Urticaria and Angioedema: Global Overview | World Allergy Organization
Advertising revenue supports our not-for-profit mission. This content does not have an English version. This content does not have an Arabic version. Hives on light skin Hives may be accompanied by angioedema, which causes red, swollen welts. Hives on dark skin Hives, also known as urticaria, are reddened, itchy welts that may be triggered by exposure to certain foods, medications or other substances. Angioedema Angioedema may cause large welts below the surface of the skin, particularly on the eyes and lips.
Request an Appointment at Mayo Clinic. Share on: Facebook Twitter. References Habif TP. Urticaria, angioedema, and pruritus. In: Clinical Dermatology.
Urticaria and Angioedema: A Practical Approach
Philadelphia, Pa. Accessed Sept. Hives urticaria. American College of Allergy, Asthma, and Immunology. Ferri FF. In: Ferri's Clinical Advisor Bingham CO. New onset urticaria. These hormones also may influence the occurrence of urticaria and angioedema during that period. In the beginning of pregnancy, progesterone is secreted by the corpus luteum, and by the 9th week, the cells of the syncytiotrophoblast in the placenta become the main source of this hormone.
Urticária e angioedema
Progesterone levels gradually peak during the second and third trimesters and reach maximum levels just before delivery. The principle action of this hormone is to support the endometrial tissue during pregnancy and to maintain quiescence of the myometrium, preventing its contractions. It also plays a role in immunomodulatory processes in the fetal—maternal interface. Estriol, the main estrogen secreted in pregnancy, also is derived from the placenta, and its levels rise gradually to peak toward the end of gestation. Estriol stimulates the growth of the myometrium and antagonizes the myometrial-suppressing activity of progesterone.
In many species, the high levels of estrogen in late gestation induce myometrial oxytocin receptors, preparing the uterus for parturition [ 5 , 6 ]. Whereas estrogens usually enhance humoral immunity, progesterones and androgens are considered suppressors [ 13 ]; both take part in the fluctuation of immune cell function during normal states, such as the menstrual cycle, and in disease states, such as autoimmunity or allergy.
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For example, some of the classic atopic diseases, such as asthma and atopic dermatitis, are more common in males before puberty and become more common in females after puberty [ 14 ]. Both estrogen and progesterone were described as contributors to the development of autoimmune diseases [ 15 ], such as rheumatoid arthritis [ 16 ] and systemic lupus erythematosus, which primarily occur in females [ 17 ].
Sex hormones play a major role in inducing this environment [ 18 ] but also might play a role in diseases during pregnancy. Progesterone and testosterone inhibit histamine release from mast cells and basophils [ 19 ]. Synthetic estrogens, from either unknown origins or environmental pollutants, tend to accumulate in bodily tissues and theoretically can induce rapid histamine release from mast cells. Some studies present this theory as the basis for chronic idiopathic and chronic autoimmune urticaria [ 20 ].
There are two specific urticarial dermatoses that occur only in pregnancy: pruritic urticarial plaques of pregnancy PUPPP and the first stage of pemphigoid gestationis.
Other urticarial rashes that are not pregnancy specific may worsen or flare during pregnancy. Progesterone urticaria is an autoimmune disease that is part of the spectrum of autoimmune dermatitis, which includes angioedema, erythema multiforme, papulovesicular eruptions, and fixed drug eruptions induced by progesterone. It is characterized by cyclic exacerbations during the luteal phase of the menstrual cycle and is related to fluctuations in serum progesterone levels, which rise a few days after ovulation and fall with the onset of menstruation.
Lesions usually occur on the trunk and extremities but rarely may appear on the face or genitalia. Diagnosis often is challenging because it is similar in appearance to other chronic urticarias; however, the cyclic nature and positive skin tests for progesterone distinguish this rash [ 21 ]. It is believed that some women treated with progesterone before and during pregnancy for placental insufficiency likely are sensitized to both the synthetic and the biologic compounds.
These women might develop progesterone dermatitis or have worsening of their already-existing rash during pregnancy and the peripartum period [ 22 ].
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Reports on progesterone dermatitis in pregnancy are scarce, most likely because of the remission of disease in this period, which probably is a result of acquired desensitization to the rising levels of progesterone in contrast to the cyclic rise and fall during the menstrual cycle [ 23 ]. Nevertheless, a few articles describe the flare of disease during pregnancy.
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In one case report, progesterone dermatitis was induced by a metabolite of progesterone that was used as supplementation during pregnancy to prevent preterm birth [ 24 ]. In another case report, progesterone dermatitis became aggravated during the third trimester of pregnancy, necessitating high doses of steroids [ 25 ]. Treatment with antihistamines and steroids for progesterone dermatitis usually is not sufficient. Oral contraceptives that prevent ovulation and thereby also prevent progesterone increases are useful, but this treatment is not suitable during pregnancy.
Desensitization to synthetic progesterone was reported as successful, especially during in vitro fertilization [ 26 ]. In severe cases, oophorectomy is an option [ 25 ]. Estrogen dermatitis is an uncommon disease occurring as cyclic exacerbations of skin eruptions during ovulation and premenstrually. Patients test positive on intradermal skin testing for estrogen, and they respond to estrogen receptor antagonists such as tamoxifen and progesterone therapy. It is postulated that Langerhans cells play a role in the pathogenesis of disease [ 28 ].
There are no reports in the literature about estrogen dermatitis during pregnancy. The high frequency of chronic urticaria in women suggests a possible correlation between sex hormones and the autoimmunity. In one study conducted by Asero [ 31 ], about three fourths of female patients with chronic urticaria had positive autologous serum skin tests, which were found to correlate with the autoantibodies mentioned earlier. The frequency in men was only one third.
Nevertheless, no direct influence of estrogen or progesterone has ever been demonstrated as a major factor in the pathogenesis of chronic urticaria. A few studies, however, found that patients with chronic urticaria tend to have lower levels of dehydroepiandrosterone sulfate, an androgenic hormone with immunomodulatory functions.
It is believed that lower levels of this hormone might contribute to disease flare [ 32 ]. Surprisingly, there is no definite pattern for chronic urticaria during pregnancy. Flare-ups may occur in some patients, whereas improvement may occur in others. Albeit the physical changes occurring in the female body during pregnancy, there are no case reports concerning the worsening of physical urticaria during that period. Hereditary angioedema HAE is a rare disease ,—50, [ 33 ] in which episodes of edema of the skin and mucous membranes develop.
However, the most common site of involvement is the abdomen [ 34 ]. In patients with HAE, the fibrinolysis and complement pathways may compensate for the lack of C1EI, so its deficiency does not lead to clinical manifestations. However, overactivation of kallikrein in the contact system is responsible for bradykinin accumulation in the blood and tissues of patients and to the vasodilatatory effect that leads to edema [ 35 ].
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More than mutations already have been described on the gene for C1EI, on chromosome 11, and the disease is inherited in an autosomal dominant fashion. The main trigger for an attack is tissue damage, which probably activates the coagulation, complement, and contact systems. For example, dental repair might lead to perioral swelling, whereas an upper respiratory tract infection may result in laryngeal edema [ 37 ]. Other triggers to the disease include drugs e. Although HAE is transferred in an autosomal dominant fashion, penetrance is increased in females [ 34 ], and the course of their disease is more severe [ 38 ].
Estrogen has been shown to affect the contact and coagulation systems by increasing the levels and function of factor XII, prekallikrein, kallikrein, and high molecular weight kininogen, all of which are involved in the process of bradykinin formation.
Estrogens also up-regulate the expression and potentiate the activity of bradykinin receptor B 2 [ 39 ]. The influence of progesterone on the course of HAE is inconclusive. It is known to raise the levels of kallikrein, thereby increasing bradykinin formation.
go site The attack rate may increase, decrease, or remain the same during pregnancy. Bouillet et al. It is believed that the rapid elevation in estrogen levels at the beginning and at the end of pregnancy and the increase in prolactogenic hormones are responsible for this rise, whereas the balance between estrogen and progesterone in the second trimester decreases the rate of attacks.
The cessation of chronic medications such as danazole during pregnancy is mandatory because of its virilizing effect on the fetus, but it often leads to exacerbations. In several case reports, a higher attack rate also was observed during puerperium [ 46 ]. Delivery itself, however, did not provoke attacks, except for rare local swelling of the genitalia [ 47 ], and there were no more caesarian sections in patients with HAE than in the normal population [ 48 ].
Factors associated with a more aggressive course in pregnancy include early onset of disease in life, bearing a fetus with C1EI deficiency, and disease exacerbation during menses [ 39 ]. In , a new type of HAE was described by Bork et al. So far, two missense mutations ThrArg, ThrLys and one deletion of 72 base pairs have been described in the gene to factor XII and by a yet unknown mechanism that potentiates the contact pathway and probably increases bradykinin production, leading to angioedema.
Inheritance is autosomal dominant with incomplete penetrance [ 52 , 53 ]. In another case series, 17 of 27 women reported the occurrence of the first attack shortly after starting oral contraceptives containing estrogens. Estrogens also decrease the levels of ACE inhibitor, a key enzyme in the degradation of bradykinin [ 39 ].
In another case, pregnant women bearing the mutation developed severe complications, including fetal death [ 58 ]. In other cases, pregnancy did not lead to exacerbations. Because it is a rare disease, not enough information is known about its course during pregnancy. Unlike in chronic urticaria and angioedema, histamine does not play a role in the pathogenesis of the disease.