Chronic Urticaria

Last Updated: 2021-10-15

Author(s): Anzengruber F., Navarini A.

ICD11: EB00.1

Recurrent urticaria episodes that last longer than 6 weeks.

  • Prevalence: about 1% of the population.
  • Women: Men = 2: 1.

Chronic urticaria: Differentiation according to aetiology.

Chronic spontaneous urticaria (CSU):

  • Idiopathic
  • Autoimmune
  • Allergy
  • Pseudoallergy - Food additives, natural salicylates, amines, spices, green teas and alcohol
  • Infections and infestations

Chronically inducible urticaria:

  • Physical urticaria (pressure, vibration)
  • Cold urticaria
  • Heat urticaria
  • Light urticaria
  • Cholinergic urticaria
  • Aquagenic urticaria
  • Exercise-induced urticaria
  • Contact urticaria 
    • Allergic
    • Non-allergic

S. Urticaria.

Step 1

Medical history

Personal and family medical history for atopy, medication history (in particular: ASS, NSAIDS, β blockers, ACE inhibitors, histamine liberators).

Frequency

Duration

Time of day?

Localization?

Infections?

Medical specialist: Atopy? Urticaria?

Nicotine or alcohol abuse?

Existence of angioedema?

Incompatibilities?

Physical stimuli?

Occupation?

Hobbies?

New implants?

Insect bites?

Association with menstrual cycle?

Previous therapies?

Status

Wheals? Petechiae, hyperpigmentation?

Dermographism? Darier?

In the case of isolated urticaria factitia: no further clarification

Lab

Blood count-  differential leucocyte count, CRP, TSH, fT4

Thyroid autoantibodies (anti-TPO, anti-TG, TRAK), ANA

Tryptase, IgE total, possibly Fx5

Skin test

Atopy screening

Step 2: according to medical history

Angioedema without urticaria

C1 inhibitor concentration, function, C4

Vd. a. Food allergy (rare)

Allergological clarification:

Skin Test, CAP, Oral Provocation Test (OPT)

Increase in intake of histamine-rich food Low-histamine diet with nutritional advice, complaint diary

Anamnestically unclear connection with food intake

Rice-potato diet (ev stationary)

With improvement attempt low-additive diet

Especially physical urticaria:

Cold urticaria
Heat urticaria
Pressure urticaria

Cholinergic Urticaria
Light Urticaria

Aquagenic urticaria

 

Cold test

Heat test

Pressure test

Bicycle ergometers

UV threshold

Water test

Suspected autoreactive urticaria

Autologous serum test

Suspected Hepatitis

Hepatitis serology, ev Cryoglobulinemia

Reflux, stomach pain

H. pylori breath test

Systemic symptoms (fever, arthralgia, etc)

Suspected Schnitzler syndrome (rare)

Cryopyrin-associated periodic syndromes (rare)

Biopsy (neutrophil urticaria?)

Immune electrophoresis, immunofixation

 Ev. NLRP3 gene mutation search, measure IL-6 in thrust

Hives >24h/ Hyperpigmentation/Petechiae, therapy resistance

Biopsy, possibly DIF, C3, C4

Darier sign positive

Biopsy (mast cell staining)

ANAs increased

≤1:80 no further clarification

1:160 Follow-up

≥ 1:320 ANAs specificities (depending on clinic and binding pattern)

Depending on sexual history

HIV test

CRP↑ or leucocytosis

Focus search based on medical history

Eosinophilia or IgE↑

or stay in tropical regions

Stool analysis + serology

Suspected Early stage bullous

Pemphigoid

Biopsy with DIF, IIF in serum

 

  • The assessment of degree of suffering by PRISM and quality of life questionnaire (CU-QoL).
  • Determination of urticaria activity using UAS-7 diagnostics.
  • According to the guidelines, the exclusion of malignant diseases is obsolete.

Usually non‐specific histology , with vascular and lymphatic dilatation, oedema and a variable perivascular cellular dermal infiltrate consisting of lymphocytes, neutrophils and eosinophils.

  • H1 antihistamines –usually 2nd generation – Acrivastine, cetirizine, levocetrizine, loratadine, fexofenadine
  • Oral corticosteroids (prednisolone 0.5 mg/kg/day) - may be used as rescue treatment over 1–3 days. Often required for disease control in severe delayed pressure urticaria and urticarial vasculitis.
  • Leukotriene receptor antagonists – in aspirin‐sensitive urticaria , delayed pressure urticaria and autoimmune urticaria when added to antihistamine.
  • Doxepin, a tricyclic antidepressant with H1 and H2 antihistaminic activity, has been used at night in a starting dose of 10–30 mg.
  • Danazol -  for patients with refractory cholinergic urticaria.
  • Sulphasalazine dapsone - for delayed pressure urticaria.
  • Tranexamic acid may suppress non‐histaminergic idiopathic angio‐oedema
  • Narrow‐band phototherapy
  • Plasmapheresis.
  •  Intravenous immunoglobulin (IVIG) infusions, at 0.4 g/kg/day for 5 days (total 2 g)
  • Ciclosporin at 2.5–3.5 mg/kg/day for 1–3 months
  • Tacrolimus
  • Mycophenolate mofetil
  • Methotrexate
  • Cyclophosphamide

  1. Zuberbier T, Henz BM. Acute and Chronic Urticaria.  Urticaria: Springer Science + Business Media; 1998:39-44.
  2. Heymann WR. Chronic urticaria and angioedema associated with thyroid autoimmunity: Review and therapeutic implications. Journal of the American Academy of Dermatology 1999;40:229-32.
  3. Sabroe RA, Poon E, Orchard GE, et al. Cutaneous inflammatory cell infiltrate in chronic idiopathic urticaria: Comparison of patients with and without anti-FcϵRI or anti-IgE autoantibodies. Journal of Allergy and Clinical Immunology 1999;103:484-93.
  4. Ring J. Desloratadine in the treatment of chronic idiopathic urticaria. Allergy 2001;56:28-32.
  5. Grattan CEH, Sabroe RA, Greaves MW. Chronic urticaria. Journal of the American Academy of Dermatology 2002;46:645-60.
  6. Kaplan AP. Chronic Urticaria and Angioedema. New England Journal of Medicine 2002;346:175-9.
  7. Yosipovitch G, Ansari N, Goon A, Chan YH, Goh CL. Clinical characteristics of pruritus in chronic idiopathic urticaria. Br J Dermatol 2002;147:32-6.
  8. Doeglas HMG. Reactions to aspirin and food additives in patients with chronic urticaria, including the physical urticarias*. British Journal of Dermatology 2006;93:135-44.
  9. Asero R, Tedeschi A, Coppola R, et al. Activation of the tissue factor pathway of blood coagulation in patients with chronic urticaria. Journal of Allergy and Clinical Immunology 2007;119:705-10.
  10. Powell RJ, Du Toit GL, Siddique N, et al. BSACI guidelines for the management of chronic urticaria and angio-oedema. Clinical & Experimental Allergy 2007;37:631-50.
  11. Engin B, Özdemir M. Prospective randomized non-blinded clinical trial on the use of dapsone plus antihistamine vs. antihistamine in patients with chronic idiopathic urticaria. Journal of the European Academy of Dermatology and Venereology 2008;22:481-6.
  12. Kaplan AP, Joseph K, Maykut RJ, Geba GP, Zeldin RK. Treatment of chronic autoimmune urticaria with omalizumab. Journal of Allergy and Clinical Immunology 2008;122:569-73.
  13. Bunselmeyer B, Laubach HJ, Schiller M, Stanke M, Luger TA, Brehler R. Incremental build-up food challenge - a new diagnostic approach to evaluate pseudoallergic reactions in chronic urticaria: a pilot study. Clinical & Experimental Allergy 2009;39:116-26.
  14. Godse K. Omalizumab in treatment-resistant chronic spontaneous urticaria. Indian Journal of Dermatology 2011;56:444.
  15. Maurer M, Altrichter S, Bieber T, et al. Efficacy and safety of omalizumab in patients with chronic urticaria who exhibit IgE against thyroperoxidase. Journal of Allergy and Clinical Immunology 2011;128:202-9.e5.
  16. Zuberbier, T. (2016). Klassifikation und Diagnostik der Urtikaria- deutschsprachige Version der internationalen S3-Leitlinie. Awmf.org. Retrieved 30 May 2016, from http://www.awmf.org/leitlinien/detail/ll/013-028.html
  17. Saini, S. (2016). Chronic urticaria: Clinical manifestations, diagnosis, pathogenesis, and natural history. Uptodate.com. Retrieved 30 May 2016, from http://www.uptodate.com/contents/chronic-urticaria-clinical-manifestations-diagnosis-pathogenesis-and-natural-history?source=search_result&search=chronic+urticaria&selectedTitle=1~55