Cutaneous Vasculitis Research Articles

Cutaneous Small Vessel Vasculitis: A Rare Manifestation of Protein Supplement Nutritional Powder

Cutaneous Small Vessel Vasculitis: A Rare Manifestation of Protein Supplement Nutritional Powder

Ambroxol-induced leukocytoclastic vasculitis: A case report and literature review

Cutaneous leukocytoclastic vasculitis (LCV) that primarily affects the skin, can be idiopathic or linked to infections, autoimmune diseases, neoplasms, and medications. An immune complex-mediated vasculitis of the dermal capillaries and venules is the histological hallmark of LCV, a small vessel vasculitis. This case report describes a 36-year previously healthy man who was presented to the outpatient department (OPD) with complaints of edema and development of numerous reddish-purple patches (caused by petechiae) after intake of cough syrup containing ambroxol. Ambroxol affects human monocytic cells THP-1, and prevents the generation of platelet-derived growth factor (PDGF)-AB that is produced by lipopolysaccharide (LPS) by inhibiting the expression of PDGF-A messenger Ribonucleic acid (RNA). An extracellular signal-regulated kinase (ERK) on p44/42 is activated by LPS, and ambroxol reduces this activation. In addition, 2-(2-amino-3-methoxyphenyl)-4H-1-benzopyran-4-one (PD 98059), a Mitogen-Activated Protein Kinase Inhibitors (MEK), MEK-1-specific inhibitor, prevents p44/42 ERK activation and PDGF synthesis brought on by LPS. Megakaryocytes, erythrocytes, leukocytes, and their progenitors are stimulated to multiply by PDGF to the numerous endogenous growth factors that mesenchymal stem and stromal cells secrete. Ambroxol reduces the number of megakaryocytic progenitors and boosts apoptosis by inhibiting the production of PDGF and related signaling pathways. It is strongly advised that patients must be made more aware of the risks associated with using such over-the-counter medications.

Magnetic resonance enterography in diagnosing and monitoring of adult-onset IgA vasculitis (Henoch-Schönlein purpura) with gastro-intestinal involvement: Report of two cases

BackgroundIgA vasculitis (IgAV) is a small vessel vasculitis affecting children and, less commonly, adults; in the latter, gastro-intestinal (GI) involvement occurs in up to 50% of cases. Diagnosis is made with computed tomography, ultrasonography and endoscopy, but no data have been to date published about the use of magnetic resonance enterography (MRE). Aim of the workThis work reports two leading cases of adult-onset IgAV with GI involvement in which MRE was used at baseline and during follow-up. Case reportThe first case was for a 55-year old male presenting with microhematuria and elevated markers of inflammation at admission. MRE evidenced jejunum and proximal ileum diffuse and asymmetric thickening and hypervascularization. Leukocytoclastic vasculitis and glomerulonephritis with IgA deposit were evidenced at skin and renal biopsy, respectively. At one-year follow-up, a relapse was suspected: microhematuria and proteinuria were found at routine urinalysis, while MRE findings were similar to the ones at baseline. The second case was for a 49-year old male previously treated with methotrexate for mild seronegative arthritis, reporting hematochezia and worsening of arthralgias. Inflammatory bowel disease (IBD) was suspected and colonoscopy revealed diffuse mucosal hyperemia. Computerized tomography of the terminal ileum appeared thickened and edematous, while skin biopsy showed leukocytoclastic vasculitis with IgA infiltrate. The patient was successfully treated with oral prednisone and, after two months from discharge, MRE was negative. ConclusionIgAV may occur in adults and presenting with GI involvement may be particularly severe, mimicking IBD. MRE is a promising tool in diagnosing and monitoring IgAV.

Concurrent Streptococcus equi subsp. equi infection, purpura haemorrhagica and immune‐mediated myositis in a Quarter Horse filly

SummaryThis report describes a 2‐year‐old Quarter Horse filly that was diagnosed with concurrent Streptococcus equi subsp. equi infection, purpura haemorrhagica, and immune‐mediated myositis. The filly was presented for evaluation of fever of unknown origin, rapid weight loss and inappetence after exposure to strangles that was initially unknown. Over the course of hospitalisation the filly developed mandibular lymphadenopathy, guttural pouch empyema, and mucosal petechiation and ecchymoses. The presence of Streptococcus equi subsp. equi was confirmed via PCR and culture of a lymph node aspirate and biopsies of the skin confirmed leukocytoclastic vasculitis, consistent with purpura haemorrhagica. Genetic testing confirmed that the filly had one copy of the MYH1 mutation (N/My). Following therapy with systemic corticosteroids, plasma transfusion and antimicrobial therapy the filly improved and was discharged. Communication with the owner 11 months later confirmed that filly appeared healthy but remained poorly muscled. This report is the first to the authors' knowledge describing concurrent Streptococcus equi subsp. equi infection, purpura haemorrhagica, and immune‐mediated myositis.

Acute neutrophilic vasculitis (leukocytoclasia) in 36 COVID-19 autopsy brains

BackgroundHypercytokinemia, the renin-angiotensin system, hypoxia, immune dysregulation, and vasculopathy with evidence of immune-related damage are implicated in brain morbidity in COVID-19 along with a wide variety of genomic and environmental influences. There is relatively little evidence of direct SARS-CoV-2 brain infection in COVID-19 patients.MethodsBrain histopathology of 36 consecutive autopsies of patients who were RT-PCR positive for SARS-CoV-2 was studied along with findings from contemporary and pre-pandemic historical control groups. Immunostaining for serum and blood cell proteins and for complement components was employed. Microcirculatory wall complement deposition in the COVID-19 cohort was compared to historical control cases. Comparisons also included other relevant clinicopathological and microcirculatory findings in the COVID-19 cohort and control groups.ResultsThe COVID-19 cohort and both the contemporary and historical control groups had the same rate of hypertension, diabetes mellitus, and obesity. The COVID-19 cohort had varying amounts of acute neutrophilic vasculitis with leukocytoclasia in the microcirculation of the brain in all cases. Prominent vascular neutrophilic transmural migration was found in several cases and 25 cases had acute perivasculitis. Paravascular microhemorrhages and petechial hemorrhages (small brain parenchymal hemorrhages) had a slight tendency to be more numerous in cohort cases that displayed less acute neutrophilic vasculitis. Tissue burden of acute neutrophilic vasculitis with leukocytoclasia was the same in control cases as a group, while it was significantly higher in COVID-19 cases. Both the tissue burden of acute neutrophilic vasculitis and the activation of complement components, including membrane attack complex, were significantly higher in microcirculatory channels in COVID-19 cohort brains than in historical controls.ConclusionsAcute neutrophilic vasculitis with leukocytoclasia, acute perivasculitis, and associated paravascular blood extravasation into brain parenchyma constitute the first phase of an immune-related, acute small-vessel inflammatory condition often termed type 3 hypersensitivity vasculitis or leukocytoclastic vasculitis. There is a higher tissue burden of acute neutrophilic vasculitis and an increased level of activated complement components in microcirculatory walls in COVID-19 cases than in pre-pandemic control cases. These findings are consistent with a more extensive small-vessel immune-related vasculitis in COVID-19 cases than in control cases. The pathway(s) and mechanism for these findings are speculative.

New-onset autoimmune disease after COVID-19.

Severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) may trigger autoimmune disease (AD) through initial innate immune activation with subsequent aberrations in adaptive immune cells leading to AD. While there are multiple reports of incident AD diagnosed after COVID-19, the risk in the context of key circulating strains is unknown. TriNetX, a global, federated, health research network providing access to electronic medical records across 74 healthcare organizations, was utilized to define an adult cohort between January 1, 2020, and March 3, 2023. Exposure was defined as COVID-19 diagnosis (ICD-10 code or positive laboratory test). Age- and sex-propensity score-matched controls never had COVID-19 diagnosed. Outcomes were assessed 1 month to 1 year after the index date. Patients with AD prior to or within 1 month after the index date were excluded from the primary analysis. Incidence and risk ratios of each AD were assessed. A total of 3,908,592 patients were included. Of 24 AD patients assessed, adjusted risk ratios for eight AD patients who had COVID-19 were higher compared to those who had no COVID-19. Cutaneous vasculitis (adjusted hazard ratio (aHR): 1.82; 95% CI 1.55-2.13), polyarteritis nodosa (aHR: 1.76; 95% CI 1.15-2.70), and hypersensitivity angiitis (aHR: 1.64; 95% CI 1.12-2.38) had the highest risk ratios. Overall, psoriasis (0.15%), rheumatoid arthritis (0.14%), and type 1 diabetes (0.13%) had the highest incidence during the study period, and of these, psoriasis and diabetes were more likely after COVID-19. The risk of any AD was lower if COVID-19 was diagnosed when Omicron variants were the predominant circulating strains. A positive antinuclear antibody was more likely and predictive of AD after COVID-19. SARS-CoV-2 may be a potential trigger for some AD, but the risk for AD may decrease with time given the apparent lower risk after infection with Omicron variants.

Infective endocarditis is the leading cause of infection-associated cutaneous vasculitis: A single academic center dermatology consultant experience

Infective endocarditis is the leading cause of infection-associated cutaneous vasculitis: A single academic center dermatology consultant experience

Dapagliflozin-Induced Cutaneous Vasculitis, a Hitherto Unreported Adverse Effect.

Dapagliflozin-Induced Cutaneous Vasculitis, a Hitherto Unreported Adverse Effect.

Dermoscopic Features of Cutaneous Vasculitis.

Dermoscopy has become widespread in the diagnosis of inflammatory skin diseases. Cutaneous vasculitis (CV) is characterized by inflammation of vessels, and a rapid and reliable technique is required for the diagnosis. We aimed to define CV dermoscopic features and increase the diagnostic accuracy of dermoscopy with machine learning (ML) methods. Eighty-nine patients with clinically suspected CV were included in the study. Dermoscopic images were obtained before biopsy using a polarized dermoscopy. Dermoscopic images were independently evaluated, and interobserver variability was calculated. Decision Tree, Random Forest, and K-Nearest Neighbors were used as ML classification models. The histopathological diagnosis of 58 patients was CV. Three patterns were observed: homogeneous pattern, mottled pattern, and meshy pattern. There was a significant difference in background color between the CV and non-CV groups (P = 0.001). The milky red and livedoid background color were specific markers in the differential diagnosis of CV (sensitivity 56.7%, specificity 96.3%, sensitivity 29.4%, specificity 99.2%, respectively). Red blotches were significantly more common in CV lesions (P = 0.038). Red dots, comma vessels, and scales were more common in the non-CV group (P = 0.002, P = 0.002, P = 0.003, respectively). Interobserver agreement was very good for both pattern (κ = 0.869) and background color analysis (κ = 0.846) (P < 0.001). According to ML classifiers, the background color and lack of scales were the most significant dermoscopic aspects of CV. Dermoscopy may guide as a rapid and reliable technique in CV diagnosis. High accuracy rates obtained with ML methods may increase the success of dermoscopy.

Cutaneous vasculitis as the sole clinical feature of primary Sjögren’s syndrome

Cutaneous vasculitis as the sole clinical feature of primary Sjögren’s syndrome

ImmunoglobulinA vasculitis

The immune-mediated small vessel vasculitis is known as Schoenlein-Henoch purpura predominantly from pediatrics and in these cases occurs more frequently after infections of the upper airways. In adults, immunoglobulinA (IgA) vasculitis often proceeds more severely und recurrently with the classical tetrad of skin manifestations in the sense of leukocytoclastic vasculitis, joint affection, gastrointestinal involvement and IgA nephritis, in contrast to the mostly mild and self-limiting course in children. The background of this systemic vasculitis with formation of IgA immune complexes is considered to be an altered glycosylation of IgA, as this causes the exposure of binding sites for autoantibodies so that an immune complex reaction can be elicited. This ultimately leads to perivascular deposition of IgA and afurther activation of neutrophils. Groundbreaking in the diagnostics is the histological detection of leukocytoclastic vasculitis and in cases of renal manifestations akidney biopsy with characteristic deposits of immune complexes, which cannot be clearly differentiated from IgA nephropathy. The treatment is aimed at the respective manifestation and is mostly based on consensus recommendations due to the lack of randomized studies. In addition to immunosuppressive medication, in the presence of achronic kidney disease general nephroprotection is becoming increasingly more important also by inhibition of sodium-glucose transporter2 (SGLT2). The type and extent of kidney involvement and also rare cardiac manifestations are the main determinants of the prognosis. Continuous medical accompaniment of those affected is necessary due to the possible progression of the disease and the risk of recurrence.

A study on clinico- epidemiological profile &amp; histopathological profile of cutaneous vasculitis

: The study aimed to analyse the potential etiological factors, clinical presentation, past medical history &amp; laboratory findings in patients with biopsy-proven cutaneous vasculitis to better understand the disease and identify the disease classifications. The objective of this study was to establish a database of the clinic-epidemiological profile and histopathological features of cutaneous vasculitis in a tertiary hospital in Eastern Nepal.: A Descriptive cross-sectional study was planned to analyse and study the clinic-epidemiological profile and histopathological features of cutaneous vasculitis in Nepal. This study has reviewed the facts which were published earlier to determine the current scenario by vast study of statistics and derivation of facts. Proper examination of data was made to evaluate with final conclusion. The study included sample size of 30 consecutive patients with biopsy-proven cutaneous vasculitis attending the Dermatology outpatient department and admitted to the dermatology ward at B.P. Koirala Institute of Health Science, Dharan. : The study population of 30 patients with biopsy-proven cutaneous vasculitis, with an average age of 32.96 years, and a female-to-male ratio of 1.3:1. The majority of patients (66.67%) had lesions lasting less than 6 weeks, and acute disease was observed in 86.66% of the cases. The most common symptoms were itching (73.66%) and arthralgia (66.67%). Palpable purpura was the predominant cutaneous manifestation (70%), mainly distributed on the legs and ankles (93.33%). Elevated ESR was the most common laboratory abnormality (73.33%). Histopathological examination revealed leukocytoclastic vasculitis in 97% of cases, with predominantly neutrophilic infiltrates. Henoch-Schoenlein purpura (HSP) was the most common classification (43.33%), followed by urticarial vasculitis (26.67%).: This study provides valuable insights into the clinic-epidemiological profile and histopathological features of cutaneous vasculitis in the Nepalese population. It highlights the diverse clinical presentation and laboratory abnormalities associated with the condition, as well as potential etiological factors and disease classifications. The findings contribute to improved understanding, diagnosis, and management of cutaneous vasculitis. Further studies with larger cohorts are needed to validate and expand upon these findings.

Thymic mucosa-associated lymphoid tissue lymphoma in a patient with Sjögren’s syndrome with cutaneous vasculitis

Introduction. The association between Sj?gren?s syndrome (SS) and the development of lymphoma is well known. The prevalence of lymphoma in patients with SS is 5%. Mucosa-associated lymphoid tissue (MALT) lymphoma is the most common lymphoma type in patients with SS. It is common for MALT lymphoma to develop in the stomach, while it is extremely rare in the thymus. Case report. We present a 61-year-old Caucasian male patient with primary SS, cutaneous vasculitis, and thymic MALT lymphoma. The patient had a two-year history of diffuse cutaneous palpable purpura on legs, intermittently enlarged left parotid gland, and dry mouth. The results of Schirmer?s test were positive, labial salivary glands biopsy revealed a focus score ? 1, serology testing showed positive anti-Ro/SS-A and anti- La/SS-B antibodies, while skin biopsy findings revealed leukocytoclastic vasculitis. Diagnosis of primary SS with extraglandular cutaneous manifestations was confirmed. Cryoglobulinemia (Cg) and monoclonal gammopathy (MG) were detected, which increased the suspicion of hematological malignancy, and additional diagnostic procedures were performed. Computed tomography of the chest revealed an enlarged, multicystically altered anterior mediastinal mass. A thymectomy was performed through video-assisted thoracic surgery. Histological findings of the tissue confirmed the presence of tumor tissue consistent with MALT lymphoma in the thymus. Induction therapy with pulse doses of glucocorticoids was applied for three days, which was continued with medium doses of the drug. The doses were gradually reduced, and hydroxychloroquine was introduced. This has shown to be an effective therapy against features of SS. Postoperative local radiotherapy was performed. Conclusion. In SS patients with CV and in the presence of Cg and MG, attention should also be paid to the eventual development of MALT lymphoma, including the rare localization in the thymus.

Renal Medullary Angiitis Associated with Cutaneous Leukocytoclastic Vasculitis.

Renal medullary angiitis is characterized by interstitial hemorrhaging in the medulla with neutrophil infiltration. An 81-year-old man presented with a fever, kidney dysfunction, and purpura of the legs, which was diagnosed as leukocytoclastic vasculitis. Proteinase 3 antineutrophil cytoplasmic antibodies were weakly positive. A kidney biopsy showed severe tubulointerstitial hemorrhaging with neutrophilic infiltration in the perivascular areas surrounding the vasa recta in the medulla without crescent formation in the glomeruli. An immunofluorescence analysis was negative, and electron microscopy revealed no immune-dense deposits, ruling out immunoglobulin A vasculitis. Intravenous methylprednisolone for three days and plasma exchange followed by oral prednisolone improved his general condition.

Therapeutic management of cutaneous and ear tip vasculitis in dogs

Therapeutic management of cutaneous and ear tip vasculitis in dogs

Eosinophilic Gastroenteritis with Ascites, Elevated Serum IgG4, and Hypereosinophilic Syndrome: A Manifestation of IgG4-related Disease?

A 76-year-old woman with persistent diarrhea was referred to our hospital. She had purpura, peripheral eosinophilia (18,177/μL), and an elevated serum IgG4 level (819 mg/dL). Abdominal computed tomography revealed massive ascites and bowel edema. A skin biopsy of the purpura revealed leukocytoclastic vasculitis with prominent eosinophilic infiltration. Biopsies of the gastrointestinal mucosa revealed dense eosinophilic infiltration, indicating eosinophilic gastroenteritis (EG) associated with the hypereosinophilic syndrome. The number of IgG4-positive cells increased in the duodenal mucosa; however, the diagnostic criteria for IgG4-related disease (IgG4-RD) were not met. Whether or not EG with ascites is a manifestation of IgG4-RD warrants further investigation.

Juvenile dermatomyositis with central nervous system involvement: two case reports from a retrospective single-center cohort, with literature review.

Juvenile dermatomyositis (JDM) is a systemic autoimmune disease primarily involving the muscles and skin; it can also affect the central nervous system (CNS). The relevant literature provides limited information regarding the characteristics of JDM with CNS involvement. We reviewed patients with JDM who were hospitalized at our center between January 2016 and August 2023, with a focus on those with CNS involvement. The aim was to provide detailed case reports on these patients, and to summarize the relevant literature about the characteristics of similar cases. Among 193 hospitalized patients with JDM, two (1.03%) had CNS involvement. Two patients, a 5.5-year-old girl and an 11-year-old boy, were admitted with severe proximal muscle weakness and seizures, and presented with active cutaneous vasculitis. Both were ultimately diagnosed with JDM, with CNS involvement. Both patients had confirmed presence of anti-NXP2 antibody through myositis-specific antibody analysis. Additionally, they all exhibited hyperferritinemia and thrombocytopenia. Salvage therapies like intravenous methylprednisolone (IVMP) pulse therapy and/or plasma exchange were administered successfully. At final follow-up, both patients had achieved complete clinical response and full neurological recovery. Our literature review identified nine similar case studies. CNS involvement usually occurred within the first 10 months of the disease course, and most of these patients had fatal outcomes, with a mortality rate of 66.6% (6/9). Including the two patients described herein, the median age for disease onset is 10.5 years (range 4-17 years), and the male: female ratio is 6:5. Seizures are the most common neurological symptom, accompanied by active cutaneous vasculitis. The brain biopsies showed two distinct pathological presentations: one was central nervous system vasculitis, and the other was cerebral macrophage activation syndrome. CNS involvement is a rare but life-threatening JDM complication. Herein, our cases and the literature indicate that it typically occurs within the first 10 months of the disease course and manifests as seizures, often accompanied by active cutaneous vasculitis, with fatal outcomes. Timely implementation of salvage therapies, like IVMP pulse therapy and plasma exchange, may significantly impact patient outcomes.

From macular lymphocytic arteritis to cutaneous polyarteritis nodosa: A spectrum of the same disease? Two cases of the same clinical presentation yet different histopathological findings

Macular lymphocytic arteritis (MLA) is a type of indolent cutaneous vasculitis that affects small to medium-sized blood vessels. Histopathologically, it is characterized by a predominantly lymphocytic leukocytic infiltrate (unlike polyarteritis nodosa, in which neutrophils predominate) in the blood vessels of the deep dermis and superficial hypodermis. The disease mainly affects females (17:3), with a mean age of forty years. In general, this entity is considered benign with a variable clinical presentation. The importance of knowing this entity lies in the fact that the pathological findings are discordant with the clinic and an erroneous diagnosis may lead to unnecessary treatments. This vasculitis usually has a benign course and no progression to systemic disease. Herein, we present two cases of this rare vasculitis. Key words: Macular lymphocytic arteritis, Cutaneous polyarteritis nodosa, Vasculitis, Systemic lupus erythematosus

Severe cutaneous vasculitis as a symptom of right-sided infectious endocarditis in a patient with intravenous using of methadone: a clinical case

A bacterial infection of the heart&amp;rsquo;s endocardial surface causes infectious endocarditis (IE). The heart valves or an intracardiac devices are most affected. In most cases of right-sided endocarditis, the tricuspid valve is involved. Using drugs like cocaine, heroin, or any number of others can cause this disorder. Infective endocarditis can develop if methadone is not administered in accordance with the Methadone program, despite the cessation of drug use. In drug users, Staphylococcus aureus is the most common cause of right-sided infective endocarditis with pulmonary embolism and persistent bacteraemia. Cutaneous vasculitis, particularly leukocytoclastic vasculitis, is a rare manifestation of IE.

Paraneoplastic Cutaneous Leukocytoclastic Vasculitis as an Initial Presentation of Chronic Lymphocytic Leukemia.

Paraneoplastic Cutaneous Leukocytoclastic Vasculitis as an Initial Presentation of Chronic Lymphocytic Leukemia.