Aetiology of Vascular Purpura in a Single Centre Experience: Contribution of Clinical and Paraclinical Data.

Background: Vascular purpura can be the clinical expression of infectious, inflammatory, drug-related, neoplastic, and endocrine pathologies. To date, there is no consensus codifying the investigation of vascular purpura, especially when it is isolated. Patients and methods: We proposed to study through a retrospective study of 73 cases of vascular purpura, occurring during the period 2004-2019 in our internal medicine department, the contribution of various clinical and paraclinical data to the aetiological diagnosis of vascular purpura. Data were considered to be contributory only when they constituted a solid argument in favour of the aetiological diagnosis of vascular purpura. Results: Our series involved 73 patients including 41 women and 32 men (Gender ratio: 0.78). Mean age was 49 ± 17 years [16-80]. Vascular purpura was isolated in 3% of cases. For the remaining patients, it was associated with functional (91%) or physical (48%) manifestations. It was associated with other skin lesions in 45% of cases. The accepted aetiologies were primary vasculitis (26%), drug-related (15%), infectious (11%) and secondary to connectivitis (10%). No cause was found in a third of cases. Clinical data alone made it possible to suggest the aetiology in more than half of cases. Special investigations were contributory in 46% of cases. The course was contributory in 18% of patients for drug-related and paraneoplastic causes. Conclusion: vascular purpura's diverse clinical presentation presents diagnostic challenges. Aetiologies include vasculitis, drug reactions, infections, and connective tissue disorders. Comprehensive clinical assessment is essential.

Cutaneous leukocytoklastic vasculitis in a patient with ankylosing spondylitis: A case report.

Extra-articular manifestations (EAMs) in ankylosing spondylitis (AS) are common and most extra-articular manifestations such as acute iritis and inflammatory bowel disease are positively correlated with disease activity of AS. Vasculitis is an extra-articular manifestation of AS. However cutaneous leukocytoclastic vasculitis (CLV) is uncommon in AS patients. In this article, we report a case of a 66-year-old female patient who has had AS for long time. Although the patient's articular manifestations were stable, the aortic aneurysm and CLV continued to occur sequentially. This article reminds clinicians that even AS patients with stable articular manifestations should be followed up regularly. All extra-articular manifestations of AS patients should be taken seriously and treated as soon as possible under the guidance of rheumatoid immunologists.

Tofacitinib as monotherapy in cutaneous polyarteritis nodosa: a case series.

Objective: Cutaneous polyarteritis nodosa (CPAN) is a distinct clinical entity represented by a chronic, relapsing, benign course, with rare systemic involvement. Treatment is with CSs, CYC or other conventional synthetic DMARDs (csDMARDs). In this case series, we aimed to share our varied clinical experience of successfully treating patients with CPAN, with tofacitinib in a refractory/relapsing course or as upfront monotherapy without CSs/csDMARDs. Methods: We report this retrospective case series managed at our rheumatology centre in Bangalore from 2019 to 2022. Four patients identified as CPAN on biopsy were able to achieve disease-free remission with tofacitinib as part of their treatment, with no relapse on further follow-up. Our patients presented with subcutaneous nodules and cutaneous ulcers. After systemic evaluation, all the patients underwent skin biopsy, which showed fibrinoid necrosis in the vessel walls of the dermis, with a histopathological impression of CPAN. They were initially treated with a conventional approach of CSs with/without csDMARDs. On experiencing a refractory/relapsing course, tofacitinib was tried in all the patients as either CS sparing or upfront monotherapy without concomitant csDMARDs. Results: Use of tofacitinib resulted in improvement of ulcers and paraesthesia and in gradual healing of skin lesions, albeit with scarring, with no further recurrence or relapse over a follow-up period of 6 months for all the patients. The therapeutic effect of tofacitinib was consistent when used either as CS sparing or as upfront monotherapy, thereby proving the drug to be a promising option that warrants larger trials in future to treat the subset of patients with established CPAN. Conclusion: Tofacitinib could be used for disease-free remission as monotherapy for CPAN either upfront or as CS sparing, even without concomitant csDMARDs, in those patients who are dependent on CSs or multiple DMARDs.

Cyclin-D1 positive B cell lymphoma with intravascular and interstitial involvement of kidney and bone marrow.

Kidney involvement is rare in non-Hodgkin's lymphomas (NHL), and can be caused by direct lymphoid infiltration, paraneoplastic glomerulonephritis (GN), or both. Herein, we present a case of acute kidney injury (AKI) due to renal cyclin-D1 positive B cell lymphoma infiltration. To our knowledge, this is the first case where a renal biopsy revealed intravascular neoplastic cells along with parenchymal infiltration.

Expert perspectives on pathological findings in vasculitis.

Pathological findings are important in the diagnosis of vasculitis. However, due to the rarity of the disease, standard textbooks usually devote only a few pages to this topic, and this makes it difficult for clinicians not specializing in vasculitis to fully understand the pathological findings in vasculitis. To address the paucity of information, we present representative pathological findings in vasculitis classified in the 2012 Revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides (CHCC2012). The CHCC2012 classifies 26 vasculitides into seven categories: (1) large-vessel vasculitis, (2) medium-vessel vasculitis, (3) small-vessel vasculitis, including antineutrophil cytoplasmic antibody-associated vasculitis and immune complex small-vessel vasculitis, (4) variable-vessel vasculitis, (5) single-organ vasculitis, (6) vasculitis associated with systemic disease, and (7) vasculitis associated with probable aetiology. Moreover, representative pathological findings of vasculitis-related diseases and non-inflammatory vasculopathy not mentioned in the CHCC2012 are also presented. This will be useful for clinicians to refer to typical pathological findings of vasculitis in daily practice.

Eosinophilic granulomatosis with polyangiitis after COVID-19: A case report.

COVID-19 can damage the endothelial cells of every organ in the body and lead to vasculopathy and vasculitis. It has been shown that various types of vasculitis could be a new manifestation of COVID-19. Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic necrotizing vasculitis that affects small vessels. Here we report our experience with a 42-year-old man with a 3-weeks history of fever of unknown origin after two months from COVID-19 recovery presented with loss of appetite, loss of weight, and paresthesia in his lower extremities. After required evaluations including nerve biopsy, EGPA was diagnosed for him.

Henoch-Schonlein Purpura in a Newly Diagnosed HIV Patient.

HIV infection is associated with multisystemic manifestations due both to secondary infections caused by a decrease in the CD4+ T-cell count and to the pathogenicity of the HIV virus itself. A common renal manifestation is HIV-associated nephropathy, which is frequently seen in the African population with the APOL1 gene mutation; however, other forms of glomerulopathy such as IgA nephropathy, commonly noted in other ethnicities, are also seen. Vasculitis has rarely been associated with HIV infection and mainly involves small blood vessels, although any size of blood vessel may be involved. The association of Henoch-Schonlein purpura (HSP) with HIV is rare and not well understood. We describe a 53-year-old African American woman with a newly diagnosed HIV infection who presented with a purpuric rash over the bilateral lower extremities with haematuria. Initial work-up revealed renal dysfunction with elevated ESR. Urinalysis was positive for glomerular haematuria and sub-nephrotic range proteinuria. Serum complement level, c-antineutrophil cytoplasmic antibody (ANCA), p-ANCA and anti-nuclear antibody (ANA) were negative. Renal biopsy revealed mesangial IgA deposits with crescent glomerulopathy and fibrinoid necrosis, while skin biopsy revealed leucocytoclastic vasculitis. A diagnosis of HSP was made based on American College of Rheumatology (ACR) criteria. The patient's renal function and purpura improved with a 5-day course of steroid pulse therapy. This case of HSP in a newly diagnosed HIV patient is unusual for the presence of crescentic glomerulopathy. LEARNING POINTS: Henoch-Schonlein purpura (HSP) associated with HIV infection is uncommon but documented; however, all four features of HSP are rarely seen together.Crescent glomerulopathy is rarely seen in HIV-associated HSP.HSP associated with HIV is treated with antiretroviral drugs, while the role of steroid and immunosuppressive therapy remains controversial.

Bilateral breast necrotizing leukocytoclastic vasculitis: First case report.

Leukocytoclastic vasculitis (LCV) is a very rare immune complex-mediated condition affecting the small vessels walls. We present the case of a 48-year-old woman with necrotizing bilateral breast LCV on treatment with glatiramer acetate for multiple sclerosis. Bilateral mastectomies and debridement of the anterior abdominal wall were required due to the rapidly evolving necrotizing process. Rapid assessment and a multidisciplinary approach are fundamental in treating this rare life-threatening condition.

Acute brucellosis associated with isolated splenic and left gastric artery vasculitis and acute ischemic bowel infarction. A systematic review of the most recent cases.

Brucellosis is a multisystem bacterial zoonosis caused by Gram-negative bacteria Brucella spp. Ingestion of infected food products, direct contact with an infected animal, or inhalation of aerosols are all ways for germs to spread from animals to humans. Intestinal vasculitis with gangrene due to brucellosis has rarely been reported. We report a 62-year-old male patient presenting with acute onset of recurrent attacks of abdominal pain, remittent fever, malaise, and weight loss, which were followed by severe left hypochondrium abdominal pain with rigidity and signs of acute abdomen. Brucellosis was clinically suspected and confirmed by an enzyme-linked immunosorbent assay against the Brucella melitensis species. An abdominal CT scan revealed isolated splenic and left gastric artery vasculitis, leading to acute bowel ischemia, bowel infarction and gangrenous jejunal bowel segment. Histopathological examination of the resected gangrenous bowel segment revealed leucocytoclastic vasculitis. The patient was successfully treated with a standardized antimicrobial therapy for brucellosis and a short course of steroids with a complete resolution of the symptoms and signs. The case is discussed and the literature is reviewed.

Management of cutaneous vasculitis.

Cutaneous vasculitis encompasses cutaneous components of systemic vasculitides, skin-limited variants of systemic vasculitides, such as IgA vasculitis or cutaneous polyarteritis nodosa, and single-organ cutaneous vasculitis, as individualized in 2012 in the Chapel Hill Consensus Conference Nomenclature. In this article, we focus on the management of skin-limited and single-organ vasculitides, often referred to, in clinical practice, as isolated "cutaneous leukocyctoclastic vasculitis", terms which may correspond to histological findings or descriptions, but are imprecise and not specific. Since most cases of isolated cutaneous vasculitis are self-limited and resolve spontaneously over 3 to 4 weeks, most patients require no systemic treatment. For those with severe, intractable, or chronic and recurring vasculitis, systemic therapy can be indicated and should be tailored to the severity of the disease. High-quality literature is lacking to guide management. Oral glucocorticoids may be required for a short period of time for painful, ulcerative, or otherwise severe disease in order to speed resolution. Among drugs which are reasonable longer-term options are colchicine, dapsone, azathioprine or hydroxychloroquine. Additional studies, including an ongoing multicenter randomized trial, are needed to determine the most effective therapies for skin-limited vasculitis.

IgA nephropathy with leucocytoclastic vasculitis.

Leucocytoclastic vasculitis is a rare type of allergic disease caused by immune complexes. IgA nephropathy is a glomerulopathy characterized by recurrent episodes of gross haematuria or microscopic haematuria and IgA deposition in the glomerular mesangial region. IgA nephropathy complicating leucocytoclastic vasculitis is rare documented. We present a case of IgA nephropathy in a 47-year-old woman with leucocytoclastic vasculitis and discuss the clinical and pathological data, aiming to promote the diagnosis and treatment of this specific clinical manifestation.

Vasculitis as a Presenting Manifestation of Chronic Hepatitis B Virus Infection: A Case Report.

Hepatitis B virus is responsible for causing hepatic complications like acute and chronic hepatitis, cirrhosis and hepatocellular carcinoma along with some uncommon immune mediated extrahepatic manifestations. Vasculitis remains an uncommon extrahepatic complication of hepatitis B virus infection. Herein we report a case of hepatitis B infection that presented with leucocytoclastic vasculitis as an initial manifestation and managed successfully with entacavir therapy.

Acute Hemorrhagic Edema of Infancy.

Acute Hemorrhagic Edema of Infancy is a small vessel leucocytoclastic vasculitis affecting young infants. It is characterized by large, target-like, macular to purpuric plaques predominantly affecting the face, ear lobes and extremities. Non-pitting edema of the distal extremities and low-grade fever may also be present. Extra-cutaneous involvement is very rare. Although the lesions have a dramatic onset in a twenty-four to forty-eight hour period, usually the child has a non-toxic appearance. In most cases there are no changes in laboratory parameters. The cutaneous biopsy reveals an inflammatory perivascular infiltrate. It is a benign and auto-limited disease, with complete resolution within two to three weeks leaving no sequelae in the majority of cases. No recurrences are described. We report a case of a 42-day old girl admitted at our hospital with Acute Hemorrhagic Edema of Infancy.

Isoniazid Induced Cutaneous Leukocytoclastic Vasculitis in Extra Pulmonary Tuberculosis (Pott's Spine): A Case Report.

Anti-tuberculosis drugs frequently result in cutaneous adverse reactions but Isoniazid is known to have least toxic potential for cutaneous reactions. We report a rare case of Isoniazid induced cutaneous leucocytoclastic vasculitis. A 64-year-old male was diagnosed to have Pott's spine with multiple vertebral body involvement (D8-12 vertebrae). Subsequently, he was treated with first line anti-TB drugs i.e., Isoniazid, Rifampicin, Pyrazinamide and Ethambutol. On the fourth day of treatment with Anti Tuberculosis Treatment (ATT), the patient developed an erythematosus rash over right upper limb not associated with itching or pain, non-blanchable macules and papules over bilateral shins on lower limbs, petechiae on both forearms and hyper pigmented, scaly rash over right axilla and buttocks. The skin biopsy report was consistent with cutaneous leukocytoclastic vasculitis. Although rare, Isoniazid among anti-tuberculosis drugs should be considered as potential cause of drug-induced cutaneous leukocytoclastic vasculitis in the differential diagnosis of erythematosus rash with petechiae.

Urticarial vasculitis and Schnitzler syndrome.

Both urticarial vasculitis and Schnitzler syndrome are serious diseases but a recently gained better understanding of their underlying pathomechanism allows better treatment options than only a decade ago.

Síndrome de Sweet / Sweet syndrome

A síndrome de Sweet, também conhecida como dermatose neutrofílica febril aguda, caracteriza-se pelo aparecimento de pápulas e/ou placas eritematosas dolorosas, muitas vezes em surtos que duram cerca de duas semanas ou mais. Foram revisados características clínicas, achados laboratoriais, doenças associadas, histopatologia e opções de tratamento da síndrome, assim como sua manifestação em crianças.