scholarly journals Diagnosis and management of leukocytoclastic vasculitis

2021 ◽  
Author(s):  
Paolo Fraticelli ◽  
Devis Benfaremo ◽  
Armando Gabrielli
Keyword(s):  
Histopathological Examination ◽  
Leukocytoclastic Vasculitis ◽  
Systemic Vasculitis ◽  
Immunosuppressive Agents ◽  
Connective Tissue Diseases ◽  
Small Vessel Vasculitis ◽  
Serological Tests ◽  
Culprit Drug ◽  
Schonlein Purpura ◽  
Higher Doses

AbstractLeukocytoclastic vasculitis (LCV) is a histopathologic description of a common form of small vessel vasculitis (SVV), that can be found in various types of vasculitis affecting the skin and internal organs. The leading clinical presentation of LCV is palpable purpura and the diagnosis relies on histopathological examination, in which the inflammatory infiltrate is composed of neutrophils with fibrinoid necrosis and disintegration of nuclei into fragments (“leukocytoclasia”). Several medications can cause LCV, as well as infections, or malignancy. Among systemic diseases, the most frequently associated with LCV are ANCA-associated vasculitides, connective tissue diseases, cryoglobulinemic vasculitis, IgA vasculitis (formerly known as Henoch–Schonlein purpura) and hypocomplementemic urticarial vasculitis (HUV). When LCV is suspected, an extensive workout is usually necessary to determine whether the process is skin-limited, or expression of a systemic vasculitis or disease. A comprehensive history and detailed physical examination must be performed; platelet count, renal function and urinalysis, serological tests for hepatitis B and C viruses, autoantibodies (anti-nuclear antibodies and anti-neutrophil cytoplasmic antibodies), complement fractions and IgA staining in biopsy specimens are part of the usual workout of LCV. The treatment is mainly focused on symptom management, based on rest (avoiding standing or walking), low dose corticosteroids, colchicine or different unproven therapies, if skin-limited. When a medication is the cause, the prognosis is favorable and the discontinuation of the culprit drug is usually resolutive. Conversely, when a systemic vasculitis is the cause of LCV, higher doses of corticosteroids or immunosuppressive agents are required, according to the severity of organ involvement and the underlying associated disease.

scholarly journals AB0463 ETIOLOGY OF PALPABLE PURPURA; A SINGLE CENTER EXPERIENCE

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1530.1-1530
Author(s):  
G. Kaynar ◽  
O. C. İçaçan ◽  
S. Çelik ◽  
M. Yalçin Mutlu ◽  
C. Bes
Keyword(s):  
Drug Use ◽  
Histopathological Examination ◽  
Leukocytoclastic Vasculitis ◽  
Systemic Vasculitis ◽  
Skin Lesions ◽  
Serological Tests ◽  
Recent Infection ◽  
Important Place ◽  
Palpable Purpura ◽  
Underlying Diseases

Background:Purpura are lesions that occur after bleeding on the skin, mucous or serosal surfaces. Purpura can be classified into 2 subgroups; which are linked to thrombocytopenia and non-trombocytopenic purpura. While thrombocytopenic purpura often occurs due to a hematological disease; in non-trombocytopenic purpura etiological causes are very variable, and systemic vasculitis has an important place among them.Objectives:The demographic features of the patients applying with purpura and the underlying causes and diseases were aimed to be revealed.Methods:44 consecutive patients (22 women, 22 men) who were admitted to the hospital in the last 6 months, due to purpura were evaluated. Average age of patients was 49.6 ± 19.6 years. Patients were questioned about a recent infection, drug use, concomitant or underlying diseases. The serological tests and other laboratory tests for etiology were performed and biopsy was taken from the skin lesions which are appropriate.Results:While 4 (9%) patients had widespread purpura on the trunk-upper-lower extremities, in 22 (50%) patients purpuric lesions were limited only to the lower limb. The period between the onset of the first symptom and the admission to the hospital was longer than 4 weeks in 35 (79.5%) patients, and shorter than 1 week in 9 (20.5%) patients. 24 (54%) patients had an anamnesis of infection 2-3 weeks before purpura, and 20 (45%) patients had an anamnesis of drug use. The most common accompanying symptom was abdominal pain and was present in 15 (34%) patients. Biopsy was performed from the skin lesion in 37 patients. Histopathological examination of all was compatible with leukocytoclastic vasculitis. In indirect immunofluorescence staining, 17 were found to be IgA positive. 2 (4.5%) patients were diagnosed PR3-ANCA positive granulomatosis with polyangiitis. 1 patient had Hepatitis B virus infection was detected in 1 patient (2.2%), HIV infection was detected in 1 patient (2.2%) and malignancy was detected in 1 patient (2.2%).Conclusion:In our study, the most common reason was found as IgA vasculitis in patients presenting with palpable purpura. Although vasculitic involvement was limited to the skin in most patients, organ-threatening systemic vasculitis was detected in a few patients. Patients applying with Purpura should be questioned for infection and drug use, should be examined for underlying diseases including systemic vasculitis, and closely monitored for organ involvement.Disclosure of Interests:None declared

scholarly journals The enigma of adult onset Henoch Schoenlein purpura and leucocytoclastic Vasculits: a case report

2021 ◽  
Vol 7 (3) ◽  
pp. 482
Author(s):  
Vasudha A. Belgaumkar ◽  
Ravindranath B. Chavan ◽  
Nitika S. Deshmukh ◽  
Ranjitha K. Gowda
Keyword(s):  
Connective Tissue ◽  
Histopathological Examination ◽  
Leukocytoclastic Vasculitis ◽  
Connective Tissue Diseases ◽  
Bed Rest ◽  
Final Diagnosis ◽  
Small Vessel ◽  
Small Vessel Vasculitis ◽  
Adult Onset ◽  
Diffuse Abdominal Pain

<p class="abstract">Leukocytoclastic vasculitis (LCV), also known as hypersensitivity vasculitis is a small vessel inflammatory disease which mainly involves the postcapillary venules. It can be idiopathic or secondary to connective tissue diseases, infection, malignancy. A 39 year old male presented with a recurrent episode of multiple palpable purpurae over the legs, trunk, back and upper limbs with diffuse abdominal pain, bloody diarrhoea and arthralgia. Anti-nuclear antibodies (ANA) were positive, but ANA profile was equivocal. Histopathological examination confirmed leukocytoclastic vasculitis. A final diagnosis of Henoch Schoenlein purpura (HSP) was reached based on EULAR Criteria. He was treated with tapering doses of prednisolone, hydroxychloroquine and bed rest with limb elevation. All symptoms and lesions gradually resolved. Various sub-types of cutaneous small vessel vasculitis may mimic each other, warranting a meticulous evaluation. Adult onset HSP though rare must be considered in the differential diagnosis of LCV, which might be the presenting manifestation of an underlying connective tissue disease. Hence long-term follow-up with stringent clinical and laboratory monitoring is recommended.</p>

scholarly journals Atypical Bacterial Pathogens and Small-Vessel Leukocytoclastic Vasculitis of the Skin in Children: Systematic Literature Review

Pathogens ◽  
2021 ◽  
Vol 10 (1) ◽  
pp. 31
Author(s):  
Céline Betti ◽  
Pietro Camozzi ◽  
Viola Gennaro ◽  
Mario G. Bianchetti ◽  
Martin Scoglio ◽  
...  
Keyword(s):  
Mycoplasma Pneumoniae ◽  
Legionella Pneumophila ◽  
Leukocytoclastic Vasculitis ◽  
Systemic Vasculitis ◽  
Bacterial Pathogen ◽  
Small Vessel ◽  
Small Vessel Vasculitis ◽  
Systemic Involvement ◽  
Symptomatic Disease ◽  
Atypical Pathogen

Leukocytoclastic small-vessel vasculitis of the skin (with or without systemic involvement) is often preceded by infections such as common cold, tonsillopharyngitis, or otitis media. Our purpose was to document pediatric (≤18 years) cases preceded by a symptomatic disease caused by an atypical bacterial pathogen. We performed a literature search following the Preferred Reporting of Systematic Reviews and Meta-Analyses guidelines. We retained 19 reports including 22 cases (13 females and 9 males, 1.0 to 17, median 6.3 years of age) associated with a Mycoplasma pneumoniae infection. We did not find any case linked to Chlamydophila pneumoniae, Chlamydophila psittaci, Coxiella burnetii, Francisella tularensis, or Legionella pneumophila. Patients with a systemic vasculitis (N = 14) and with a skin-limited (N = 8) vasculitis did not significantly differ with respect to gender and age. The time to recovery was ≤12 weeks in all patients with this information. In conclusion, a cutaneous small-vessel vasculitis with or without systemic involvement may occur in childhood after an infection caused by the atypical bacterial pathogen Mycoplasma pneumoniae. The clinical picture and the course of cases preceded by recognized triggers and by this atypical pathogen are indistinguishable.

scholarly journals Koebner phenomenon in leukocytoclastic vasculitis: A case report and an updated review of the literature

2019 ◽  
Vol 7 ◽  
pp. 2050313X1985035
Author(s):  
Farah Kassam ◽  
Sabrina Nurmohamed ◽  
Richard M Haber
Keyword(s):  
Case Report ◽  
Leukocytoclastic Vasculitis ◽  
Immunoglobulin A ◽  
Small Vessel Vasculitis ◽  
Review Of The Literature ◽  
Henoch Schonlein Purpura ◽  
Koebner Phenomenon ◽  
Vessel Injury ◽  
Schonlein Purpura ◽  
Henoch Schönlein Purpura

Leukocytoclastic vasculitis is the most common form of cutaneous vasculitis. It is a neutrophilic small vessel vasculitis resulting from the deposition of circulating immune complexes. Henoch-Schonlein purpura is a systemic type of leukocytoclastic vasculitis, characterized by immunoglobulin A-mediated blood vessel injury. We present a case of Henoch-Schonlein purpura in an adult female manifesting with a vasculitic rash with Koebner phenomenon.

scholarly journals Genetic Factors Associated with Rheumatoid Arthritis and Systemic Vasculitis: Evaluation of a Panel of Polymorphisms

2009 ◽  
Vol 27 (5) ◽  
pp. 217-223 ◽  
Author(s):  
Elisa Menegatti ◽  
Annalisa Davit ◽  
Simona Francica ◽  
Daniela Berardi ◽  
Daniela Rossi ◽  
...  
Keyword(s):  
Rheumatoid Arthritis ◽  
Connective Tissue ◽  
Frequency Analysis ◽  
Gene Polymorphisms ◽  
Genetic Factors ◽  
Systemic Vasculitis ◽  
Connective Tissue Diseases ◽  
Study Cohort ◽  
Mixed Cryoglobulinaemia ◽  
Schonlein Purpura

Immune and inflammatory response activation is a common feature of connective tissue diseases and systemic vasculitis. The aim of our study was to evaluate the possible involvement of TNFα c.-308A > G, IL-10 c.-1082A > G, uteroglobin c.38A > G, TGFβ 1 c.869C > T and NFκB2 c.-1837T > C gene polymorphisms in susceptibility to connective tissue diseases. Our study cohort included 68 unrelated patients affected by rheumatoid arthritis (RA) (37 patients) and ANCA-positive [micropolyangiitis (mPA) 17 patients] or ANCA-negative systemic vasculitis [including 8 patients with Henoch-Schönlein purpura (HSP) and 6 patients with mixed cryoglobulinaemia (MC)] as well as 98 control subjects. Allele frequency analysis of uteroglobin c.38G > A polymorphism showed a significant increase in the c.38A allele in patients (p= 0.002). Genotype frequency analysis of uteroglobin and NF-κB2 gene polymorphisms in patients showed an increase in c.38GA and c.38AA genotypes in the uteroglobin gene (p=0.02) coupled with an increase in homozygous c.-1837CC in the NF-κB2 gene (p=0.02). Our data suggest that genetic variation in UG and NF-κB2 pathways could have effects in connective tissue disease susceptibility.

scholarly journals Large Pyoderma Gangrenosum-Like Ulcers: A Rare Presentation of Granulomatosis with Polyangiitis

2014 ◽  
Vol 2014 ◽  
pp. 1-4 ◽  
Author(s):  
Basheer Tashtoush ◽  
Roya Memarpour ◽  
Yasmin Johnston ◽  
Jose Ramirez
Keyword(s):  
Pyoderma Gangrenosum ◽  
Granulomatosis With Polyangiitis ◽  
Leukocytoclastic Vasculitis ◽  
Systemic Vasculitis ◽  
Rare Presentation ◽  
Serological Tests ◽  
Cutaneous Manifestations ◽  
Wide Range ◽  
Common Skin ◽  
Nasal Septum Perforation

Granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis (WG), is a rare systemic vasculitis that classically manifests as necrotizing granulomas of the upper and lower respiratory tract, kidneys, and blood vessels; however, it may affect any organ system, including the skin. Cutaneous manifestations occur in up to 45% of patients during the disease course, and are the presenting feature in 9% to 14% of patients. The most common skin lesion specific to GPA is palpable purpura, with the histopathologic correlate of leukocytoclastic vasculitis. However, a wide range of clinical and histologic features may be seen. We herein report a case of a previously healthy 52-year-old Caucasian man who presented with multiple progressively enlarging painful ulcers on his face, upper extremities, back, and abdomen over a two-month period. Skin biopsies revealed pyoderma gangrenosum-like features. Serological tests were positive for PR3/c-ANCA. Six months later, the patient developed recurrent episodes of sinusitis associated with nasal bleeds and eventually nasal septum perforation. Despite aggressive treatment with Cyclophosphamide and steroids over one year, the patient had persistent nonhealing large ulcers and developed multiple lung nodules with cavitary lesions.

scholarly journals Henoch-Schonlein Purpura—A Case Report and Review of the Literature

2010 ◽  
Vol 2010 ◽  
pp. 1-7 ◽  
Author(s):  
Amit B. Sohagia ◽  
Srinivas Guptha Gunturu ◽  
Tommy R. Tong ◽  
Hilary I. Hertan
Keyword(s):  
Early Recognition ◽  
Leukocytoclastic Vasculitis ◽  
Systemic Disease ◽  
Renal Involvement ◽  
Symptomatic Treatment ◽  
Adolescent Male ◽  
Small Vessel Vasculitis ◽  
Henoch Schonlein Purpura ◽  
Schonlein Purpura ◽  
Henoch Schönlein Purpura

We describe a case of an adolescent male with Henoch-Schonlein purpura (HSP), presenting with cutaneous and gastrointestinal manifestations. Endoscopy revealed diffuse ulcerations in the stomach, duodenum, and right colon. Biopsies revealed a leukocytoclastic vasculitis in the skin and gastrointestinal tract. Steroid therapy led to complete resolution of the symptoms. HSP is the most common childhood vasculitis, and is characterized by the classic tetrad of nonthrombocytopenic palpable purpura, arthritis or arthralgias, gastrointestinal and renal involvement. It is a systemic disease where antigen-antibody (IgA) complexes activate the alternate complement pathway, resulting in inflammation and small vessel vasculitis. Mild disease resolves spontaneously, and symptomatic treatment alone is sufficient. Systemic steroids are recommended for moderate to severe HSP. The prognosis depends upon the extent of renal involvement, which requires close followup. Early recognition of multiorgan involvement, especially outside of the typical age group, as in our adolescent patient, and appropriate intervention can mitigate the disease and limit organ damage.

scholarly journals Henoch-Schonlein purpura masquerading as vesiculobullous lesions in a child

2020 ◽  
Vol 8 (1) ◽  
pp. 170
Author(s):  
Rajeshwari N. ◽  
Prahada J. ◽  
Kala K.
Keyword(s):  
Leukocytoclastic Vasculitis ◽  
Lower Limbs ◽  
Small Vessel Vasculitis ◽  
Direct Immunofluorescence ◽  
Henoch Schonlein Purpura ◽  
Diagnostic Challenge ◽  
Serum Iga ◽  
Ulcerative Lesions ◽  
Schonlein Purpura ◽  
Henoch Schönlein Purpura

Henoch-Schönlein purpura (HSP) is a systemic small vessel vasculitis occurring commonly in children presenting with palpable non-thrombocytopenic purpura, arthralgia and abdominal pain. Haemorrhagic bullous and ulcerative lesions are rare in HSP in children and can be a diagnostic challenge. We present a case of 6-year-old boy child who presented with bullous purpuric lesions in lower limbs, arthralgia and increased serum IgA. Histopathology of skin lesion revealed leukocytoclastic vasculitis. However, the direct immunofluorescence was negative for IgA deposits. 

scholarly journals AB0511 INTERNATIONAL CONSENSUS ON ANCA TESTING AND INTERPRETATION BEYOND SYSTEMIC VASCULITIS

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1553.2-1553
Author(s):  
S. Moiseev ◽  
J. W. Cohen Tervaert ◽  
Y. Arimura ◽  
D. Bogdanos ◽  
C. Elena ◽  
...  
Keyword(s):  
Interstitial Pneumonia ◽  
Lupus Erythematosus ◽  
Inflammatory Bowel Diseases ◽  
Systemic Vasculitis ◽  
Connective Tissue Diseases ◽  
Idiopathic Interstitial Pneumonia ◽  
Small Vessel Vasculitis ◽  
Research Support ◽  
Bowel Diseases ◽  
Inflammatory Bowel

Background:ANCA can be detected in sera from patients with autoimmune, inflammatory, infectious or neoplastic diseases.Objectives:To issue a Consensus Statement on ANCA testing and interpretation beyond systemic vasculitis.Methods:This Statement was prepared by a group of experts, based on the results of a comprehensive search in PubMed.Results:In certain settings beyond systemic vasculitis, ANCA may have diagnostic, clinical, and/or prognostic relevance. Testing for PR3- and MPO-ANCA by specific immunoassays should be performed in any patient with clinical features suggesting ANCA-associated vasculitis and in patients with anti-GBM disease and idiopathic interstitial pneumonia. Routine ANCA testing is not recommended in patients with connective tissue diseases (CTD), autoimmune liver diseases, inflammatory bowel diseases, infections, and/or malignancy unless there is evidence for small vessel vasculitis. ANCA testing by specific immunoassays may be useful in patients with rheumatoid arthritis, systemic sclerosis or primary Sjögren’s syndrome who have kidney disease with a nephritic sediment or in patients with systemic lupus erythematosus if a kidney biopsy shows prominent necrotizing and crescentic lesions or proliferative lupus nephritis. ANCA testing may be justified in patients with suspected autoimmune hepatitis type 1, who do not have conventional disease-related autoantibodies, or in patients with inflammatory bowel diseases in case of diagnostic uncertainty to discriminate ulcerative colitis from Crohn’s disease. In these cases, ANCA should be tested by indirect immunofluorescence since target antigens are not well characterized. ANCA against bactericidal/permeability-increasing protein may be a biomarker for deteriorating lung function and a poor prognosis in patients with cystic fibrosis.Conclusion:ANCA testing is clinically relevant not only in patients with manifestations suggesting systemic vasculitis, but also in patients with certain other disorders, particularly in patients with anti-GBM disease or idiopathic interstitial pneumonia.Disclosure of Interests:Sergey Moiseev Grant/research support from: This work was supported by the 5-100 Project, Sechenov University, Moscow, Jan Willem Cohen Tervaert: None declared, Yoshihiro Arimura: None declared, Dimitrios Bogdanos: None declared, Csernok Elena: None declared, Jan Damoiseaux: None declared, Marc Ferrante: None declared, Luis Felipe Flores-Suárez: None declared, Marvin Fritzler: None declared, Pietro Invernizzi: None declared, David Jayne Grant/research support from: ChemoCentryx, GSK, Roche/Genentech, Sanofi-Genzyme, Consultant of: Astra-Zeneca, ChemoCentryx, GSK, InflaRx, Takeda, Insmed, Chugai, Boehringer-Ingelheim, J. Charles Jennette: None declared, Mark Little: None declared, Stephen P. McAdoo: None declared, Pavel Novikov Grant/research support from: This work was supported by the 5-100 Project, Sechenov University, Moscow, Charles D. Pusey: None declared, Antonella Radice: None declared, Alan D. Salama: None declared, Judith Savige: None declared, Mårten Segelmark: None declared, Yehuda Shoenfeld: None declared, Renato Alberto Sinico: None declared, Maria Jose Rego de Sousa: None declared, Ulrich Specks: None declared, Benjamin Terrier: None declared, Athanasios Tzioufas: None declared, Severine Vermeire: None declared, Ming-hui Zhao: None declared, Xavier Bossuyt: None declared

scholarly journals Henoch-Schonlein Purpura in Children: The Role of Corticosteroids

2019 ◽  
Vol 7 (11) ◽  
pp. 1812-1814
Author(s):  
Bella Kurnia
Keyword(s):  
Abdominal Pain ◽  
Renal Disease ◽  
Systemic Vasculitis ◽  
Small Vessel ◽  
Small Vessel Vasculitis ◽  
Henoch Schonlein Purpura ◽  
Progression Of Renal Disease ◽  
Schonlein Purpura ◽  
Henoch Schönlein Purpura

BACKGROUND: Henoch- schonlein purpura (HSP) is an IgA- mediated systemic small vessel vasculitis. It is the most common form of systemic vasculitis in children.CASE REPORT: A 9 years old girl admitted to the hospital with chief complain of purplish red rash on both legs since approximately 1 week with painful knees and ankles that make the patient unable to walk. The patient was diagnosed with HSP and was treated with corticosteroid and analgesics. The patients only stayed for 2 nights at the hospital and discharged from the hospital with the ability to walk and experience no pain. CONCLUSION: The role of corticosteroids in the treatment of HSP is still controversial. But from various research, we can conclude that the role of corticosteroid in HSP is as a symptom reliever (reduce abdominal pain and arthritis), but does not slow the progression of renal disease.

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