Cutaneous Vasculitis Research Articles

Клинический случай идиопатической формы синдрома Свита

Background. Sweet’s syndrome (acute febrile neutrophilic dermatosis) is an extremely rare disease of the group of neutrophilic dermatoses with undulating courses of illness, which is an inflammatory non-infectious skin reaction characterized histopathologically by the presence of dense diffuse neutrophil infiltrate in the dermis without signs of leukocytoclastic vasculitis, edema and fragmentation of neutrophil nuclei. The mechanism of development of this disease is presumably associated with a cytokine-mediated hypersensitivity reaction followed by neutrophil infiltration of the dermis, however, the etiology of the development of this pathogenesis is not known. Sweet syndrome is a current problem, since complications can occur in the form of damage to the organs of the central nervous system, eyes, lungs, skin breakdown, as well as changes in the immune system in the form of immunodeficiency. Aim. To evaluate, describe, study the courses and complications of the idiopathic form of Sweet’s syndrome. Materials and methods. Study of examination data of a (3-year-old girld) who was being treated at the department of pulmonology of Kursk Regional Children's Clinical Hospital. Study and analysis of scientific literature on the problem of Sweet's syndrome. Conclusion. As a result of studying this Clinical case, we came to the conclusion that despite the fact that Sweet’s syndrome is an extremely rare disease and more often debuts at the age of 60, it can also be observed in children, which must be taken into account in the presence of a skin rash without a known etiology and pathogenesis. Since complications may occur, it is necessary to identify and treat exacerbations of this disease in a timely manner. The prognosis of the disease is relatively favorable, since complications of Sweet’s syndrome can lead to a decrease in the quality of life of patients.

A case of eosinophilic granulomatosis with polyangiitis associated with diffuse alveolar haemorrhage: A case report and case-based review.

A 76-year-old man with bronchial asthma was admitted for respiratory failure and bloody sputum. A significant drop in haemoglobin and multiple consolidations supported clinical diagnosis of diffuse alveolar haemorrhage (AH). Myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA) was positive and urinalysis suggested glomerulonephritis. Based on eosinophilia, sinusitis, peripheral nerve involvement, and leukocytoclastic vasculitis, he was diagnosed with eosinophilic granulomatosis with polyangiitis (EGPA) associated with AH. Our case-based review suggested that male predominance (65%), high positivity for ANCA (88%), and a high frequency of renal involvement (45%) may be characteristic of AH in EGPA. Although AH is rare in EGPA, we should be aware of this life-threatening complication.

E024 A pregnant woman with vasculitis and a new rash

Abstract Background/Aims A 41-year-old pregnant woman at 16 weeks of gestation was followed-up in the combined obstetric and rheumatology clinic as she had a history of eosinophilic granulomatosis with polyangiitis (EGPA) and two previous miscarriages at first trimester. Her vasculitis was well-controlled with a baseline daily dose of prednisolone 7.5mg. Methods During her pregnancy, she started on vaginal progesterone by the IVF (In Vitro Fertilization) team for pregnancy support following a vaginal bleed. During her second review in clinic, she complained about a widespread pruritic rash for the last 3 weeks. She had already been seen by her GP who prescribed her emollients without any improvement. On examination, the active lesions looked urticarial and were tender. There was also residual hyperpigmentation. The lesions persisted for more than 24 hours before spontaneous resolution. Cutaneous vasculitis was suspected based on the duration of the rash more than 24 hours, the hyperpigmentation, tenderness, and the history of EGPA. Otherwise, she was well in herself with no symptoms of active vasculitis and no recent infections. Therefore, prednisolone was increased to 15mg daily and loratadine was given for the pruritus. Her repeat blood tests were reassuring with negative ANCA, normal C3 and C4, CRP, and eGFR. Results During her early follow-up, the rash was the same despite the higher dose of steroids and she volunteered that the rash is worse in the morning and at night. Autoimmune progesterone dermatitis (AIPD) was considered then. AIPD is a rare autoimmune hypersensitivity reaction to endogenous or exogenous progesterone. AIPD varies greatly from patient to patient and can present as urticaria, eczema or vesiculobullous eruptions. Urticarial or EGPA vasculitis, and urticaria would have improved with steroids. Furthermore, she did not have any symptoms suggestive of systemic vasculitis and the relevant blood tests were also normal. Of note, the rash was worse in the morning and at night, which corresponded to the time of vaginal exposure to progesterone. The patient stopped the progesterone pessaries after discussion with the obstetrician. The rash improved gradually and resolved in five days, confirming the diagnosis of AIPD. Conclusion The rash could have been mistaken for urticaria or vasculitis. Consequently, the patient may have had unnecessarily antihistamines and steroids during pregnancy with all relevant side effects, such as gestational diabetes. In November 2021, NICE recommended vaginal progesterone in women who experience bleeding in early pregnancy and have a history of miscarriage as it slightly reduces the risk of a further miscarriage. The new guidelines suggest that more pregnant women will use topical progesterone during pregnancy who may develop AIPD. This means that we are going to see more pregnant women in the rheumatology clinic with AIPD and the rheumatology community should be aware of this entity. Disclosure A. Madenidou: Grants/research support; Janssen. H. Kither: None. I. Bruce: Consultancies; AstraZeneca, GSK, Eli Lilly, and UCB. Honoraria; AstraZeneca, GSK, and UCB. Grants/research support; AstraZeneca, GSK, and Janssen.

P015 Nodular skin rash associated with relapsing polychondritis, a challenging diagnostic journey to VEXAS syndrome

Abstract Background/Aims VEXAS syndrome, first described in 2020, is a haemato-inflammatory disease caused by a mutation in the gene-UBA1.This syndrome can coexist with or without Myelodysplastic syndrome. What makes this case interesting is its long and challenging diagnostic journey and the involvement of different clinical specialties such as dermatology, ophthalmology, rheumatology, respiratory, haematology, and stroke teams. Ultimately, two years after the initial presentation, this appeared to be the first case of VEXAS with confirmed genetic mutation in the region. VEXAS being a relatively new entity, treating clinician/s were not aware of the possibility initially. Therefore, spreading awareness regarding the clinical picture of VEXAS is prudent. This case report highlights rheumatological and dermatological manifestations of the disease to aid in the timely diagnosis and management of future cases. Methods We report a 74-year-old previously healthy male presented in 2021 with a rash on his legs, arms, and torso since October 2019. It was progressive, painful, palpable, erythematous, non-pruritic, intermittent rash, cleared with hyperpigmentation. He also had oligoarticular arthritis, constitutional symptoms, recurrent episodes of polychondritis, uveitis, bibasal lung fibrosis, and an ESR and CRP above 100. He also had lymphadenopathy above the diaphragm, but the biopsy only showed necrotic material, and the bone marrow biopsy did not show any features of haematological malignancy. After excluding infections, he was started on high-dose oral prednisolone for relapsing polychondritis. The skin and inflammatory markers improved immediately following high doses of steroids. However, whenever steroids were weaned down, even with the help of Methotrexate, his skin was flaring. He also had unprovoked DVT and, while on anticoagulation, developed occipital infarction. He had extensive skin involvement when he had to stop Methotrexate following a chest infection. Results The skin biopsy was inconclusive. There was no significant cytopenia but persistent macrocytosis even after stopping Methotrexate. As a result, he underwent a repeat bone marrow biopsy, which showed Vacuoles and gene UBA1 mutation in 2023. Conclusion The heterogeneity and complexity of the disease are due to multi-organ involvement, including skin, joints, eyes, lungs, blood vessels, and cartilage, simultaneously or cumulatively. Skin involvement in VEXAS can also be vast, ranging from neutrophilic dermatosis, erythema nodosum-like lesions, leukocytoclastic, or urticarial vasculitis. Cutaneous manifestations develop in the majority with VEXAS syndrome and can be the first presenting symptom in this new rare syndrome. Disclosure M. Gunawardena: None. L. Yalakki: None. R. Moitra: None. E.L. Williams: None. H. Wong: None. D. D'Cruz: None.

P031 An case of cryofibrinogen associated necrotising cutaneous vasculitis on background of aortitis

Abstract Background/Aims Cryofibrinogen is an abnormal protein that forms precipitate in plasma but not in serum. Cryofibrinogenaemia can be detected in healthy individuals and patients with autoimmune diseases, cancer, and infections. Current data suggest that isolated cryofibrinogenaemia can be detected in up to 3% of healthy individuals and it is usually asymptomatic. However, cryofibrinogenaemia can be associated with systemic inflammatory manifestations, of which cutaneous involvement is the most prominent. Methods A 76-year-old male patient presented with acute and progressive necrotising ulcers in the lower limbs along with high inflammatory markers and general decline. He had a background of large vessel vasculitis (aortitis), which was diagnosed with a PET-CT scan three years prior to the current presentation. He was previously treated with oral steroids and eight cycles of cyclophosphamide demonstrating an excellent response with complete resolution of the aortitis on the follow-up PET-CT scan. The patient had remained on maintenance treatment with azathioprine (3mg/kg) as well as therapeutic anticoagulation for atrial fibirillation. Results After thorough investigations for an infective cause and poor response to broad spectrum antibiotics, he was reviewed further for recurrence of large vessel vasculitis. A CT angiogram showed concentric symmetrical soft tissue thickening surrounding the thighs and lower limb arteries without luminal stenosis, suggestive of periarterial inflammation. A further MR angiogram of the aorta did not confirm any evidence of aortic, iliac or visceral artery stenosis. A CT chest/abdomen/pelvis excluded any other pathology or malignancy. Additional investigations showed negative ANA and ENA screen, rheumatoid factor, antiphospholipid antibodies, cryoglobulins, hepatitis virus screen, whilst complement and immunoglobulin levels were normal. However, cryofibrinogen was reported positive and a diagnosis of cryofibrinogen-associated necrotising cutaneous vasculitis was concluded. The patent was started on IV cyclophosphamide (15mg/kg) along with IV methylprednisolone (250mg for the first 3 doses) along with each pulse therapy. After the first two pulses, the patient had a favourable clinical and serological response to the immunosuppressive treatment with improvement in cutaneous lesions and significant reduction in the inflammatory markers. He subsequently underwent an angioplasty for tightly stenotic/occluded tibioperoneal trunk with good recanalisation. Conclusion This is a rare overlap syndrome of new-onset small vessel necrotising cutaneous vasculitis on previous background of large vessel disease (aortitis). In most severe cases with essential cryofibrinogenaemia, the mainstay treatment is with corticosteroids and other immunosuppressive agents in combination with oral anticoagulants and/or low-dose aspirin. Post treatment, the risk of relapse remains high and immunosuppressive therapy may only have a limited impact on the course of thrombotic vasculopathy in the absence of inflammation. More research and longitudinal data are required to optimise treatment protocols and outcomes in patients with cryofibrinogen-associated systemic vasculitis. Disclosure A. Psarras: None. J. David: None. R. Luqmani: None. S. Dubey: Consultancies; Boehringer Ingelheim. Member of speakers’ bureau; Janssen.

P158 Cutaneous vasculitis in systemic lupus erythematosus: trends of incidence and risk factors

Abstract Background/Aims Cutaneous vasculitis (CV) is the most common form of vasculitis observed in patients with SLE; however, the precise epidemiological trends and underlying risk factors remain uncertain. The aim of this study was to define the incidence, prevalence, and risk factors of CV in systemic lupus erythematosus (SLE) patients over a period of 20 years. Methods The Birmingham Lupus Cohort is a longitudinal observational cohort of patients with SLE. All patients fulfilled the 1997 ACR Updated Classification Criteria for SLE. Inception patients were recruited within three years of their fourth ACR criterion. Disease activity was assessed using the classic BILAG index and laboratory results recorded. The BILAG vascular domain was used to determine the incidence rate, which refers to the rate at which new cases of minor and major CV attributed to active SLE occur. Cox proportional hazards frailty models were developed to identify clinical and laboratory variables associated with development of CV. Results We analysed 392 patients, of whom 375 (92.5%) were females. There were 213 (55.0%) White, 72 (18.6%) African-Caribbean, 82 (21.1%) South Asian, 9 (2.3%) East Asian, and 11 (2.8%) other ethnicities. The median (IQR) age at enrolment was 33 (26 - 44) years and the median duration of follow up was 9.2 (5.1 - 14.7) years. The incidence rates of minor and major CV were reported in Table 1. In Cox proportional hazards models, there was a positive association between CV and discoid rash (hazard ratio [HR] 2.16 [95% CI: 1.01, 4.61]), Raynaud’s phenomenon (HR 5.67 [95% CI: 4.11, 7.81]), livedo reticularis (HR = 4.69 [95% CI: 1.83, 12.0]), arthritis (HR 3.02 [95% CI: 2.20, 4.15]), renal involvement (OR 2.40 [95% CI: 1.55, 3.71]), haematological involvement (HR = 2.46 [95% CI: 1.80, 3.36]), and Afro-Caribbean descent (HR 1.96 [95% CI: 1.03, 3.74]). In addition, CV was positively associated with anti-dsDNA (HR = 2.33 [95% CI: 1.67, 3.27]), low C3 or C4 (HR 2.29 [95% CI: 2.18, 4.10]), and history of anti-Ro or anti-La (HR = 1.92 [95% CI: 1.15, 3.21]). Conclusion The incidence rates of CV decrease over the duration of follow-up and are associated with defined clinical and serological features. Disclosure A. Saleh: Grants/research support; A.S. is a sponsored PhD student by the Egyptian ministry of higher education represented by The Egyptian Bureau for Cultural & Educational Affairs in London. C. Gordon: None. J. Reynolds: None.

Leg Ulcers in a Patient With Systemic Lupus Erythematosus and Successful Treatment With Anticoagulant Therapy.

Among specific skin manifestations of systemic lupus erythematosus such as leukocytoclastic vasculitis, and vasculopathy, the development of leg ulcers is rare and frequently seen in patients with antiphospholipid antibody positivity. Here we report the rapid healing of a leg ulcer without antiphospholipid antibody positivity in a patient with lupus in response to anticoagulant therapy. As in our case, when immunosuppressive agents are inadequate in lupus patients who develop leg ulcers, it may be beneficial to support the treatment with anticoagulants.

AYURVEDIC MULTIMODAL INTERVENTION IN LEUCOCYTOCLASTIC VASCULITIS - A CASE REPORT

Leucocytoclastic vasculitis is a complex immune-mediated vasculitis of the dermal capillaries and venules. The annual incidence of biopsy-proven leucocytoclastic vasculitis is approximately 45 per million individuals. An agent used to treat vasculitis include corticosteroids and immunosuppressive drugs. There is no cure for leucocytoclastic vasculitis. A 61-year-old male patient with leucocytoclastic vasculitis was hospitalised and convinced of Panchakarma treatment. Based on the Ayurvedic concept, the diagnosis was considered Vatarakta with Raktavritta vata. Raktamokshana, Virechana and Basti were advised along with Shaman and Rasayana medications. The result was assessed using a numerical pain assessment scale to subjective and objective parameters. In the present case study, the diagnosis was established based on skin biopsy and clinical features, then treated successfully through multimodal Ayurvedic intervention.

Annular Leukocytoclastic Vasculitis: A New Feature of IgA Vasculitis.

Annular erythema is a rare manifestation of leukocytoclastic vasculitis. It may be associated with various drugs, infections, malignancies, or systemic diseases. A 36-year-old woman with no personal medical history presented with annular erythema with target lesions and petechial purpura. The patient had fever and joint arthralgia. A skin biopsy showed leukocytoclastic vasculitis with IgA deposits on direct immunofluorescence. The diagnosis of immunoglobulin A vasculitis with annular leukocytoclastic vasculitis was made. The patient showed global improvement with topical steroids without relapse. An annular variant of leukocytoclastic vasculitis is a rare manifestation of immunoglobulin A vasculitis. Annular erythema may reveal an IgA vasculitis.Annular leukocytoclastic vasculitis is a rare manifestation of IgA vasculitis.This presentation is treated with topical steroids.

Evaluation of the histopathological spectrum of skin biopsies in patients with chronic kidney disease: A single-center experience.

Patients with chronic kidney disease (CKD), particularly those with end-stage renal disease (ESRD), frequently experience skin problems. Here, we present and discuss the histopathology results of skin biopsies performed on renal patients at our facility. We retrospectively reviewed all histopathological reports and slides of skin biopsies taken from CKD patients for the last 5 years at the Pathology Department of King Saud University Medical City, King Saud University in Riyadh. This is a retrospective cross-sectional study. Patients were 19 (43.2%) males and 25 (56.8%) females, with a mean age of 53.4 19.8 years. Diabetic nephropathy, hypertensive nephropathy, and lupus nephritis were the three most common kidney pathologies (59.1%, 11.4%, and 11.4%, respectively). Seventeen patients (38.5%) were receiving hemodialysis. Acquired perforating collagenosis (APC), which was observed in 16 (36.4%) of our patients, was the most prevalent skin pathology, followed by bullous drug reactions in nine (20.5%), leukocytoclastic vasculitis (LCV) in eight (18.2%), calciphylaxis in six (13.6%), and prurigo nodularis in five (11.4%) patients. Erythematous plaques and papules were the most prevalent skin conditions at the time of presentation in 12 patients (27.3%), followed by pruritus in six (13.6%) and ulcerations in six (13.6%) patients. Collected data were analyzed using the Statistical Package for Social Sciences (SPSS) version 26.0. A spectrum of skin conditions may be seen in CKD patients. The quality of life of CKD patients will be significantly improved by identifying and managing these conditions. Pathologists' familiarity with this spectrum is important, as a correct histopathologic diagnosis will lead to a better outcome.

Cutaneous vasculitis: insights into pathogenesis and histopathological features.

Cutaneous vasculitis: insights into pathogenesis and histopathological features.

155 There is more to see with Hepatitis C viremic kidney donation: A case of leukocytoclastic vasculitis in aviremic recipient

155 There is more to see with Hepatitis C viremic kidney donation: A case of leukocytoclastic vasculitis in aviremic recipient

Cutaneous Leukocytoclastic Vasculitis Induced by the Iodinated Contrast Media Iohexol With Cross-Reactivity to Iodixanol

Cutaneous Leukocytoclastic Vasculitis Induced by the Iodinated Contrast Media Iohexol With Cross-Reactivity to Iodixanol

Is chronic urticaria or urticarial vasculitis a diagnostic dilemma?

Introduction. Urticarial vasculitis (UV) is a rare disease that has two components: clinical manifestations of urticaria and histopathological signs of cutaneous leukocytoclastic vasculitis of small vessels, predominantly involving postcapillary venules. This condition is characterized by chronic or recurrent episodes of urticaria, each element of which lasts more than 24 hours and is accompanied by a feeling of pain and burning. The aim is to reveal the key points of pathogenetic mechanisms, differential diagnosis and therapeutic tactics of UV based on a clinical case. Clinical case. A clinical case of a 17-year-old boy with normocomplementemic UV is described. The patient's main complaint was a long-lasting rash (more than three weeks) with itching. From the anamnesis it is known that the provoking factors for the onset of the disease were an insect bite and the start of taking a new drug, namely vitamin K (two days before the onset of the disease). Throughout this time, the child was examined by various specialists and received treatment. Alternative diagnoses: bacterial folliculitis, viral exanthem, unspecified urticaria. There was no positive effect from the received treatment. The diagnosis of UV was made in the sixth week of the disease using a punch biopsy. Regression of the skin syndrome was achieved using a combination of antihistamine and antileukotriene drugs. Conclusions. Performing a punch biopsy, which is currently the gold standard for diagnosis, allows us to solve the diagnostic dilemma: “UV or chronic urticaria”. Timely diagnosis helps to avoid false diagnoses and, as a result, incorrect treatment of UV. The description of this clinical case is a contribution to the disclosure of this globally complex problem. The research was carried out in accordance with the principles of the Declaration of Helsinki. The informed consent of the child and child's parents was obtained for conducting the research. No conflict of interest was declared by the authors.

Ustekinumab-induced leukocytoclastic vasculitis in a young male with Crohn's disease

Ustekinumab-induced leukocytoclastic vasculitis in a young male with Crohn's disease

Japanese Spotted Fever with Systemic Leukocytoclastic Vasculitis.

Japanese Spotted Fever with Systemic Leukocytoclastic Vasculitis.

Comparison of clinical and laboratory data of adult patients with cutaneous IgA vasculitis and non-IgA vasculitis.

Immune complex vasculitides may be subdivided into adult IgA small vessel vasculitis (aIgA-SVV; i.e. adult Henoch-Schönlein purpura) and non-IgA-SVV (hypersensitivity vasculitis, etc.). To evaluate the clinical and laboratory parameters of inpatients fulfilling the diagnostic criteria for aIgA-SVV and non-IgA-SVV. Twenty-nine adults aged ≥ 20 years with aIgA-SVV [according to the European League Against Rheumatism/Paediatric Rheumatology International Trials Organisation/Paediatric Rheumatology European Society (EULAR/PRINTO/PRES) criteria] and 53 adults with non-IgA-SVV (according to the 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides) were compared with respect to a variety of clinical and laboratory parameters by uni- and multivariable analyses. Compared with patients with aIgA-SVV, the platelet-to-lymphocyte ratio was significantly higher in patients with non-IgA-SVV. Serum C3 levels and mean corpuscular haemoglobin concentration in patients with non-IgA-SVV were significantly lower compared with patients with aIgA-SVV. Proteinuria and haematuria were significantly more common in patients with aIgA SVV, and were significantly correlated with systemic immune-inflammation biomarkers only in patients with aIgA-SVV. In patients with aIgA-SVV, higher lactate dehydrogenase and C-reactive protein were strong independent predictors for the presence of proteinuria and proteinuria. In patients with non-IgA-SVV, female sex was a protective factor for proteinuria, while skin lesions on the upper extremities proved to be a significant independent predictor of haematuria. We detected several clinical and laboratory differences between patients with aIgA-SVV and non-IgA-SVV. Distinct predictors for renal involvement were not observed in either group, indicating that aIgA-SVV and non-IgA-SVV are similar conditions but do not appear to represent the same entity.

Leukocytoclastic Vasculitis Localized to the Uterine Cervix

Leukocytoclastic Vasculitis Localized to the Uterine Cervix

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.