There are different types of physical urticaria. It has yet to nbe determined the reason why a rash appears. The physical stimulus somehow causes a release of histamine and other chemicals which causes the rash.
Dermographism
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Dermographism means skin writing. People with this condition develop the rash on areas of skin that have been firmly stroked or using an object or finger creating pressure a rash appears as a line or writing on the skin. The affected area of skin is usually very itchy, but in some mild cases it does not itch much. Although any part of the skin can be affected, the palms, soles of the feet, genital skin and scalp are less commonly affected. Dermographism is more prone to occur when you are hot. For example, it may develop more easily when you rub yourself firmly with a towel after a hot shower. The rash tends to last less than an hour. In many cases the pressure needed to be applied to cause the rash is quite firm. However, some people develop dermographism with just light pressure.It most commonly first develops in early adult life. In most cases, the condition tends to improve gradually over a few years and it goes or becomes less severe. However, in some cases the condition remains troublesome for many years.
Cholinergic urticaria
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Cholinergic urticaria is quite common. It is caused by sweating and is sometimes called heat bumps. The urticarial rash is quite distinct as the weals are very small (2-3 mm) with a red flare around each one. The rash appears within a few minutes of sweating and tends to be worst on the chest, back and arms. The rash lasts from 30 minutes to an hour or more before fading away. Some people become slightly wheezy and short of breath for the duration of the rash.The sweating that triggers the rash may be due to exercise, heat, fever, emotion or eating spicy food. It can be a real nuisance when trying to exercise. In severe cases, hundreds of tiny weals develop when you run or do other types of exercise. Sometimes the tiny weals join together to form larger ones. Cholinergic urticaria most commonly first develops in early adult life. In many cases the condition tends to improve after a few years and it goes or becomes less severe. However, in some cases the condition remains troublesome for many years.
Cold urticaria
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Cold urticaria is a relatively uncommon. An urticarial rash develops after being exposed to cold, including rain, cold winds and cold water. It may be the cold that triggers the rash, or the re-warming of the skin after coming in from the cold. The rash affects the chilled parts of the skin. If a large area of skin has been chilled, the rash can be very extensive. For example, swimming in cold water may cause a widespread and severe rash over most of the body that can make you dizzy and faint. (For this reason, if you are known to develop cold urticaria, you should never go swimming alone.)
Delayed pressure urticaria
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Delayed pressure urticaria is uncommon. It can develop alone but it commonly affects people who also have chronic urticaria. In this type of urticaria the rash develops after the affected area of skin has had deep prolonged pressure applied. The rash can be painful and tends to last several hours, or even a day or so.
Solar urticaria
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This is rare. In this condition an urticarial rash develops on skin exposed to sunlight.
Aquagenic (water contact) urticaria
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In this rare condition an urticarial rash develops on skin exposed to water of any temperature.
There are tests out there to determine if its autoimmune and one important test is called the
ReplyDeleteautologous serum skin test (ASST). This test is still not widely available, so it may take some hunting around to find a local clinic or hospital that offers the test. The ASST requires a blood sample and then spinning it down in a centrifuge to separate the serum and then injecting the serum back into your arm. A wheal/flare response to the injection is considered positive for autoimmune CU.
Some things many people with autoimmune disease have in common:
If you are a female:
You may see a worsening of symptoms during periods
Remissions during pregnancy
Development of other autoimmune diseases
Family history of various autoimmune problems (one family member may have rheumatoid arthritis, another may have thyroid disease, still another may have endometriosis)
Onset of symptoms with an event or trauma such as accident, illness, surgery, or infection within approximately 6 months—something that kicks the immune system into high gear.
If you've experienced any of these autoimmune CU you need to seek out a physcian.
Article by Medscape titled Autoimmune Urticaria
ReplyDeleteAutoimmune Urticaria
The wheals, redness and itching of CIU are due to release of histamine and other mediators, including eicosanoids, cytokines and proteases, from dermal mast cells. In CIU, the triggering stimulus causing the characteristically unpredictable dermal mast-cell activation has proved elusive. That an antibody-mediated reaction might be responsible was suggested early on by authors such as Rorsman[10] and Gruber et al.[11] There was also some indirect evidence to suggest that CIU might have an autoimmune basis, at least in some patients, since the incidence of autoimmune thyroid disease is significantly raised in these patients,[12-15] and there is a high frequency of human leukocyte antigen alleles that are characteristically associated with autoimmune disease.[16]
Evidence That Chronic Idiopathic Urticaria in Some Patients is Cause By Autoimmunity
Prima facie evidence of the involvement of functional autoantibodies against the high-affinity receptor (FcεR1) of dermal mast cells and basophils was first reported in 1993 and 1996 by Hide et al..[3,4] These investigators used basophils and dermal mast cells of healthy human donors to show that the serum and purified IgG of a subset of about 25% of patients with CIU would release histamine from these cells, and that release could be inhibited by preincubation with recombinant FcεR1α, the α-chain of the high-affinity IgE receptor. In a further 5% of patients, the histamine-releasing factor was inhibited not by the α-chain of FcεR1 but by IgE itself. Thus, patients with CIU appear to have functional autoantibodies of the IgG subclass with specificity for FcεR1 or IgE. That these autoantibodies are the cause of the lesions in the CIU patients who have them is supported by their ability to cause whealing upon intradermal injection in skin of human volunteers[17] and to release histamine from healthy donor basophils and mast cells.[3,4] The plasma levels of these autoantibodies vary in proportion with disease activity,[18] and removal (by plasmapheresis) causes remission.[19] These findings have been confirmed by a number of independent groups, the percentage of patients with CIU and anti-FcεR1 autoantibodies varying from 35 to 55% of the total.[5,6,20]
Does Identification of These Patients Matter?
Patients with AICU are more treatment-resistant, and their disease runs a more aggressive course, than those with non-autoimmune CIU.[2] Thus, if a patient with AICU proves unresponsive to H1 antihistamines, even in off-label dosage, and is suffering serious disability as a result of the disease, then immunosuppressive therapy (cyclosporin,[21] intravenous Ig,[22] or even plasmapheresis[18]) may be warranted. Even in those autoantibody-positive patients for whom immunotherapy is deemed inappropriate, an understanding of the autoantibody basis of his/her urticaria may help the patient to cope with the problem and avoid unnecessary investigations.
Diagnosis of Autoimmune Urticaria: the Autologous Serum Skin Test
Unfortunately, there is no straightforward rule-in/rule-out test for autoimmune urticaria. Clinical and histological evaluations are unhelpful. Although there are subtle differences,[2,23] these do not provide adequate discrimination between CIU and AICU. We have found the autologous serum skin test to be a useful screening test for AICU. In this test, 0.05 ml of the patient's serum, removed during a period of disease activity, is injected intradermally volume 0.05 ml into the same patient's uninvolved forearm skin, along with equal volumes of saline and histamine (10 µg/ml) at adjacent sites. The test is read 30 min later. A positive result is recorded if the diameter of the wheal at the serum-injected site is 1.5 mm greater than that of the bleb at the saline-injected site. The sensitivity and specificity of the test were calculated as 65-81% and 71-78%, respectively.[24] Using in-vitro release of histamine from healthy donor basophils (see below) as the 'gold standard', the autologous serum skin test was negative in 39/40 healthy controls, in 23/24 patients with a physical urticaria (symptomatic dermographism, cholinergic urticaria), and in 10/10 patients with atopic eczema. We advise that a positive result should be confirmed by in-vitro testing.
Diagnosis of Autoimmune Urticaria: In-Vitro Tests
Numerous attempts have been made to devise specific and sensitive in-vitro tests for AICU, including enzyme-linked immunosorbent assay and other immunobinding assays,[25] but, in terms of specificity, the results have been disappointing; Kikuchi and Kaplan[26] were recently obliged to conclude, on the basis of a study of 260 patients with chronic urticaria, that immunobinding assays were unacceptable as a method for diagnosis of AICU, and that functional assays are required. Furthermore, immunoreactive anti-FcεR1 autoantibodies are also occasionally found in autoimmune connective tissue and immunobullous diseases.[25] The 'gold standard' laboratory test for AICU is the demonstration and measurement of histamine (or other mediator) release from target basophils or dermal mast cells. Basophils are normally obtained from healthy donors. Basophils from both low-IgE and high-IgE donors should be used to enable identification of patients with anti-FcεR1 or anti-IgE autoantibodies. Dermal mast cells are obtained from skin removed during circumcision.[3,4] Alternatively, a rat basophil leukaemia cell line transfected with the genes that code for the α, γ1 and γ2 chains of FcεR1 can be used.[6] These cells express functioning high-affinity IgE receptors, and can be used to detect anti-FcεR1 autoantibodies by using release of β-glucuronidase histamine or serotonin as quantitative markers of activation.
Current Critical Issues in Autoimmune Chronic Urticaria: Mode of Action of Anti-FcεR1 Autoantibodies
Initial studies argued against the involvement of complement activation in AICU.[3,4] Recent work suggests that this may be an oversimplification, on the basis of the following observations. The antibodies involved in AICU turn out to be predominantly IgG1 and IgG3, isotypes conventionally considered to be involved in complement activation[25]; using highly purified IgG anti-FcεR1 and patients' decomplemented sera, it was found that whole sera, but not decomplemented sera, would release histamine from dermal mast cells.[26-28] It was also shown that release of histamine from dermal mast cells by anti-FcεR1 could be inhibited by an antibody to the C5a receptor and was dependent on the concentration of C5a. Thus, release of histamine from dermal mast cells by FcεR1 appears to be at least augmented by complement activation. This finding may explain the well-known but puzzling fact that patients with severe AICU do not suffer from bronchospasm, since lung, but not skin, mast cells are devoid of complement receptors.[29]
The Role of the Basophil
That basopenia is a regular feature of chronic urticaria[10] and of AICU[30] has long been recognized.[31] Lowered basophil numbers correlate well with lowered whole-blood histamine content.[31] Although the reason for the paucity of circulating basophils may be straightforward activation and depletion by circulating autoimmune factors, relocation to the urticated tissues of the skin and mucous membranes has been considered, but not supported by recent quantitative histometric and cytometric studies in CIU and AICU.[32] In any case, basopenia has been considered as a diagnostic screening test for AICU,[33**] but, for this possibility to be carried forward, convenient and reproducible methods for counting basophils in the peripheral circulation need to be found.
Treatment of Autoimmune Chronic Urticaria
Patients with AICU are initially treated in the same way as patients with CIU. However, AICU tends to be resistant to routine H1 antihistamine treatment and I often have recourse to off-label dosage of the newer non-sedative antihistamines such as fexofenadine (120 mg twice daily) and desloratidine (5 mg twice daily).[9*] The administration of sedative antihistamines, such as hydroxyzine (25 mg), at night may also help relieve nocturnal itching, but the patient must be warned of continuing impairment of cognitive function during the following day. For the above reasons this regime may prove inadequate, and in severely disabled patients immunosuppressive measures may be necessary. Although short tapering courses of oral corticosteroids may be reasonable in emergency situations, long-term corticosteroid therapy is not normally recommended for AICU. However, other authorities[34] have advocated alternate-day corticosteroid protocols for long-term management, evidently with satisfactory results. Cyclosporin is a satisfactory alternative, used frequently by the author in patients with AICU who are severely handicapped by their urticaria, and are unresponsive to antihistamines. Given in dosages of 3-5 mg/kg daily for up to 3 months, this treatment has proved effective in a placebo-controlled double-blind trial[21] in patients with CIU, all of whom gave positive results in the autologous serum skin test and had serum showing evidence of histamine-releasing activity, and were therefore highly likely to have AICU. Cyclosporin is contraindicated in patients with a history of cancer, a positive cervical smear or renal impairment. In the author's experience (unpublished observation), at least 75% of treated patients experience total, or almost total, remission. About one-third of patients treated successfully for 2-3 months remain in remission after withdrawal; one-third have a mild relapse that is easily controlled by routine antihistamines; however, one-third relapse badly upon withdrawal and may require a further course of treatment, or an alternative medication. Alternative immunotherapeutic treatments include intravenous immunoglobulin[22] or plasmapheresis.[19]
Symptoms of Mastocytosis:
ReplyDeletecause many acute and potentially serious symptoms, which include the following:
Abdominal pain
Hives & other rashes
Anaphylaxis
Inflammation of the esophagus
Blood pressure changes & shock
Intestinal cramping and bloating
Bone pain (mild to severe/debilitating)
Itching, with and without rashes
Chest pain
Liver, spleen and other organ involvement
Cognitive difficulties/brain fog
Malabsorption
Degenerative disc disease
Migraine headaches
Diarrhea
Muscle pain
Dizziness/vertigo/lightheadedness
Nausea
Faintness
Osteoporosis/ Osteopenia
Fatigue
Peripheral neuropathy and paresthesias
Flushing
Rapid heart rate
Gastroesophageal reflux
Vomiting
Hematological abnormalities
People who have been told they have Mast Cell Activation Syndrome or Disorder (MCAS/MCAD) may have a normal, or nearly normal, number of mast cells. Nevertheless, people with either Mastocytosis and MCAS/MCAD can be either very stable or extraordinarily ill on a day-to-day basis, and managing the unpredictability of the Mast Cell Diseases and their symptoms can be quite challenging. See the article on Mastocytosis Society for more information.
Testing for Systemic Mastocytosis:
ReplyDeleteA 24-h urine collection for measurement of histamine and other metabolites, blood levels of histamine or the mast cell-derived neutral protease tryptase assist in confirming things. Additional investigations may be required depending on the clinical symptoms. Obviously a tissue diagnosis is easy if the skin lesions are present, but to confirm a diagnosis of the systemic involvement of other organs a bone marrow biopsy and aspiration is often performed.
See article: http://www.netdoctor.co.uk/ate/heartandblood/202783.html
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