AB0463 ETIOLOGY OF PALPABLE PURPURA; A SINGLE CENTER EXPERIENCE

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

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Diagnosis and management of leukocytoclastic vasculitis

Internal and Emergency Medicine ◽

10.1007/s11739-021-02688-x ◽

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.

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

Case Reports in Rheumatology ◽

10.1155/2014/850364 ◽

2014 ◽

Vol 2014 ◽

pp. 1-4 ◽

Cited By ~ 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.

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Antineutrophilic Cytoplasmic Antibody Positive Vasculitis Associated with Methimazole Use

Case Reports in Endocrinology ◽

10.1155/2015/530319 ◽

2015 ◽

Vol 2015 ◽

pp. 1-3 ◽

Cited By ~ 2

Author(s):

Deep Shikha ◽

Jonathan Harris ◽

Christine Resta ◽

Patricia Park

Keyword(s):

Drug Use ◽

Multinodular Goiter ◽

Leukocytoclastic Vasculitis ◽

Antithyroid Drug ◽

Skin Lesions ◽

Careful Monitoring ◽

Anca Associated Vasculitis ◽

Life Threatening ◽

Toxic Multinodular Goiter ◽

Life Threatening Complication

ANCA-associated vasculitis (AAV) is a rare and potentially life threatening complication associated with antithyroid drug use. It is more commonly reported with propylthiouracil, with fewer cases reported with methimazole use. We present the case of a 55-year-old man with toxic multinodular goiter which was treated with methimazole for 6 months. He developed ANCA positive leukocytoclastic vasculitis with hemorrhagic and necrotic bullous lesions of lower extremities. The vasculitis was initially thought to be secondary to recent cephalosporin use; however, the skin lesions progressed despite stopping the cephalosporin and treatment with steroids, and he developed osteomyelitis. His vasculitis resolved after cessation of methimazole use. This case highlights the importance of careful monitoring for variable manifestations of AAV in patients treated with methimazole.

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Leukocytoclastic Vasculitis as an Extraintestinal Manifestation of Crohn’s Disease

Case Reports in Gastroenterology ◽

10.1159/000519003 ◽

2021 ◽

pp. 825-831

Author(s):

Thiara Barcelos Rocha ◽

Ana Lorena Sousa de Vasconcelos Garate ◽

Rodrigo Fedatto Beraldo ◽

Sean Hideo Shirata Lanças ◽

Fábio Vicente Leite ◽

...

Keyword(s):

Leukocytoclastic Vasculitis ◽

Systemic Vasculitis ◽

Correct Diagnosis ◽

Lower Extremities ◽

Skin Lesions ◽

Systemic Autoimmune Disease ◽

Extraintestinal Manifestation ◽

Rare Manifestation ◽

Factor Therapy ◽

Skin Healing

Cutaneous involvement is one of the most common extraintestinal manifestations of inflammatory bowel disease (IBD). More commonly, pyoderma gangrenosum and erythema nodosum are noted, but psoriasis, aphthous stomatitis, Sweet’s syndrome, and vasculitis may also occur. Leukocytoclastic vasculitis (LCV) is a rare cutaneous manifestation, characterized by the appearance of palpable purpura, urticaria, and ulcer-necrotic lesions predominantly in the lower extremities that improve with immunosuppressive therapy. In this case, we report a patient with CD and LCV. We also searched the literature on the diagnosis and treatment of LCV in patients with CD. Female, 31, presented with diarrhea containing mucus and blood, abdominal pain, arthralgia, and enanthematous plaques and ulcers with a hematinic background in the lower extremities. The results of the colonoscopy were compatible with CD and skin biopsy showed signs of LCV. Systemic autoimmune disease and primary vasculitis were ruled out. The patient received treatment with a systemic corticosteroid and the skin lesions improved. Outpatient treatment with antitumor necrosis factor therapy was initiated to promote skin healing and IBD clinical remission. As LCV is a rare manifestation of IBD, it is necessary to distinguish this dermatopathy from other systemic vasculitis. The engagement of a multidisciplinary team is essential for the correct diagnosis and management.

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Erythema Multiforme and its Clinicopathological Features in a Horse in Brazil

Acta Scientiae Veterinariae ◽

10.22456/1679-9216.111736 ◽

2021 ◽

Vol 49 ◽

Author(s):

Pollyana Rennó Campos Braga ◽

Lissandro Gonçalves Conceição ◽

Fabricia Hallack Loures ◽

Roberta Martins Basso ◽

José Paes de Oliveira Filho ◽

...

Keyword(s):

Distribution Pattern ◽

Erythema Multiforme ◽

Histopathological Examination ◽

Clinical Investigation ◽

Leukocytoclastic Vasculitis ◽

Skin Lesions ◽

Hair Follicles ◽

Dietary History ◽

Immune Mediated ◽

Bilateral Distribution

Background: Erythema multiforme (EM) is an immune-mediated skin disease which may manifest as cutaneous or mucocutaneous lesions. It is uncommon in horses. EM lesions have a symmetrical bilateral distribution; they are usually urticarial, necrotizing, and, less commonly, ulcerative. In equines, the trigger is usually unknown, and cases are often classified as idiopathic. Diagnosis is based on a thorough history and physical and histopathological examination of lesions. According to the clinical presentation and histopathological characteristics of the cutaneous lesions, this case is the first report to describe diagnosis and treatment of a horse with EM in Brazil. Case: A Quarter Horse filly was followed clinically for 12 months after sudden onset of skin lesions at 18 months of age. The initial lesions were non-alopecic papules with a symmetrical bilateral distribution. Six months after onset, the skin lesions maintained the original distribution pattern; however, they had progressed to papules and plaques with varying annular, arciform, serpiginous, targetoid, or alopecic appearance. At 8 months, the same distribution pattern and appearance remained, but the lesions had become more severe and extensive, with involvement of the labial commissures and perineal region, without any erosions/ulcerations, scaling/crusting, pain, or pruritus. At 12 months, new nodular lesions were found on the medial and lateral surfaces of the hind limbs, neck, bilateral trunk, and root of the tail. The lesions were firm, non-pruritic, and non-tender on palpation. Swabs were obtained from the papular lesions. Skin specimens were also obtained with a 6-mm punch and via incisional biopsy and histological sections were made. Bacterial and fungal cultures were negative. Appropriate stains did not identify fungal structures, were negative for acid-fast bacilli, and did not reveal any metachromatic granules in the sampled cell population. The histopathological findings were characteristic of immune-mediated disease, with a vacuolar interface dermatitis affecting the hair follicles more than the epidermis, necrotic keratinocytes, lymphocyte satellitosis, leukocytoclastic mixed vasculitis of the mid-dermis and deep dermis, and variable granulation tissue, consistent with erythema multiforme and leukocytoclastic vasculitis. Immunosuppressive therapy with corticosteroids and oral supplementation with omega-3 and omega-6 fatty acids and vitamin E were prescribed. After institution of therapy, no new lesions developed, the existing lesions remained stable (though permanent), and hair regrew in the previously alopecic areas. All physiological parameters remained normal throughout the follow-up period. Discussion: Erythema multiforme is rarely reported in horses. According to our literature review, this is the first description of EM in horses in Brazil. EM should be included in the differential diagnosis of horses that present with plaques in a diverse, geographic distribution and a negative initial dermatological screening examination. Further clinical investigation is warranted, with special attention to potential antigenic triggers. A thorough drug and dietary history and close attention to comorbidities are essential, as the suppression of potential culprit factors has important prognostic value and contributes to the elucidation of EM triggers.

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A Rare Case of Spinal Leprosy Mimicking Spinal Tuberculosis

International Journal of Human and Health Sciences (IJHHS) ◽

10.31344/ijhhs.v0i0.151 ◽

2019 ◽

pp. 33

Author(s):

K Thuraikumar ◽

V Naveen ◽

Mustaqim A ◽

Arieff AA ◽

K Shri ◽

...

Keyword(s):

Back Pain ◽

Soft Tissue ◽

Histopathological Examination ◽

Posterior Instrumentation ◽

Spinal Tuberculosis ◽

Previous History ◽

Skin Lesions ◽

Titanium Implants ◽

Paravertebral Abscess ◽

History Of

Introduction: Spinal tuberculosis is the most common manifestation of extrapulmonar y tuberculosis. A combination of leprosy and tuberculosis is a rare entity.Case report: A 44-year-old male patient working as a laborer presented to our hospital with complaints of severe back pain and swelling over the back, difficulty in walking, associated with constitutional symptoms. On admission, he was febrile and had leukocytosis. Initial spine X-ray showed end plate destruction and increase in soft tissue shadow at the level of T8-T9. CT spine revealed thoracic paravertebral collection extending from T7 to T9 levels, suggest ive of tuberculous spondylitis with cold abscess. Patient refused a transpedicular biopsy and was started on anti-tubercular therapy. Two weeks after commencement of treatment, he developed worsening back pain and weakness of the lower extremities. MRI spine showed a paravertebral abscess and posterior soft tissue edema involving level of T7 to T11. Patient underwent a posterior decompression, debridement and posterior instrumentation. He was discharged well, there was improvement of his lower limb power. Upon clinic review, he complained of multiple hyperpigmented, painless, nonpruritic skin lesions over the trunk and back. No previous history of eczema, psoriasis and Tinea corporis. Given the history of allergy, initial impression was hypersensitivity reaction towards the titanium implants, and he was started on anti-histamines. However, there was no improvements seen. Histopathological examination of skin lesions revealed presence of granuloma within the dermis layer, composed of epitheloid, histiocytes, lymphocytes and plasma cells. Wade-Fite stain for Mycobacterium leprae is positive. Slit skin smear shows multibacillary leprosy. Patient was started on multidrug therapy (rifampicin, clofazimine and dapsone) for 1 year. He has recovered well.International Journal of Human and Health Sciences Supplementary Issue: 2019 Page: 33

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COVID-19: a Great Mime or a Trigger Event of Autoimmune Manifestations?

Current Rheumatology Reviews ◽

10.2174/1573397116666201005122603 ◽

2020 ◽

Vol 16 ◽

Author(s):

Maria Celeste Fatone

Keyword(s):

Autoimmune Diseases ◽

Systemic Vasculitis ◽

Skin Lesions ◽

Endothelial Damage ◽

Direct Role ◽

Acute Interstitial Pneumonia ◽

Trigger Event ◽

The Common ◽

Microvascular Inflammation ◽

Autoimmune Phenomena

: Viruses can induce autoimmune diseases, in addition to genetic predisposition and environmental factors. Particularly, coronaviruses are mentioned among the viruses implicated in autoimmunity. Today, the world's greatest threat derives from the pandemic of a new human coronavirus, called “severe acute respiratory syndrome coronavirus 2” (SARS-CoV-2), the responsible agent of coronavirus disease 2019 (COVID-19). COVID-19 originated in Wuhan, the capital of Hubei, China in December 2019 and, to date, has spread to at least 187 countries. This review focuses on autoimmune manifestations described during COVID-19, including pro-thrombothic state associated to antiphospholipid antibodies (aPL), acute interstitial pneumonia, macrophage activation syndrome, lymphocytopenia, systemic vasculitis, and autoimmune skin lesions. This offers the opportunity to highlight the pathogenetic mechanisms common to COVID19 and several autoimmune diseases, in order to identify new therapeutic targets. In a supposed preliminary pathogenetic model, SARS-CoV-2 plays a direct role in triggering widespread microthrombosis and microvascular inflammation, because it is able to induce transient aPL, endothelial damage and complement activation at the same time. Hence, endothelium might represent the common pathway in which autoimmunity and infection converge. In addition, autoimmune phenomena in COVID-19 can be explained by regulatory T cells impairment and cytokines cascade.

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

Pathogens ◽

10.3390/pathogens10010031 ◽

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.

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Stereotactic Radiotherapy in the Treatment of Paraneoplastic Vasculitis in Oligometastatic Renal Cell Carcinoma

Current Oncology ◽

10.3390/curroncol28030162 ◽

2021 ◽

Vol 28 (3) ◽

pp. 1744-1750

Author(s):

Laura Burgess ◽

Marissa Keenan ◽

Alan Liang Zhou ◽

Kiefer Lypka ◽

Delvina Hasimja Saraqini ◽

...

Keyword(s):

Renal Cell Carcinoma ◽

Cell Carcinoma ◽

Stereotactic Radiotherapy ◽

Renal Cell ◽

Systemic Vasculitis ◽

Primary Lesion ◽

Paraneoplastic Syndromes ◽

Healthy Male ◽

Palpable Purpura ◽

First Case

Approximately 20% of renal cell carcinoma (RCC) is diagnosed because of paraneoplastic manifestations. RCC has been associated with a large variety of paraneoplastic syndromes (PNS), but it is rarely associated with PNS vasculitis. We present a case of a previously healthy male who presented with systemic vasculitis; bitemporal headaches, diplopia, polyarthritis, palpable purpura, tongue lesion, peri-orbital edema, scleritis, chondritis and constitutional symptoms. He was subsequently found to have oligometastatic RCC. Both his primary lesion and site of oligometastasis were treated with stereotactic radiotherapy (SBRT) and resulted in the resolution of his vasculitis, as well as sustained oncologic response. This is the first case to demonstrate that effective sustained treatment for PNS vasculitis due to oligometastatic RCC is possible with SBRT.

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Leukocytoclastic Vasculitis in a Patient with Ankylosing Spondylitis

Case Reports in Rheumatology ◽

10.1155/2014/653837 ◽

2014 ◽

Vol 2014 ◽

pp. 1-3 ◽

Cited By ~ 1

Author(s):

Senol Kobak ◽

Hatice Yilmaz ◽

Ahmet Karaarslan ◽

Murat Yalcin

Keyword(s):

Low Back Pain ◽

Ankylosing Spondylitis ◽

Clinical Laboratory ◽

Morning Stiffness ◽

Leukocytoclastic Vasculitis ◽

Skin Lesions ◽

Low Back ◽

Hla B27 ◽

Positive Skin ◽

Rheumatology Clinic

A 26-year-old male patient presented to our rheumatology clinic with pain, swelling and limitation of movement in his right ankle, and also purpuric skin lesions in the lower extremity pretibial region. He was asked questions, and he said that he had been having chronic low back pain and morning stiffness for the last few years. His physical examination revealed that he had arthritis in his right ankle, purpuric skin lesions in pretibial regions of both legs, and bilateral FABERE/FADIR positivity. The sacroiliac joint imaging and MRI revealed bilateral sacroiliitis findings, and the lateral heel imaging revealed enthesitis. HLA-B27 was positive. Skin biopsy from lower skin lesions was reported to be consistent with leukocytoclastic vasculitis. Based on clinical, laboratory, radiological, and pathological examinations, the patient was diagnosed with ankylosing spondylitis and leukocytoclastic vasculitis. Administration of corticosteroid, salazopyrin, and nonsteroid anti-inflammatory medications was started. Notable clinical and laboratory regression was observed during his checks 3 months later.

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