In the name of God

1. به نام خداوند جان و خرد In the name of God

2. ِِDr AnahitaVali Dermatologist & venereologist Urticaria

3. Urticaria is a transient element

4. Is it transient???Less than 24 hours

5. MARKING AROUND THE LESIONS

6. Urticaria is acutaneous reaction pattern • (from the Latin urtica, nettle,] commonly referred to as hives, • is a kind of skin rash notable for pale red, raised, itchy bumps. Hives might also cause a burning or stinging sensation.Hives are frequently caused by allergic reactions; however, there are many nonallergic causes.

7. Why transient & itching???

10. How Long???Most cases of hives lasting less than six weeks (acuteurticaria) are the result of an allergic trigger.Chronicurticaria (hives lasting longer than six weeks) is rarely due to an allergy.

11. Clinical findings • Distribution • Any body site • Morphology • Central, oedematous plaques without scale (a wheal) and surrounding redness (a flare)  • Annular lesions result from central clearing  • Lesions vary in size, some can be very large • Can be associated with angioedema, and much less commonly anaphylaxis. Anaphlaxisis defined by a progressive reaction with rash, breathing difficultiesand cardiovascular compromise. The most common causes of anaphylactic reactions are in the following order: • Food allergy • Drugs  • Stings / bites 

12. The majority of chronic hives cases have an unknown (idiopathic) cause. • In perhaps as many as 30–40% of patients with chronic idiopathic urticaria, it is caused by an autoimmune reaction. • Acute viral infection is another common cause of acute urticaria (viral exanthem). • Less common causes of hives include friction, pressure, temperature extremes, exercise, and sunlight. slightly raised, no scale

13. What is the differences?

14. Wheals (raised areas surrounded by a red base) from urticaria can appear anywhere on the surface of the skin. Whether the trigger is allergic or not, a complex release of inflammatory mediators, including histamine from cutaneousmast cells, results in fluid leakage from superficial blood vessels. (well-demarcated,erythmatousus) Wheals may be pinpoint in size, or several inches in diameter. itching Angioedema is a related condition (also from allergic and nonallergic causes), though fluid leakage is from much deeper blood vessels in the subcutaneous or submucosal layers.(poorly –defined borders) Usually mucosa, lips, palm &soles/lids/genitalia/tongue/larynx/GI/GU/painful

15. Hives during life: 20-25% • All age groups • Acute urticariamore common in children & young adults • Chronic urticariais more prevalent in adults/F>M • How many percent of Pts are free of chronic urticaria after 1 years??? • After 5 years??? • & after 20 years???

16. Conclusion: If urticaria associated with angioedema is more persistent. • Urticaria alone :40% • Urticaria & angioedema:49% • Angioedema alone:11% • Some of the more severe chronic cases have lasted more than 20 years. A survey indicated chronic urticaria lasted a year or more in more than 50% of sufferers and 20 years or more in 20% of them.

17. Morbidity depends on the severity and duration of the condition. One study found that urticaria patients can have as much psychologic, social, and occupational distress as patients who are awaiting triple coronary artery bypass surgery.

18. Urticaria can also be classified by the purported causative agent. Many different substances in the environment may cause urticaria, including medications, food and physical agents. • After contact with a Conifer

19. Management • Avoid triggers • Use non-sedating antihistamines PRN • Occasionally a short course of oral steroids (prednisolone) may be needed • Patients with anaphylaxis will often be diagnosed at an accident and emergency department. Such patients should be provided with two Epipens and relevant advice on how to manage an attack including how to use an Epipen • Some patients will benefit from a referral to an immunologist / paediatrician, for example: • Patients with moderate-severe reactions to eggs • Patients with nut allergies, who often have multiple allergies and may require further investigations • Other cases of life-threatening reactions

20. Drug-induced urticaria • Drugs that have caused allergic reactions evidenced as urticaria include dextroamphetamine,aspirin, ibuprofen, penicillin, clotrimazole, sulfonamides, anticonvulsants, cefaclor and antidiabetic drugs. The antidiabeticsulphonylureaglimepiride (trade name Amaryl), in particular, has been documented to induce allergic reactions manifesting as urticaria. Drug-induced urticaria has been known to have an effect on severe cardiorespiratory failure.

21. Infection or environmental agent • Urticaria can be a complication and symptom of a parasitic infection, such as fascioliasis (Fasciola hepatica) and ascariasis (Ascarislumbricoides). • The rash that develops from poison ivy, poison oak, and poison sumac contact is commonly mistaken for urticaria. This rash is caused by contact with urushiol and results in a form of contact dermatitis called urushiol-induced contact dermatitis. • Urushiol is spread by contact, but can be washed off with a strong grease- or oil-dissolving detergent and cool water and rubbing ointments.

23. Dermatographicurticaria "skin writing") • Hives caused by • stroking the skin • (often linear in appearance) • are due to a benign condition • in 4–5% of the population • usually becomes evident soon after the scratching, and disappears within 30 minutes.

24. Dermatographism is the most common form of a subset of chronic hives, acknowledged as "physical hives • It stands in contrast to the linear reddening that does not itch seen in healthy people who are scratched. In most cases, the cause is unknown, although it may be preceded by a viral infection, antibiotic therapy, or emotional upset. Dermographism is diagnosed by taking a tongue blade and drawing it over the skin of the arm or back. The hives should develop within a few minutes. Unless the skin is highly sensitive and reacts continually, treatment is not needed. Taking antihistamines can reduce the response in cases that are annoying to the patient.

25. Pressure or delayed pressure urticaria • This type of urticaria can occur right away, precisely after a pressure stimulus or as a deferred response to sustained pressure being enforced to the skin. In the deferred form, the hives only appear after about six hours from the initial application of pressure to the skin. • Under normal circumstances, these hives are not the same as those witnessed with most urticariae. Instead, the protrusion in the affected areas is typically more spread out. The hives may last from eight hours to three days. The source of the pressure on the skin can happen from tight fitted clothing, belts, clothing with tough straps, walking, leaning against an object, standing, sitting on a hard surface, etc. The areas of the body most commonly affected are the hands, feet, trunk, abdomen, buttocks, legs and face. Although this appears to be very similar to dermatographism, the cardinal difference is that the swelled skin areas do not become visible quickly and tend to last much longer. This form of the skin disease is, however, rare.

26. Treat your pts based on pathophysiology PMN-RICH URTICARIA LYMOCYTE & MAST-CELL RICH URTICARIA

27. urticarialvasculitis • Individual hives that are • painful, • last more than 24 hours • leave a bruise as they heal are more likely to be a more serious condition

28. Cholinergic or stress • Cholinergic urticaria (CU) is one of the physical urticaria which is provoked during sweating events such as exercise, bathing, staying in a heated environment, or emotional stress. The hives produced are typically smaller than classic hives and are generally shorter-lasting. • Multiple subtypes have been elucidated, each of which require distinct treatment.

29. Sudden death after diving

30. Cold-induced • is caused by exposure of the skin to extreme cold, damp and windy conditions; it occurs in two forms. • The rare form is hereditary and becomes evident as hives all over the body 9 to 18 hours after cold exposure. • The common form of cold urticaria demonstrates itself with the rapid onset of hives on the face, neck, or hands. Cold urticaria lasts for an average of five to six years. The population most affected is young adults, between 18 and 25 years old. Many people with the condition also suffer from dermographism and cholinergic urticaria. • Severe reactions can be seen with exposure to cold water; swimming in cold water is the most common cause of a severe reaction. This can cause a massive discharge of histamine, resulting in low blood pressure, fainting, shock and even loss of life. Cold urticaria is diagnosed by dabbing an ice cube against the skin of the forearm for 1 to 5 minutes. A distinct hive should develop if a patient suffers cold urticaria. This is different than the normal redness that can be seen in people without cold urticaria. • Patients with cold urticaria need to learn to protect themselves from a hasty drop in body temperature. Regular antihistamines are not generally efficacious. One particular antihistamine, cyproheptadine (Periactin), has been found to be useful. The tricyclic antidepressant doxepin .Finally, ketotifen, which keeps mast cells from discharging histamine, has also been employed with widespread success.

32. Heat-induced urticaria • This rare form of urticaria is triggered by the continued application of heat on the skin. • Hives begin to appear within two to five minutes on the area of the skin exposed to heat. • Do not last more than an hour.

34. Solar urticaria • This form of the disease occurs on areas of the skin exposed to the sun; • the condition becomes evident within minutes of exposure. After the individual is no longer exposed to the sun, though, the condition starts to weaken within a few minutes to a few hours, and hardly ever lasts longer than 24 hours. • Solar urticaria is classified into six different types, depending upon the wavelength of light involved. • Since glass absorbs light with a wavelength of 320 nm and below, people suffering from solar urticaria in response to wavelengths of less than 320 nm are protected by glass.

36. Water urticaria • rare, and occurs upon contact with water. The response is not temperature-dependent and the skin appears similar to cholinergic form of the disease. The appearance of hives is within one to 15 minutes of contact with the water, and can last from 10 minutes to two hours. The hives that last for 10 to 120 minutes do not seem to be stimulated by histamine discharge like the other physical hives. Most researchers believe this condition is actually skin sensitivity to additives in the water, such as chlorine. • diagnosed by dabbing tap water and distilled water to the skin and observing the gradual response. Aquagenicurticaria is treated with capsaicin (Zostrix) administered to the chafed skin. This is the same treatment used for shingles. Antihistamines are of questionable benefit in this instance, since histamine is not the causative factor.

37. Exercise urticaria • hives, itchiness, shortness of breath and low blood pressure five to 30 minutes after beginning exercise. These symptoms can progress to shock and even sudden death. Jogging is the most common exercise to cause EU, but it is not induced by a hot shower, fever, or with fretfulness. This differentiates EU from cholinergic urticaria • EU sometimes occurs only when someone exercises within 30 minutes of eating particular foods, such as wheat or shellfish. For these individuals, exercising alone or eating the injuring food without exercising produces no symptoms. EU can be diagnosed by having the patient exercise and then observing the symptoms. This method must be used with caution and only with the appropriate resuscitative measures at hand. EU can be differentiated from cholinergic urticariaby the hot water immersion test. In this test, the patient is immersed in water at 43°C (109.4°F). Someone with EU will not develop hives, while a person with cholinergic urticaria will develop the characteristic small hives, especially on the neck and chest • The immediate symptoms of this uncanny type are treated with antihistamines, epinephrine and airway support. Taking antihistamines prior to exercise may be effective. Ketotifenis acknowledged to stabilise mast cells and prevent histamine release, and has been effective in treating this hives disorder. Avoiding exercise or foods that cause the mentioned symptoms is very important. In particular circumstances, tolerance can be brought on by regular exercise, but this must be under medical supervision

38. Food urticaria • The most common food allergies in adults are shellfish and nuts. The most common food allergies in children are shellfish, nuts, peanuts, eggs, wheat, and soy. A less common cause is exposure to certain bacteria, such as Streptococcus species or possibly Helicobacter pylori.

40. Vibratory angioedema & urticaria • This very rare form of angioedema develops in reply to contact with vibration. In vibratory angioedema, symptoms develop within two to five minutes after contact with vibration and dissolve after about an hour. Patients with this disorder do not suffer from dermographism or pressure urticaria. Vibratory angioedema is diagnosed by holding a vibrating device such as a laboratory vortex machine against the forearm for four minutes. Speedy swelling of the whole forearm extending into the upper arm is also noted later. The principal treatment is avoidance of vibratory stimulants. Antihistamines have also been proven helpful.

41. Pathophysiology • The skin lesions of urticarial disease are caused by an inflammatory reaction in the skin, causing leakage of capillaries in the dermis, and resulting in an edema which persists until the interstitial fluid is absorbed into the surrounding cells. • Urticaria is caused by the release of histamine and other mediators of inflammation (cytokines) from cells in the skin. This process can be the result of an allergic or nonallergic reaction, differing in the eliciting mechanism of histamine release

42. Autoimmune urticaria • In the past decade, many cases of chronic idiopathic urticaria have been noted to be the result of an autoimmune trigger. For example, roughly one-third of patients with chronic urticaria spontaneously develop autoantibodies directed at the receptor FcεRI located on skin mast cells. Chronic stimulation of this receptor leads to chronic hives. Patients often have other autoimmune conditions, such as autoimmune thyroiditis.

43. Nonallergicurticaria • Mechanisms other than allergen-antibody interactions are known to cause histamine release from mast cells. Many drugs, for example morphine, can induce direct histamine release not involving any immunoglobulin molecule. • Also, a diverse group of signaling substances, called neuropeptides, have been found to be involved in emotionally induced urticaria. • Dominantly inherited cutaneous and neurocutaneousporphyrias (porphyriacutaneatarda, hereditary coproporphyria, variegate porphyria and erythropoieticprotoporphyria) have • been associated with solar urticaria. The occurrence of drug-induced solar urticaria may be associated with porphyrias. This may be caused by IgG binding, not IgE.

44. Nonallergic release of mediators • A number of drugs, such as aspirin, NSAIDs, opiates, succinylcholine, and certain antibiotics (eg, polymyxin, ciprofloxacin, rifampin, vancomycin, some beta-lactams) can cause urticaria by a nonallergic mechanism rather than by IgE-mediated hypersensitivity. • Certain foods or beverages, such as spoiled fish (scombroidosis), aged cheeses, or red wine, can contain histidine, • Certain venoms may cause urticaria. • Radiocontrast media sensitivity is not related to iodine, fish, or shellfish allergy.

45. Medical causes • Urticaria has been reported with infectious diseases. • Viral infections associated with acute urticaria include acute viral syndromes, hepatitis (A, B, and C), Epstein-Barr virus, and herpes simplex virus. • Streptococcal infection has been reported as the cause of 17% of acute urticaria cases in children. • Urticaria has also been reported with chronic parasitic infections. sinusitis, cutaneous fungal infections, Helicobacter pylori infection, or other occult infections Urticaria associated with acute group A beta-hemolytic streptococci infection.

46. Hormonal causes • Hormonal causes via endocrine tumors or ovarian pathology are rare. Oral contraceptive use or changes in the menstrual cycle have been reported as a possible cause of urticaria: patients commonly report worsening of hives with the menstrual cycle. This may be hormonally mediated, and the cyclical use of analgesics should also be considered as a possible etiology

47. Dangerous causes of urticaria • Urticaria can be the presenting symptom of lymphoma, and a careful history and review of systems is important.

49. Rare causes of urticaria • Cryoglobulinemias (eg, associated with hepatitis C, chronic lymphocytic leukemia) • Serum sickness • Other immune complex–mediated inflammation • Systemic lupus erythematosus, rheumatoid arthritis, juvenile rheumatoid arthritis, or other rheumatologic diseases (rare causes of urticaria) • Hypothyroidism and hyperthyroidism, although euthyroid patients with antithyroid antibodies (ie, vide infra) can be affected • Lymphoreticular malignancies (eg, chronic lymphocytic leukemia) • Pregnancy (ie, pruriticurticarial papules and plaques of pregnancy [PUPPP]

50. Physical causes (physical urticaria) • Cold • Pressure • Vibration • Cholinergic (triggered by heat, exercise, or emotional stress) • Sunlight • Water • Dermographism (can occur as an isolated condition) • Exercise