly observed in male adolescents. CONCLUSION: Maternal PE and PA were associated with an increased risk of severe atopy, especially in male adolescent offspring.

[Netherton's syndrome in two sisters]. / Het syndroom van Netherton bij twee zusjes.

Two girls, sisters aged 4.5 years and 6 months, had experienced serious erythrodermia since birth, with scarcely any hair growth and they exhibited poor growth despite a hypoallergenic diet. On the basis of the dermatological condition ichthyosis linearis circumflexa and microscopic examination of a hair shaft in which trichorrhexis invaginata (bamboo hair) was observed, the diagnosis of Netherton's syndrome was established. In this autosomal recessive hereditary condition there is a defective production or maintenance of the stratum corneum. Apart from the skin and the hair abnormalities there is often an atopic constitution as well. The treatment consists of skin ointments and a high-energy diet due to the loss of protein via the skin.

Impact of in utero Th2 immunity on T cell deviation and subsequent immediate-type hypersensitivity in the neonate.

It was the aim of this study to analyze the impact of maternal Th2 immune responses on onset and subsequent development of allergen-specific immunity and immediate-type hypersensitivity in early childhood. In a well characterized mouse model of Th2 immunity, BALB / c mice were sensitized to ovalbumin (OVA) before mating followed by allergen aerosol exposure during pregnancy. At the end of pregnancy mice developed allergen-specific Th2 / Th0 immunity and immediate-type hypersensitivity responses to OVA. T cells from these newborns, when restimulated with PMA / ionomycin, demonstrated a lowered capacity to produce IFN-gamma. To assess whether prenatal allergen exposure favors postnatal onset of a Th2-type immune response, these offspring were immunized to a novel antigen by a single injection of beta-lactoglobulin (BLG). In contrast to offspring from non-sensitized mothers, offspring from OVA-sensitized mice showed both higher anti-BLG immunoglobulin titers and higher frequencies of immediate-type skin test responses. Our data suggest that Th2 / Th0 immunity present during pregnancy has a decisive impact on shaping of the Th1 / Th2 T cell profile in the neonate. Furthermore, this effect favors the development of Th2 immune responses, when mice are exposed to a novel antigen during early childhood.

[Influence of diet during pregnancy and the coexistence of allergy during the 1st year of life]. / Influencia de la dieta durante el embarazo y la coexistencia de alergia en el primer año de vida.

Thirty atopic pregnant women by clinical history, skin testing, and laboratory data, and 10 non-atopic women, were studied for the influence of a diet on the outcome of their babies. The atopic women were divided into two groups: non-diet and diet group, the last group, decided to follow a non-allergenic diet that they started after 11 weeks of pregnancy. We had a follow up of the babies from all pregnancies after birth for a year. As a part of the babies clinical history, we determined the IgE concentration within 2 months after birth. Our data indicated that two babies from diet group and non from the control group, had an atopic disease during the first year of life, in contrast, five babies from the non-diet group, these preliminary results, suggested that a non-allergenic diet in atopic pregnant women, may decrease the morbidity of atopic disease during the first year of life.

[Severe hypernatremic dehydration disclosing Netherton syndrome in the neonatal period]. / Déshydratation hypernatrémique sévère révélant un syndrome de Netherton en période néonatale.

BACKGROUND: Netherton's syndrome is characterized by ichthyosis, trichorrhexis invaginata and other air shaft anomalies, and atopic diathesis. The ichthyosis is present at birth and can be complicated by hypernatremic dehydration. CASE REPORT: Adrien was the first child of non-consanguineous parents. His birth weight was 3,110 g. Ichthyosis was present at birth and the diagnosis of Netherton's syndrome was subsequently confirmed by skin biopsy. He was admitted to intensive care at the age of 4 days for a 20% loss of weight and dehydration. His blood chemistry was: Na+ = 192 mmol/l; K+ = 6.9 mmol/l; Cl- = 136 mmol/l; urea = 21 mmol/l; glucose = 12.1 mmol/l; creatinine = 209 mumol/l; hematocrit = 51%. Urinary analysis gave: Na+ = 113 mmol/l; K+ = 97 mmol/l; urea = 105 mmol/l. Progressive rehydration resulted in normal diuresis 24 hours later and a slow normalization of natremia to 138 mmol/l on day 10. Despite preventive i.v. phenobarbital, he developed convulsions on day 5 when his natremia was 177 mmol/l i.e. after a drop of 0.6 mmol/hour. The convulsions disappeared when he was given phenytoin and placed on respiratory support, but status epilepticus appeared on day 18 with normal natremia. This status epilepticus was not influenced by several antiepileptic drugs and was only cured when the patient was given tetracosactide followed by hydrocortisone plus sodium valproate. Adrien is now 3 years old and is neurologically and mentally normal. CONCLUSION: Hypernatremic dehydration can be a complication of neonatal ichthyosis; it may be severe with very high blood sodium concentration. Corticosteroids can be useful for treating persistent convulsions.