Urticarial Vasculitis in Children

Abstract

URTICARIAL VASCULITIS IN CHILDREN O. M. Оkhotnikova, O. A. Oshlyanskaya Shupyk National Healthcare University of Ukraine, Kyiv, Ukraine Abstract. Different autoimmune diseases can present with organs malfunctioning and chronic urticaria symptoms in particular such urticarial vasculitis. This variant of vasculitis can be a separate nosological form, which includes a rare disease as hypocomplementemic urticarial vasculitis syndrome. In addition to chronic urticaria symptoms, hypocomplementemic urticarial vasculitis syndrome is characterized by severe systemic manifestations in different organs, decreased serum level of complement components, and appearance of specific markers, such as anti-C1q-antibodies. The diagnosis is confirmed by the results of skin biopsy, which is the «gold standard» of diagnosis. The condition often requires combined treatment with two immunosuppressive drugs. Hypocomplementemic urticarial vasculitis syndrome is a very rare disease, but, because of the multisystem manifestations, it can be encountered by any specialist (pediatrician, general practitioner, allergist, rheumatologist, ophthalmologist, dermatologist, nephrologist, etc.), therefore doctors should consider this condition when dealing with such patients in order to reduce the risk of hypocomplementemic urticarial vasculitis syndrome misdiagnosis as the variants of chronic urticaria or another immunopathogenetic disease. That is why doctors should be well aware of the features of the course of this disease, not only to reduce the likelihood of erroneous diagnosis of urticarial vasculitis as chronic urticaria or another disease of immunopathological genesis, but also because modern therapy of these conditions is radically different from each other: сhronic idiopathic urticaria requires adherence to a modern patient management algorithm with the initial use of H1-antihistamines with a possible increase in the dose (up to a 4-fold increase), and with their low efficiency — the transition to the use of cytostatics, monoclonal antibodies against immunoglobulin E –omalizumab, and is extremely limited and only for short-term use of glucocorticosteroids. Key words: urticarial vasculitis, its forms, hypocomplementemia, chronic urticaria, children. Olena Okhotnikova MD, PhD, professor, Head Department of Pediatrics #1, Shupyk National Healthcare University of Ukraine, 9, Dorogozhytska str., 04112, Kyiv, Ukraine e-mail: kafedra.ped1@gmail.com Аsthma and Allergy, 2021, 3, P. 16–29.

Is chronic urticaria or urticarial vasculitis a diagnostic dilemma?

“…The uncertainty of the pathogenesis of the disease does not facilitate differential diagnosis with other conditions, especially SLE and cryoglobulinemia. Distinguishing urticarial vasculitis from urticaria is usually not difficult because the skin lesions last more than 24 hours (usually 2-3 days), they are sometimes painful or «burning» and may have residual bruising or hyperpigmentation of the skin [1,5,13]. But in our clinical case, the rash periodically disappeared and reappeared, leading to an incorrect and prolonged initial diagnosis.…”

“…Urticarial vasculitis is sometimes discussed as a clinical feature that is part of another diagnosis, and sometimes as a primary diagnosis or syndrome. Based on the levels of complement and the presence and/or absence of specific systemic signs, the terms that appear with UV as a separate diagnostic entity are HUVS, normocomplementemic urticarial vasculitis, hypocomplementemic urticarial vasculitis [12,13].…”

“…rticarial vasculitis (UV) is a rare disease that has two components: clinical manifestations of urticaria and histopathological signs of cutaneous leukocytoclastic vasculitis of small vessels, predominantly involving postcapillary venules [2,8,9,13]. This condition is characterized by chronic or recurrent episodes of urticaria, each element of which lasts more than 24 hours and is accompanied by a feeling of pain and burning [6,12].…”

“…UV can primarily affect the skin or spread systemically, affecting various organs, including the musculoskeletal, renal, respiratory, gastrointestinal, and visual systems [6]. UV has been described in children, the youngest patient was reported at the age of one year [13,15]. UV accounted for 9% of cases in a study of children with leukocytoclastic vasculitis [7].…”

“…At the moment, there are no uniform clinical recommendations, and approaches to the diagnosis and treatment of UV are different [8,9,13]. First of all, this is due to the variety of skin, systemic and serological signs, which make it difficult to establish a diagnosis.…”