In Urticarial Vasculitis, Long Disease Duration, High Symptom Burden, and High Need for Therapy Are Linked to Low Patient-Reported Quality of Life.

BACKGROUND: Urticarial vasculitis (UV) is a rare and difficult-to-treat chronic skin disease defined by long-lasting urticarial lesions and the histopathologic finding of leukocytoclastic vasculitis. As of yet, little is known about UV patients' perspective on the disease. OBJECTIVE: To assess UV patients' perspective on the clinical course, treatment response, greatest challenges, and quality-of-life (QOL) impairment. METHODS: A web-based questionnaire was disseminated in a Facebook group of patients with UV. Patients with UV confirmed by skin biopsy were included. RESULTS: Patients with UV had a mean age of 47.3 ± 12.3 years and were mostly female (94.3%; n = 82 of 87). The median delay in diagnosis was 8.1 months (interquartile range, 2.0-46.3). Normocomplementemia and hypocomplementemia were present in 54.0% (n = 27) and 46.0% (n = 23) of 50 patients, respectively. Most patients with UV (51.8%; n = 43 of 83) reported severely decreased QOL due to their disease. Low QOL was also the most frequently reported greatest challenge for patients with UV (40.7%), followed by the long-standing course of UV with frequent relapses (14.8%). Low QOL correlated with long disease duration (r = 0.298; P = .02) and high numbers of clinical symptoms (r = 0.294; P = .007). Patients with UV with allergies, lung diseases, and chronic infections reported lower QOL. Patients with UV with low QOL were treated with analgesics, dapsone, montelukast, omalizumab, and colchicine more often than patients with UV with higher QOL (P < .05 for all). CONCLUSIONS: Our results show a considerable impairment in QOL in patients with UV associated with long disease duration, high symptom burden, and a high need for therapy. Improvement of the management of UV by further research is necessary.

Urticarial vasculitis: Clinical and laboratory findings with a particular emphasis on differential diagnosis.

Urticarial vasculitis (UV) is a rare cutaneous vasculitis of small vessels characterized by recurrent episodes of wheal-like lesions that tend to last more than 24 hours, healing with a residual ecchymotic postinflammatory hyperpigmentation. The histopathologic pattern of UV is that of leukocytoclastic vasculitis, consisting of fibrinoid necrosis of dermal vessels' walls and neutrophil-rich perivascular inflammatory infiltrates. Although its etiopahogenesis remains still undefined, UV is now regarded as an immune complex-driven disease with activation of the complement cascade, leading to exaggerated production of anaphylatoxins that are responsible for neutrophil recruitment and activation. This condition can be categorized into 2 main entities according to serum complement levels: normocomplementemic UV and hypocomplementemic UV, the latter being associated with circulating anti-C1q autoantibodies and possible extracutaneous manifestations. Systemic multiorgan involvement may be seen particularly in syndromic hypocomplementemic UV, also known as McDuffie syndrome. This review summarizes the clinicopathological and laboratory features as well as the underlying pathophysiological mechanisms of UV. A focus on its main differential diagnoses is provided, that is, chronic spontaneous urticaria, bullous pemphigoid, IgA (Henoch-Schönlein purpura) and IgM/IgG immune complex vasculitis, lupus erythematous tumidus, Wells syndrome, erythema multiforme, cutaneous mastocytosis, cryopyrin-associated periodic syndromes, and coronavirus disease 2019-associated and anti-severe acute respiratory syndrome coronavirus 2-vaccine-associated urticarial eruptions.

Leukocytoclastic vasculitis after COVID-19 vaccination.

Leukocytoclastic vasculitis (LCV) is the vasculitis of small vessels. In this report, we describe a 38-year-old male patient who presented to our outpatient clinic with a 1-week history of rash on his lower extremities that had started 4 days after receiving the Pfizer-BioNTech SARS-CoV-2 vaccine. A diagnosis of LCV was made based on clinical and histopathological findings. The patient was treated with antihistamines and prednisolone, after which improvement was observed in the lesions. With this paper, we aim to raise awareness concerning the possibility of LCV development after COVID-19 vaccination.

Leucocytoclastic vasculitis secondary to COVID-19 infection in a young child.

The current case report describes a 13-year-old young boy who presented with purpuric rashes following a completely asymptomatic COVID-19 infection and biopsy-confirmed leucocytoclastic vasculitis, mild haematuria and mild elevation of serum IgA. This case highlights one of the dermatological manifestations of COVID-19 infection which has not been reported so far. Paediatricians should explore the history of this infection when evaluating any child presenting with a vasculitic rash.

Leucocytoclastic vasculitis in a patient with COVID-19 with positive SARS-CoV-2 PCR in skin biopsy.

Main skin manifestations of COVID-19 have been recently classified. However, little is known about cutaneous histopathological patterns and the presence of SARS-CoV-2 in these skin lesions. We present a healthy 29-year-old man who developed a leucocytoclastic vasculitis for COVID-19 with positive SARS-CoV-2 PCR in skin biopsy.