Leukocytoclastic vasculitis (LCV) is a histopathologic descriptor for a prevalent type of small-vessel vasculitis (SVV) that affects arterioles, capillaries, and postcapillary venules. Although the association between vasculitis and malignancy only accounts for <5% of vasculitis cases, it has been recognized as a true paraneoplastic syndrome in several studies. A 57-year-old Filipino male presented with erythematous, nonblanching macules on his lower extremities, which rapidly progressed to violaceous lesions on his trunk, buttocks, and lower extremities. He also reported significant weight loss, decreased appetite, and vomiting. A skin biopsy confirmed LCV. Initially treated for meningococcemia, his condition did not improve. Abdominal imaging revealed an enlarged heterogeneous liver with retroperitoneal lymphadenopathy and a parenchymal nodule. He was eventually diagnosed with vasculitis secondary to an underlying hepatic malignancy and expired later from multiorgan failure.

INTRODUCTION

Eosinophilic Granulomatosis Polyangiitis (EGPA) is the rarest among the ANCA-associated vasculitis with an incidence of seven per million individuals. Cardiac involvement occurs in 15-60% of patients and is the most severe manifestation associated with poor prognosis and mortality. EGPA typically affects the left side of the heart. There is only one published study to date that describes a case of right sided heart failure from pulmonary arterial hypertension.

A 40-year-old, Filipino, female, complained of rash, wheezing and right sided heart failure symptoms. After a thorough work-up, she was managed as a case of EGPA based on palpable, erythematous, nonpruritic rash on the lower extremities, peripheral eosinophilia (54%), adult-onset asthma, mononeuritis multiplex, cardiac symptoms, (+) p-ANCA and leukocytoclastic vasculitis with eosinophils and early granuloma formation on skin punch biopsy. The 2D-echocardiography showed an elevated estimated pulmonary pressure with signs of right sided volume overload. Chest computed tomography with contrast revealed right atrial and biventricular enlargement, hepatomegaly and unremarkable pulmonary findings. Methylprednisolone along with intravenous cyclophosphamide pulse therapy were initiated which resulted in the resolution of symptoms with normalization of blood eosinophils. Repeat 2D-echocardiogram had unremarkable findings as well. With the improvement noted, she was then maintained on glucocorticoids and mycophenolate mofetil.

Although EGPA commonly presents with symptoms of asthma, rhinosinusitis and/or peripheral eosinophilia, one uncommon presentation would be cardiac manifestations, specifically progressive pulmonary arterial hypertension with subsequent right sided heart failure. High dose glucocorticoids along with other immunosuppressants such as cyclophosphamide, are the treatment options in managing life-threatening conditions. Early detection is crucial in the prevention of grave outcomes.

Acute cerebral infarction predominantly occurs in middle-aged and elderly individuals with multiple underlying diseases， and it is often accompanied by atherosclerosis or heart diseases. It has a low incidence rate in children and adolescents， who tend to have atypical symptoms in the early stage， leading to misdiagnosis or missed diagnosis. With the continuous development of imaging technology， the diagnosis of cerebral infarction has become more precise， which helps to further clarify the etiology of cerebral infarction and identify an increasing number of patients with different subtypes of cerebral infarction in clinical practice. This article reports a rare case of a pediatric patient with acute cerebral infarction caused by primary central nervous system vasculitis， which led to neurological dysfunction， in order to improve the awareness of cerebral infarction in children among clinicians.
Vasculitis

Humans ; Lupus Vasculitis, Central Nervous System/pathology* ; Magnetic Resonance Imaging/methods* ; Diffusion Tensor Imaging/methods* ; Patients ; Lupus Erythematosus, Systemic/pathology* ; Brain/pathology*

BACKGROUND

Takayasu Arteritis (TA) is a rare, primary large-vessel vasculitis frequently leading to stenosis and less commonly, aneurysm formation. Saccular aneurysms of the aortic arch in patients with TA are fatal, have rarely been reported and represent a significant technical challenge due to the difficult anatomical location and need for protection of the cerebral circulation. Concomitant intracerebral aneurysms in patients with TA are extremely uncommon and have mostly been documented in very few case reports in literature.

We present a case of a 31 year-old Filipino female with recurrent chest and neck pain radiating to the upper back. Computed tomographic (CT) angiography demonstrated a large saccular aortic arch aneurysm without branch stenosis. CTA of the cerebral circulation likewise demonstrated multiple, saccular, intra-cerebral aneurysms. She underwent hybrid thoracic arch repair with supra-aortic debranching via mini-sternotomy and proximal ligation of the left common carotid artery and staged endovascular aortic arch replacement with coil embolization of the ostial-to-proximal left subclavian artery segment. Post-operative aortogram showed optimal repair with thrombosed aneurysmal sac, optimal graft position, no endoleaks and preservation of cerebral circulation. Patient improved symptomatically post-procedure and remained symptom-free during follow-up after six months. Careful review of local literature suggests that this is the first Philippine TA case with a saccular aortic arch aneurysm successfully managed in this manner.

Saccular aortic arch aneurysms in patients with Takayasu are unusual and presence of concomitant multiple cerebral saccular aneurysms have rarely been reported in literature. This case highlighted that hybrid endovascular arch repair in patients with TA is feasible, minimally invasive and effective.

Pemphigus erythematosus is an autoimmune chronic bullous disease involving the skin and mucous membranes. It is a variant of Pemphigus foliaceus with features of both pemphigus and lupus erythematosus, clinically presenting as flaccid bullae with crusted erosions in a seborrheic distribution, at times concurrent with more lupus-like discoid lesions. Several case reports have documented that Pemphigus erythematosus may have atypical presentation, mimicking contact dermatitis, small plaque parapsoriasis, or even seborrheic keratosis. This is a case of a 27-year old Filipino Female who presented with erythematous, non-blanching macules, patches, and plaques after intake of anti-TB medications. ANA immunofluorescence, anti-dsDNA, and anti-histone IgG were high while complement C3 was low. Histopathology was consistent with leukocytoclastic vasculitis. However, there was also noted subtle acantholysis with subcorneal blister. Direct immunofluorescence (DIF), on the other hand, showed granular intercellular deposits of IgG, granular intercellular deposits of lgA, and strong granular vascular deposits of fibrinogen. Due to financial constraints, ELISA Desmoglein 1 and 3 were done one month after starting the patient on systemic corticosteroids, giving a negative result. Still, coupling the histopathology and DIF result, the patient was diagnosed as a case of Pemphigus erythematosus with connective tissue-associated vasculitis. The patient was given systemic and topical corticosteroids with noted 90% improvement of erythematous patches and plaques after two weeks. This case demonstrated that pemphigus erythematosus may present with atypical lesions such as vasculitis. Careful examination and correlation of clinical features, laboratories, and histopathology is fundamental to arrive at a correct diagnosis.

Erythema elevatum diutinum (EED) is a rare, chronic leukocytoclastic vasculitis characterized by erythematous to violaceous plaques and nodules, typically on extensor surfaces like the hands, elbows, and knees. It results from immune complex deposition in blood vessels, leading to inflammation and fibrosis. EED is often associated with infections, autoimmune disorders, or hematologic malignancies, but can also occur idiopathically. Although globally documented, EED is extremely rare in the Philippines, particularly in adolescents, highlighting its significance in local literature.

A 14-year-old Filipino female presented with a 5-month history of asymptomatic, skin-colored papules on her right elbow, gradually spreading to both elbows and knees, with occasional mild pruritus and knee joint pain. Past medical and family history were unremarkable. After temporary relief from an unrecalled cream prescribed by a private dermatologist, she was referred for skin punch biopsy, which revealed spongiotic epidermis with papillary dermal edema, moderate inflammatory infiltrates, eosinophilic inclusion bodies, and nuclear dusts surrounding the blood vessels, consistent with EED. The patient was treated with dapsone 50 mg/day, clobetasol propionate ointment twice daily for two weeks, and cetirizine 10 mg as needed for pruritus, resulting in clinical improvement.

This case highlights the extreme rarity of EED in the Philippines, particularly in adolescents. It emphasizes the need to consider EED in chronic papular eruptions and demonstrates the effectiveness of dapsone and topical steroids in managing the condition. Early diagnosis and timely intervention are crucial for preventing disease progression and improving patient outcomes, as seen in this case.

Vasculitis refers to the inflammation and damage of blood vessel walls. This presents as erythematous macules or palpable purpura with a predilection for dependent parts, particularly the legs. Vesicles and bullae are less common manifestations. Vasculitis can be idiopathic or associated with infections, systemic diseases, drugs, or vaccines. Vaccine-associated vasculitis has been reported in BCG, influenza, hepatitis, and more recently, COVID-19 vaccines. However, there is limited data on the adverse events associated with rabies vaccine administration.

A 53-year-old Filipino male presented with petechiae evolving into purpura and urticarial targetoid plaques with bullae on the extremities and trunk, after administration of inactivated purified Vero cell rabies vaccine (Verorab, Sanofi Pasteur) due to a Category 3 bite. There were no mucosal lesions. Nikolsky and Asboe-Hansen signs were negative. The patient had no previous reactions to drugs or vaccines.

Histopathology results confirmed the diagnosis of vasculitis. Resolution of skin lesions was achieved with administration of intravenous steroids and oral antihistamines.

Community surveys conducted in three Philippine provinces report the incidence of animal bites at 50 injuries per 1,000 population (5 percent) per year with 45 percent of patients seeking medical treatment at animal bite treatment centers.

There is limited data on adverse events associated with rabies vaccine administration. In general, allergic reactions to vaccines are rare, occurring in approximately 1 in 50,000 to 1 in 1,000,000 doses. These reactions may be due to antigens, residual media, stabilizers, preservatives, or other excipients in the vaccine.

Rash, urticaria, and pruritus have been reported, along with injection site reactions such as erythema, edema, and pain. Fever, malaise, headaches, low mood, purpura, urticaria, Stevens-Johnson Syndrome and erythema multiforme may also occur.

Adequate documentation and reporting of adverse reactions due to vaccines contributes to long-term safety studies and establishing treatment guidelines.

Ultra-performance liquid chromatography-quadrupole time of fight/mass spectrometry(UPLC-Q-TOF-MS) and UNIFI were employed to rapidly determine the content of the components in Liangxue Tuizi Mixture. The targets of the active components and Henoch-Schönlein purpura(HSP) were obtained from SwissTargetPrediction, Online Mendelian Inheritance in Man(OMIM), and GeneCards. A "component-target-disease" network and a protein-protein interaction(PPI) network were constructed. Gene Ontology(GO) functional annotation and Kyoto Encyclopedia of Genes and Genomes(KEGG) pathway enrichment analysis were performed for the targets by Omishare. The interactions between the potential active components and the core targets were verified by molecular docking. Furthermore, rats were randomly assigned into a normal group, a model group, and low-, medium-, and high-dose Liangxue Tuizi Mixture groups. Non-targeted metabolomics was employed to screen the differential metabolites in the serum, analyze possible metabolic pathways, and construct the "component-target-differential metabolite" network. A total of 45 components of Liangxue Tuizi Mixture were identified, and 145 potential targets for the treatment of HSP were predicted. The main signaling pathways enriched included resistance to epidermal growth factor receptor tyrosine kinase inhibitors, phosphatidylinositol 3-kinase/protein kinase B(PI3K-AKT), and T cell receptor. The results of molecular docking showed that the active components in Liangxue Tuizi Mixture had strong binding ability with the key target proteins. A total of 13 differential metabolites in the serum were screened out, which shared 27 common targets with active components. The progression of HSP was related to metabolic abnormalities of glycerophospholipid and sphingolipid. The results indicate that the components in Liangxue Tuizi Mixture mainly treats HSP by regulating inflammation and immunity, providing a scientific basis for rational drug use in clinical practice.
Animals ; Rats ; IgA Vasculitis/drug therapy* ; Network Pharmacology ; Molecular Docking Simulation ; Phosphatidylinositol 3-Kinases ; Metabolomics

Both anti-glomerular basement membrane (GBM) disease and the anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) are common causes of pulmonary-renal syndrome. Organizing pneumonia (OP), a special pattern of interstitial lung disease, is extremely rare either in AAV or anti-GBM disease. We report an old woman presented with OP on a background of co-presentation with both ANCA and anti-GBM antibodies.
Female ; Humans ; Antibodies, Antineutrophil Cytoplasmic ; Organizing Pneumonia ; Autoantibodies ; Glomerulonephritis ; Anti-Glomerular Basement Membrane Disease ; Pneumonia ; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/complications*