Urticarial vascuilitis is a rare form of vascuilitis, but very painful and disfigurement form of urticaria.

1. Marwa Abo Elmaaty Besar
Lecturer Of Internal Medicine
(Rheumatology Immunology Unit)
(Pediatric Rheumatology)
Mansoura University
Urticarial vasculitis is
still a mystery

2. https://www.researchgate.net/publication/342214895_Pathophysiology_and_therapy_of_systemic_vasculitides

3. Urticarial vasculitis
• is a rare clinicopathologic entity
• that manifests as a result of inflammatory injury to the small vessels of the skin.
• This condition is characterized by:-
• Chronic or recurrent episodes of urticarial lesions
• features of leukocytoclastic vasculitis on histopathologic specimens.
• Common site;
• Skin or extend systemically, affecting various organs, including
• The musculoskeletal,
• Renal, pulmonary,
• Gastrointestinal, and ocular systems

4. Epidemiology:
• Incidence:
• Rare; the exact prevalence of urticarial vasculitis is unclear.
• One study conducted in Sweden estimated an annual incidence of 0.7% with a
point prevalence of 9.5 per million as of December 2015
• In patients presenting with chronic urticarial lesions, prevalence range from 2%
to 20%.
• Gender:
• Female > male.
• Normocomplementemic subtype is seen with a slight female predominance
(male-female ratio of 1:2)
• Hypocomplementemic subtype is almost only observed in female
• Age:-
• Peak during the fourth and sixth decade of life.
• Very rare affect children and reported cases affect infants
Sjöwall et al., 2018
Jachiet et al., 2015

5. Cause:
• Although the causality is not clear,
• its association to a wide variety of systemic diseases has been reported,
• hepatitis B and C
• infectious mononucleosis
• Lyme disease , COVID-19
• haematological disorders
• Malignancies; multiple myeloa, NHL
• Autoimmune diseases
• Sjogren’s syndrome, rheumatoid arthritis,
• Systemic lupus erythematosus
• Inflammatory bowel disease,
• Some drugs have been implicated:
• Cimetidine, diltiazem, potassium iodide,
• Fluoxetine, nonsteroidal anti-inflammatory drugs,
• Methotrexate, telmisartan, enalapril, levetiracetam,
• And over-the-counter diet pills
Kolkhir et al., 2019; Nasiri et al., 2020;
Ozçakar et

6. • Environmental exposure;
• Cold contact to reproduce LCV.
• Cryo-pyrinopathies, associated with uv.
• Cryoglobulins should be evaluated in such cases.
• Genetic component;
• One gene mapping study revealed an association between such cases and a
homozygous frameshift mutation in DNASE1L3, which encodes for a protein in
the deoxyribonuclease I family
• Familial cases of hypocomplementemia urticarial vasculitis syndrome
Pérez-Bustillo and Sánchez-Sambucety, Ducarme et al., 2003; Jachiet et al., 2018, Kassim et al., 20

7. Kolkhir et al. World Allergy Organization Journal (2020) 13:1001
http://doi.org/10.1016/j.waojou.2020.100107

8. Pathogenesis:
• UV is thought to be immune-complex mediated
• UV is classified as a type III hypersensitivity reaction.
• Activation of classical pathway, C3a and C5a.
• Mast-cell degranulation and the release of chemokines and cytokines.
• A role of interleukin 1 (IL-1).
Venzor et al., 2002
Kamyab et al., 2019

10. Classification:
• The two types include:
• Normo-complementemic Urticarial Vasculitis (NUV):
• It is the less severe form of UV
• Is characterized by normal complement protein levels in blood
• Hypo-complementemic Urticarial Vasculitis (HUV):
• It is the more severe form of UV
• Is characterized by low complement protein levels in blood
(below normal range)

11. Clinical presentation:

12. Kolkhir et al. World Allergy Organization Journal (2020) 13:1001
http://doi.org/10.1016/j.waojou.2020.100107

14. Clinical presentation:
• Systemic involvement;
• A myriad of systemic findings.
• More frequent in hypo-complementemic patients or among those expressing anti-c1q antibodies.
• Musculoskeletal symptoms, arthralgias and myalgias
• Renal involvement;
• Nearly 20%
• Glomerular impairment, primarily resulting from membranoproliferative glomerulonephritis (MPGN)
• Others; extracapillary, extramembranous, mesangial, crescentic, focal-proliferative, and segmental
hyalinosis glomerulonephritis.
• Some patients have diffuse interstitial involvement
• The most common renal symptoms are hematuria and proteinuria, with a small minority eventually
developing kidney failure requiring dialysis.
• Fortunately, even patients with extensive kidney involvement maintain a fairly good prognosis
• Ocular manifestation:
• 10% patients, 30% HUV.
• Manifesting as uveitis, most often posterior, episcleritis, and conjunctivitis
Boyer et al., 2020
Kolkhir et al., 2020

15. • The lungs:
• About 20%-30% of them develop COPD patients, reached to 50% HUV.
• The exact pathogenesis for development COPD is stillunknown.
• Risk factor for developing COPD:
• Younger patints.
• Smoking tobacco.
• Hypocomplementemia UV.
• Pleuritis;
• Recalcitrant to treatments,
• Many patients requiring lung transplantation,
• Tend to worsen with disease progression.
• The leading cause of mortality for urticarial vasculitis
• Laryngeal oedema may occur.
• GIT symptoms:
• 30% patients.
• Commonly manifest as abdominal pain, nausea, vomiting, and diarrhea
• Cases of intestinal ischemia secondary to urticarial vasculitis have also been reported.
• Other rare extracutaneous pathologies include pericarditis, pericardial effusion, pseudotumor cerebri,
cranial nerve palsies, and transverse myelitis
Buck et al., 2012
Koç et al., 2017

16. Kolkhir et al. World Allergy Organization Journal (2020) 13:1001
http://doi.org/10.1016/j.waojou.2020.100107

17. 2020 Acta Dermato-Venereologica.

18. Differential Diagnosis:

20. Laboratory findings
• Blood and urine tests related to overall inflammatory and autoimmune activity:
• Complete blood count (CBC) with differential: This measures the red blood cell count and hemoglobin levels in
blood
• Erythrocyte sedimentation rate (ESR): ESR is a blood test that can help the healthcare provider identify any
inflammatory activity within the body
• C-reactive protein (CRP) test: This blood test measures the amount of a protein, called C-reactive protein, in
blood. It also provides information on the inflammation condition of the body
• Anti-nuclear antibody (ANA) test: ANA test identifies the antinuclear antibodies within the blood
• Basic metabolic panel tests
• Anti-streptolysin O (ASO) titer test, if a streptococcal infection is the suspected trigger
• Specific antibodies test
• Protein electrophoresis and immunofixation electrophoresis tests
• Complement blood test
• Cryoglobulin blood test
• HIV antibody blood test
• Urine tests for protein and presence of blood
• Urinalysis
Koç et al.,2017; Zuberbier and Maurer,

21. • Tests and procedures related to the skin and underlying tissue
(integumentary system):
• Dermoscopy: It is a diagnostic tool where a dermatologist examines the skin using a special
magnified lens
• Wood’s lamp examination: In this procedure, the healthcare provider examines the skin using
ultraviolet light. It is performed to examine the change in skin pigmentation
• Punch biopsy of skin for immunofluorescence studies, especially direct immunofluorescence
studies. The immunofluorescence deposits can be performed with fluorescein-labeled
antibodies against IgG, IgM, IgA, and C3
• Skin biopsy: A skin biopsy is performed and sent to a laboratory for a pathological
examination. The pathologist examines the biopsy under a microscope. After putting
together clinical findings, special studies on tissues (if needed) and with microscope findings,
the pathologist arrives at a definitive diagnosis
Baigrie et al., 2020
Damman et al., 2020

22. Kolkhir et al. World Allergy Organization Journal (2020) 13:1001
http://doi.org/10.1016/j.waojou.2020.100107

23. UV prevention:-
• Currently, there are no methods available to prevent Urticarial Vasculitis.
• An early diagnosis and prompt treatment can help decrease the burden of the condition.
• Undertaking early and appropriate treatment of the underlying infection or condition (potential
risk factors) may help in lowering one’s risk for Urticarial Vasculitis and is an important
consideration
• Treating and managing the underlying condition that triggered the initial episode helps in
preventing subsequent episodes
• Maintaining a healthy lifestyle with good eating habits, adequate physical activities, and
avoidance of smoking or drinking is beneficial
• Ensuring that individuals, especially children, are up-to-date on their vaccinations is important
• Patients are required to be vigilant during the remission period and report any new symptoms
promptly to the healthcare provider
• Regular medical screening at periodic intervals with blood tests, scans, and physical examinations,
are mandatory, due to the possibility of recurrence/relapse. Often several years of active vigilance
is necessary

24. Treatment:-
• The treatment of Urticarial Vasculitis depends upon a variety of factors:-
• Type of Urticarial Vasculitis - whether normocomplementemic or
hypocomplementemic subtype
• Severity of the signs and symptoms
• Regions of the body affected and the level of functioning of the affected organs
• Overall health status of the individual and his/her age
• Underlying condition triggering Urticarial Vasculitis
• The main treatment of Urticarial Vasculitis includes
• Treating the underlying cause.
• If a clearly identifiable cause is not known, then the primary role of treatment is to reduce
inflammation.
• A multidisciplinary team of specialists that include general practitioners,
rheumatologists, neurologists, pulmonologists, cardiovascular experts, nephrologists,
dermatologists, ophthalmologists, among others, may be involved in the treatment.

25. International Journal of Women’s Dermatology 7 (2021) 290–297
Diagnostic algorithm for evaluation of urticarial lesions

27. Kolkhir et al. World Allergy Organization Journal (2020) 13:1001
http://doi.org/10.1016/j.waojou.2020.100107

30. Home message:
• Most reported symptoms; wheals, burning of the
skin, and residual hyperpigmentation occur in 60–
100% of UV patients
• Wheals of longer than 24 hours duration, the
results of histological analysis, and post
inflammatory hyperpigmentation are the most
important features for establishing the diagnosis
of UV.
• The most common tests ordered in UV patients
are complete blood count, erythrocyte
sedimentation rate, C-reactive protein,
complement components, antinuclear antibodies
and skin biopsy

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