Leukocytoclastic vasculitis in children: clinical characteristics, subtypes, causes and direct immunofluorescence findings of 56 biopsy‐confirmed cases

Johnson, Emma F.; Wetter, David A.; Lehman, Julia S.; Hand, Jennifer L.; Davis, Dawn M.; Tollefson, Megha M.

doi:10.1111/jdv.13952

Cited by 21 publications

(15 citation statements)

References 17 publications

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“…A skin biopsy within 24–48 h of the appearance of a new lesion is preferable [14]. Characteristic findings, as observed in our patient, include neutrophilic infiltration of the dermal small blood vessel walls associated with fibrinoid necrosis and disruption of the vessel wall, nuclear debris (leukocytoclasis), endothelial swelling, and extravasation of erythrocytes [7, 14].…”

Section: Discussion/conclusionmentioning

confidence: 92%

“…The annual incidence of IgA vasculitis is estimated at 0.8–1.8/100,000 in adults and 3–26.7/100,000 in children[6]. Among adults, idiopathic cutaneous small-vessel vasculitis and IgA vasculitis are the most common subtypes of leukocytoclastic vasculitis [7]. IgA vasculitis has been diagnosed in adults up to the age of 86 years [2].…”

Section: Discussion/conclusionmentioning

confidence: 99%

“…IgA vasculitis is most often idiopathic, though many triggers have been reported, including infections, neoplasms, inflammatory disorders, and certain drugs. In children, seasonal peaks, frequent occurrence following upper respiratory tract infection, and onset in index cases and in other family members all suggest a transmissible infectious process [6, 7]. Possible infectious triggers in adults include, but are not limited to, Streptococcus, Mycobacterium , hepatitis B and C, Staphylococcus aureus, Chlamydia, Neisseria, and HIV [10].…”

Section: Discussion/conclusionmentioning

confidence: 99%

“…Clinically, patients with IgA vasculitis present with palpable purpura located on the bilateral lower extremities. Symptoms may include tenderness, pain, and pruritus [7]. Two patients with alcohol-associated IgA vasculitis, including our patient, experienced symptomatic purpuric eruptions: tenderness and burning pain, respectively.…”

Section: Discussion/conclusionmentioning

confidence: 99%

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Alcohol-Associated Immunoglobulin A Vasculitis: A Case Report and Review of the Literature

et al. 2020

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Immunoglobulin A (IgA)-mediated leukocytoclastic vasculitis is a cutaneous small-vessel vasculitis characterized by skin findings of palpable purpura. It may occur secondary to infections, neoplasms, drugs, and systemic conditions, although it is most commonly idiopathic. A known, but rare, trigger for IgA vasculitis is alcohol consumption. We present a case of a man with IgA vasculitis associated with alcohol use and review the literature on alcohol-associated vasculitis. Although rarely reported, alcohol-associated IgA vasculitis is an important entity to consider for appropriate diagnosis and management of such patients.

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Section: Discussion/conclusionmentioning

confidence: 92%

Section: Discussion/conclusionmentioning

confidence: 99%

Section: Discussion/conclusionmentioning

confidence: 99%

Section: Discussion/conclusionmentioning

confidence: 99%

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Alcohol-Associated Immunoglobulin A Vasculitis: A Case Report and Review of the Literature

et al. 2020

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“…The most common are IgA vasculitis (IgAV) (formerly known as Schonlein-Henoch purpura, (SHP)), which represents more than half of the cases, followed by cutaneous small-vessel vasculitis (formerly known as hypersensitivity vasculitis). Other disorders, such as urticarial vasculitis or ANCA associated vasculitis are poorly represented in children 1 . The global incidence is not known 2 , while incidence of IgAV in children range from 3–26.7 per 100,000 3 .…”

Section: Introductionmentioning

confidence: 99%

Cutaneous vasculitis in children: A nationwide epidemiological study in Spain

Riancho‐Zarrabeitia

Santurtún

2017

F1000Res

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Background: Cutaneous vasculitis (CV) are a complex group of conditions in children, of which IgA vasculitis (IgAV) is the most common. The objectives of the current study are to describe the incidence of CV in Spain and to analyze the temporal trend in the last 11 years, as well as it seasonal distribution. Methods: Hospital discharges of patients aged 0-18 years with a diagnosis consistent with CV in Spain from 2005 to 2015 were collected from the Spanish National Institute of Statistics (INE) databases. Results: A total of 7304 patients from January 2005 to December 2015 were included; 6991 patients (95%) had a diagnosis of IgAV. The yearly incidence in the whole group was 7.7 per 100,000. Mean age at diagnoses was 6±3 years and 52% were male. The highest rate of admissions was found in the 5-9 year-old group, followed by those with 0-4 years of age (15.7 and 9.0 admissions per 100.000, respectively). Admissions due to CV followed an annual cyclic pattern, with the highest number of daily admissions during fall and winter months and the lowest number in summer months. There was an overall downwards trend of the number of hospital admissions during the period of study, in both males and females (p=0.01). Conclusions: We have estimated an incidence of a 7.7 cases per 100,000 CV in children in Spain. CV-related hospitalization rates have a marked seasonal pattern, with a peak in fall and winter and a nadir in summer months. Children between 5 and 9 years of age are most frequently affected. There is a decreasing trend in CV-related hospitalization, the causes of which should be further assessed.

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A novel histopathological scoring system to distinguish urticarial vasculitis from chronic spontaneous urticaria

Puhl

Bonnekoh

Hawro

et al. 2021

Clinical & Translational All

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Background: Urticarial vasculitis (UV) is defined by long-lasting urticarial lesions combined with the histopathologic findings of leukocytoclastic vasculitis. As one of the major unmet needs in UV, diagnostic criteria are rather vague and not standardized. Moreover, there seems to be considerable overlap with chronic spontaneous urticaria (CSU), particularly for the normocomplementemic variant of UV.Therefore, this study aimed to develop a diagnostic scoring system that improves the histopathologic discrimination between UV and CSU.Methods: Lesional skin sections of patients with clinical and histopathologic diagnosis of UV (n = 46) and CSU (n = 51) were analyzed (blinded to the diagnosis) for the following pre-defined criteria: presence of leukocytoclasia, erythrocyte extravasation, fibrin deposits, endothelial cell swelling, ectatic vessels, blurred vessel borders, dermal edema, intravascular neutrophil, and eosinophil numbers and numbers of dermal neutrophils, macrophages and mast cells.Results: The greatest differences between UV and CSU samples were observed for leukocytoclasia (present in 76% of UV vs. 3.9% of CSU samples; p < 0.0001), erythrocyte extravasation (present in 41.3% of UV vs. 2.0% of CSU samples; p < 0.0001), and fibrin deposits (present in 27.9% of UV vessels vs. 9.7% of CSU vessels; p < 0.0001). Based on these findings, we developed a diagnostic score, the urticarial vasculitis score (UVS), which correctly assigned 37 of 46 cases of UV and 49 of 51 cases of CSU to the previously established diagnosis. Conclusion:Our results suggest that the UVS, a combined quantitative assessment of the three criteria leukocytoclasia, fibrin deposits and extravasated erythrocytes, distinguishes UV from CSU in skin histopathology. The UVS, if validated in larger patient samples, may help to improve the diagnostic approach to UV.

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Leukocytoclastic vasculitis in children: clinical characteristics, subtypes, causes and direct immunofluorescence findings of 56 biopsy‐confirmed cases

Abstract: The most common subtype of biopsy-confirmed LCV in children is IgA vasculitis. Clinical features, exam characteristics and DIF results can be helpful in determining the subtype of cutaneous vasculitis in children.

Cited by 21 publications

References 17 publications

Alcohol-Associated Immunoglobulin A Vasculitis: A Case Report and Review of the Literature

Alcohol-Associated Immunoglobulin A Vasculitis: A Case Report and Review of the Literature

Cutaneous vasculitis in children: A nationwide epidemiological study in Spain

A novel histopathological scoring system to distinguish urticarial vasculitis from chronic spontaneous urticaria

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