Increase in the incidence of invasive fungal infections due to Volvariella Volvacea.

PURPOSE: We aimed to show the increasing incidence of invasive fungal infections due to Volvariella Volvacea in patients with immunosuppression. METHODS: We present a case of an invasive fungal infection caused by Volvariella volvacea, and summarize the clinical and pathological features based on this case and a review of the literature. RESULTS: A total of seven patients with IFIs due to Volvariella Volvacea have been reported in the literature. The majority of cases have been obtained between 2019 and 2022. Including our case, they all had acquired immunosuppression. The lung and brain were the most commonly affected organs. All eight of these patients received antifungal therapy, but five still died one to seven months after occurrences of IFIs. CONCLUSION: The incidence of invasive fungal infections due to Volvariella Volvacea is increasing in recent years. It mainly occurred in patients with immunosuppression, especially in patients with malignant hematological cancers, and increased mortality.

Diagnostic utility of direct immunofluorescence test panels for cutaneous vasculitis: A scoping review.

BACKGROUND: Due to the immune-mediated nature of non-infectious cutaneous vasculitis, skin biopsy specimens are often submitted for direct immunofluorescence (DIF) testing when vasculitis is considered clinically. However, evidence regarding the clinical value of DIF has not been rigorously appraised. OBJECTIVE: In this scoping review, we aimed to systematically evaluate the peer-reviewed literature on the utility of DIF in vasculitis to assist with the development of appropriate use criteria by the American Society of Dermatopathology. METHODS: Two electronic databases were searched for articles on DIF and vasculitis (January 1975-October 2023). Relevant case series involving more than or equal to three patients, published in English, and with full-text availability were included. Additional articles were identified manually via reference review. Due to study heterogeneity, findings were analyzed descriptively. RESULTS: Of 255 articles identified, 61 met the inclusion criteria. Cumulatively representing over 1000 DIF specimens, several studies estimated DIF sensitivity to be 75%. While vascular immunoglobulin A (IgA) deposits on DIF were associated with renal disease, other systemic associations were inconsistent. Vascular IgG deposition may be overrepresented in ANCA-associated vasculitis. Granular vascular and epidermal basement membrane zone Ig deposition differentiated hypocomplementemic from normocomplementemic urticarial vasculitis. Few studies have assessed the added value of DIF over routine microscopy alone in vasculitis. CONCLUSIONS: This scoping review discovered that DIF testing for vasculitis has been performed not only for diagnostic confirmation of vasculitis but also for disease subtype classification and prediction of systemic associations. Future studies on test sensitivity of DIF compared to that of histopathology are needed.

Onset of leukocytoclastic vasculitis following covid-19 vaccination: case based comprehensive review.

With the global introduction and widespread administration of COVID-19 vaccines, there have been emerging reports of associated vasculitis, including leukocytoclastic cutaneous vasculitis (LCV). In this paper, we present a case of a 68-year-old female patient who developed painful purpuric skin lesions on her feet 12 days after administration of the inactivated COVID-19 vaccine BBIBP Cor-V with histopathological confirmation of LCV and no signs of systemic involvement. The case is followed by a comprehensive literature review of documented LCV cases associated with COVID-19 vaccination with overall 39 articles and 48 cases of LCV found in total. In the majority of cases (56.3%) the first symptom occurred after the first dose of the COVID-19 vaccine, with symptoms manifesting within an average of seven days (6.8 ± 4.8) post-vaccination. The adenoviral vaccine Oxford-AstraZeneca (41.7%) and the mRNA vaccine Pfizer-BioNTech (27.1%) were most frequently associated with LCV occurrences. On average, LCV resolved within 2.5 (± 1.5) weeks. The preferred treatment modality were glucocorticoids, used in 70.8% of cases, resulting in a positive outcome in most cases, including our patient. While the safety of a subsequent dose appears favorable based on our review, individual risk-benefit assessment is crucial. This review emphasis the importance of considering COVID-19 vaccination as a potential trigger for the development of cutaneous vasculitis. Despite rare adverse events, the benefits of the COVID-19 vaccination outweigh the risks, highlighting the importance of immunization programs.

Cutaneous vasculitis: insights into pathogenesis and histopathological features.

The mechanisms underlying the onset and progression of vasculitis remain poorly understood. This condition is characterized by damage to the vascular wall, recruitment of inflammatory cells, and subsequent structural remodeling, which are hallmarks of vasculitis. The histopathological classification of vasculitis relies on the size of the affected vessel and the predominant type of inflammatory cell involved - neutrophils in acute cases, lymphocytes in chronic conditions, and histiocytes in granulomatous forms. Pathological changes progress in every context, and a single vasculitic pattern can be associated with various systemic conditions. Conversely, a single causative agent may lead to multiple distinct clinical and pathological manifestations of vasculitis. Moreover, many cases of vasculitis have no identifiable cause. A foundational understanding of the normal structure of the cutaneous vascular network is crucial. Similarly, identifying the cellular and molecular participants and their roles in forming the "dermal microvascular unit" is propedeutical.This review aims to elucidate the complex mechanisms involved in the initiation and progression of vasculitis, offering a comprehensive overview of its histopathological classification, underlying causes, and the significant role of the cutaneous vascular network and cellular dynamics. By integrating the latest insights from studies on NETosis and the implications of lymphocytic infiltration in autoimmune diseases, we seek to bridge gaps in current knowledge and highlight areas for future research. Our discussion extends to the clinical implications of vasculitis, emphasizing the importance of identifying etiological agents and understanding the diverse histopathological manifestations to improve diagnostic accuracy and treatment outcomes.

VEXAS syndrome (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) for the dermatologist.

In 2020, Beck et al1 described a novel adult autoinflammatory syndrome entitled VEXAS (Vacuoles, E1 enzyme, X-linked, Autoinflammatory, Somatic), a newly-discovered disorder that connected previously unrelated inflammatory syndromes and a prototype for a new class of hematoinflammatory diseases.2 Eighty-nine percent of published cases have documented skin involvement, but despite the high incidence and diagnostic accessibility of skin manifestations, there has been little focus on the dermatological features of VEXAS syndrome thus far. A PubMed search of all published case reports of VEXAS syndrome to date was performed, with inclusion of all cases confirmed by genetic sequencing, and this review summarizes the reported dermatological signs. There have already been 141 confirmed published cases since original publication, 126 of which had documented cutaneous signs.1-34 A wide range of skin presentations are reported, including Sweet-like urticated and tender erythematous nodules, cartilaginous involvement with chondritis, cutaneous vasculitis, and periorbital angiodema.1-34 Many patients had been diagnosed with Sweet syndrome, relapsing polychondritis, polyarteritis nodosa, or erythema nodosum.1-34 Hallmarks of skin histopathology are a neutrophilic dermatosis with coexisting or exclusive leukocytoclastic vasculitis.1 The new classification therefore helps link previously disparate inflammatory skin conditions into a unifying pathophysiological pathway.

Accurate diagnosis of acute hemorrhagic edema of infancy: a French multicenter observational study.

The purpose of the study is to highlight clinical signs that are either suggestive of or against the diagnosis of AHEI to improve diagnosis and management. The medical records of children under 3 years old diagnosed with AHEI were retrospectively reviewed. Clinical data and photographs were reviewed by three independent experts, and the cases were classified as probable, doubtful, or unclear AHEI. Of the 69 cases of children diagnosed with AHEI included in 22 centers, 40 were classified as probable, 22 as doubtful, and 7 as unclear. The median age of patients with probable AHEI was 11 months [IQR 9-15], and they were in overall good condition (n = 33/40, 82.5%). The morphology of the purpura was targetoid in 75% of cases (n = 30/40) and ecchymotic in 70% of cases (n = 28/40) and affected mostly the legs (n = 39/40, 97%), the arms (n = 34/40, 85%), and the face (n = 33/40, 82.5%). Edema was observed in 95% of cases and affected mostly the hands (n = 36/38, 95%) and feet (n = 28/38, 74%). Pruritus was absent in all patients with probable AHEI and described for 6/21 with doubtful AHEI (29%). AHEI was the original diagnosis in only 24 patients (n = 24/40, 60%). The major differential diagnoses were purpura fulminans and urticaria multiforme.  Conclusion: AHEI, which the diagnosis is made on clinical findings, is often misdiagnosed. Purpuric lesions localized on the face/ears, arms/forearms, and thighs/legs with edema of the hands without pruritus in a young child with a good overall condition are highly suggestive of AHEI. What is Known: •Acute hemorrhagic edema of infancy (AHEI) is a cutaneous leukocytoclastic vasculitis affecting children under 3 years old. •Appropriate diagnosis is important to distinguish this benign disease from more serious diseases to avoid investigations and treatments, iatrogenic harm and unnecessary follow-up. What is New: •AHEI is an uncommon disorder often misdiagnosed by pediatricians and dermatologists. •Purpuric lesions localized on the face/ears, arms/forearms, and thighs/legs with edema of the hands without pruritus in an infant with a good overall condition are highly suggestive of AHEI.

Leukocytoclastic vasculitis associated with capecitabine.

BACKGROUND: Leukocytoclastic vasculitis (LCV) is a vasculitic inflammation against blood vessels. Various anticancer therapies can cause vasculitis, but capecitabine-induced LCV is an unusual entity. Here, we describe an LCV case associated with neoadjuvant capecitabine use for locally advanced rectal cancer (LARC). CASE REPORT: A 70-year-old man presented with rectal bleeding. A colonoscopic biopsy revealed rectal adenocarcinoma and he was diagnosed with LARC after imaging studies. Capecitabine plus radiation therapy was started as a neoadjuvant treatment. MANAGEMENT AND OUTCOME: Seven days after the first capecitabine dose, the patient was admitted with a rash. The LCV diagnosis was histopathologically proven. Capecitabine was withheld. After the patient's rash began to regress under corticosteroid pressure, capecitabine was started at a lower dose. His treatment was completed successfully with oral corticosteroids plus low-dose capecitabine. DISCUSSION: We aimed to point out a rare and unusual adverse effect of a frequently used drug in oncologic practice.

Acute generalized exanthematous pustulosis complicated by cutaneous small vessel vasculitis: A case report and literature review.

Acute generalized exanthematous pustulosis (AGEP) is a rare skin eruption characterized by widespread erythematous lesions covered with numerous pustules. Leukocytoclastic vasculitis is now considered an uncommon but possible histopathological feature within the clinical and pathological spectrum of AGEP. Our report describes a rare case of AGEP overlapping with cutaneous small vessel vasculitis, a condition that has only been reported once in the literature.

Spontaneous Improvement of Aortitis Associated with Severe COVID-19 Infection-A Case Report.

Aortitis is a rare complication of the coronavirus disease 2019 (COVID-19) and is often treated empirically with steroids. We present a case of spontaneous resolution of aortitis without treatment. A 65-year-old man was admitted to our intensive care unit for severe COVID-19 pneumonia and underwent rehabilitation in the general ward. On day 12, he developed fever, and on day 13, he developed right cervical pain and increased inflammatory markers. On day 16, a cervical echocardiogram showed vasculitis in the right common carotid artery, and on day 17, computed tomography (CT) of the neck showed thickening of the arterial wall of the right common to the internal carotid arteries. A retrospective assessment of the CT scan on day 12 showed wall thickening from the thoracic aorta to the abdominal aorta, and a diagnosis of aortitis was made. Autoantibody analysis, culture, and magnetic resonance imaging (MRI) of the head and neck showed no abnormalities. During the investigation of the cause of aortitis, the fever and inflammatory reaction spontaneously resolved and the right cervical pain gradually improved. Therefore, the patient was diagnosed with transient COVID-19-related aortitis. To our knowledge, this is the first report describing the spontaneous resolution of COVID-19-related aortitis.

Leukocytoclastic vasculitis (cutaneous small-vessel vasculitis) after COVID-19 vaccination.

Vaccinations may induce cutaneous adverse events, due to nonspecific inflammation or immuno-mediated reactions. Several types of vasculitis have been observed. We report on a 71-year-old woman who developed cutaneous small-vessel vasculitis after the second dose of Vaxzevria COVID-19 vaccination, showing leukocytoclastic vasculitis on histopathological examination of a skin biopsy. Cutaneous small-vessel vasculitis is a rare condition which can be idiopathic or secondary to underlying infections, connective tissue disorders, malignancy, and medications. The pathogenesis involves immune complex deposition in small blood vessels, leading to activation of the complement system and recruitment of leukocytes. Exacerbation of small-vessel vasculitis has been reported following the administration of various vaccines, particularly influenza vaccine. It is expected that SARS-CoV-2 vaccine results in the activation of B- and T-cells and antibody formation. We hypothesize that leukocytoclastic vasculitis caused by immune complex deposition within cutaneous small vessels could be a rare side effect of Vaxzevria COVID-19 vaccination.

Post-COVID-19 vaccination IgA vasculitis in an adult.

Leukocytoclastic vasculitis has been reported in the setting of COVID-19 infection and post-COVID-19 vaccination. We report a case of IgA vasculitis (IgAV) post-COVID-19 vaccination, with immunoglobulin A (IgA) immune deposits in the skin and renal involvement. SARS-CoV spike protein immunohistochemical staining was negative. IgAV with skin and renal involvement is a potential reaction to COVID-19 vaccination.

Leukocytoclastic vasculitis after COVID-19 vaccination.

Leukocytoclastic vasculitis (LCV) is the vasculitis of small vessels. In this report, we describe a 38-year-old male patient who presented to our outpatient clinic with a 1-week history of rash on his lower extremities that had started 4 days after receiving the Pfizer-BioNTech SARS-CoV-2 vaccine. A diagnosis of LCV was made based on clinical and histopathological findings. The patient was treated with antihistamines and prednisolone, after which improvement was observed in the lesions. With this paper, we aim to raise awareness concerning the possibility of LCV development after COVID-19 vaccination.

A case of vasculitis triggered by infective endocarditis in a patient undergoing maintenance hemodialysis: a case report.

BACKGROUND: Immunoglobulin A vasculitis (IgA vasculitis) is one of the most common forms of vasculitis in children. It rarely occurs in adults. It is a systemic vasculitis with IgA deposition and is characterized by the classical tetrad of purpura, arthritis/arthralgia, gastrointestinal and renal involvement. Certain types of infections, and pharmacological agents have been reported to be associated with IgA vasculitis. Here, we describe a case of IgA vasculitis triggered by infective endocarditis in a patient undergoing maintenance hemodialysis. CASE PRESENTATION: A 70-year-old man undergoing hemodialysis was admitted because of skin purpura, abdominal pain, diarrhea, and lower back pain. We suspected him as IgA vasculitis based on the clinical features and skin biopsy findings. Transesophageal echocardiography revealed infective endocarditis, which predisposed him to IgA vasculitis. He was treated with antibiotics and low-dose corticosteroids, which led to resolution of vasculitis. CONCLUSIONS: This is the first case of IgA vasculitis triggered by infective endocarditis in a patient undergoing hemodialysis. Patients undergoing hemodialysis are at a high risk of infection because of immune dysfunction and frequent venipuncture. The incidence of infective endocarditis associated with IgA vasculitis is very low, but it has been repeatedly reported. Therefore, it is necessary to consider infective endocarditis in patients with clinical features that indicate IgA vasculitis.

A case of severe vasculitis after FLOT chemotherapy in a patient with metastatic gastric cancer who received multiple line chemotherapy.

INTRODUCTION: Leukocytoclastic vasculitis is a histopathological term describing vasculitis in which the inflammatory infiltrate in small vessels consists of neutrophils. Although FLOT is given perioperatively in locally advanced, resectable gastric or gastroesophageal junction adenocarcinoma, it has recently become a popular treatment option for metastatic cancers. In this case report, we present a case of FLOT-induced LCV. CASE REPORT: We present a 52-year-old patient with metastatic gastric adenocarcinoma treated with FLOT. The patient developed necrotizing vasculitis in the lower extremity after 5 cycles of FLOT. MANAGEMENT & OUTCOME: After discontinuation of the FLOT regimen, the necrotizing morbid LCV gradually regressed with steroid therapy. DISCUSSION: To the best of our knowledge, our case is the first case of LCV that developed after FLOT chemotherapy. The clinical appearance of the patient, occurrence after chemotherapy, erythematous rash developing on bilateral lower extremities, and palpable purpuric vasculitis made us suspect. We found a potential relationship between FLOT and vasculitis according to the Naranjo scale (score 4 + ).

Leukocytoclastic vasculitis due to ruxolitinib treatment: A rare adverse effect.

WHAT IS KNOWN AND OBJECTIVE: Primary myelofibrosis (PMF) is characterized by myeloid cell proliferation and prominent bone marrow fibrosis. Ruxolitinib, a selective inhibitor of JAK 1 and 2, significantly reduces constitutional symptoms and spleen size compared with placebo, and has significant clinical benefits in patients with myelofibrosis. The most common haematological side effects are thrombocytopenia and anaemia, and the most common non-haematological side effects are grade 1-2 diarrhoea and pyrexia. Leukocytoclastic vasculitis is small vessel vasculitis, characterized histopathologically by immune complex-mediated vasculitis of the dermal capillaries and venules in the lower extremities, which can be seen as palpable purpura. Although the cause is 50% idiopathic, the aetiology of leukocytoclastic vasculitis can be collected under many headings. CASE SUMMARY: The case is here presented of a patient with PMF who developed leukocytoclastic vasculitis after ruxolitinib treatment. Ruxolitinib was discontinued as the lesions were thought to be drug-related and all skin lesions disappeared approximately 2 months after termination of the drug. When the ruxolitinib treatment was restarted at the same dose (2 × 15 mg), the skin lesions recurred. The drug dose was reduced to 1 × 15 mg, and the rashes disappeared. Currently, the patient has no active complaints and is being followed up with ruxolitinib 1 × 15 mg without any complications. WHAT IS NEW AND CONCLUSION: To the best of our knowledge, leukocytoclastic vasculitis due to ruxolitinib is extremely uncommon. This case report can be considered to contribute to the literature of this rare event.

A Case of Leukocytoclastic Vasculitis Following SARS-COV-2 Vaccination.

BACKGROUND: Although vaccination against coronavirus severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) has been proven generally safe, rare but potentially serious adverse reactions do occur. Leukocytoclastic vasculitis (LCV) is a small-vessel vasculitis that has been associated with other immunizations, but, to our knowledge, has not been previously reported in association with vaccines directed against SARS-CoV-2. CASE REPORT: We report the case of a 22-year-old man with no known past medical history who presented to the Emergency Department with 2 days of migratory arthritis in his ankles and palpable purpura on his bilateral lower extremities, occurring 10 days after receiving the Johnson & Johnson SARS-CoV-2 vaccine. The patient's clinical presentation was suggestive of leukocytoclastic vasculitis, and this diagnosis was confirmed on skin biopsy. Why Should an Emergency Physician Be Aware of This? Recognition of vasculitides is important for timely treatment and prevention of complications. In a patient presenting with palpable purpura after immunization against SARS-CoV-2, LCV should be promptly considered and worked up by the Emergency Physician, though management is most often entirely outpatient and the clinical course is typically mild and self-resolving.

Localized Forms of Vasculitis.

PURPOSE OF REVIEW: To provide an updated review on epidemiology, clinical manifestations, diagnostic assessment, treatment, and prognosis of localized vasculitis, following the 2012 Revised International Chapel Hill Consensus Conference Nomenclature on single-organ vasculitis. RECENT FINDINGS: Localized, single-organ vasculitides encompass a group of rare conditions in which there is no evidence of concomitant systemic vasculitis. Most data on this topic derives from case reports and small case series. Although some aspects of these diseases, such as clinical manifestations and histologic findings, have already been extensively investigated, there is still a lack of robust data concerning the pathogenesis, epidemiology, and treatment. Localized vasculitides may have a wide range of clinical features depending on the organ affected. The inflammatory process may have a multifocal/diffuse or unifocal distribution. Diagnosis is usually based on histopathology findings and exclusion of systemic vasculitis, which may frequently pose a challenge. Further research on treatment is warranted.

A detailed analysis of the distribution, morphology, and histopathology of complex purpura in hospitalized patients: A case series of 68 patients.

BACKGROUND: Purpura in inpatients commonly leads to dermatologic consultation. The differential diagnosis is broad and algorithms are intricate. OBJECTIVE: We evaluated inpatient consultations for complex purpura to document the most common diagnoses and to validate the true diagnostic utility of histopathology, clinical morphology, and distribution. METHODS: We reviewed a case series of 68 inpatients during a 4-year period with a dermatologic consultation for purpura and biopsy findings of vasculitis or microvascular occlusion. RESULTS: Key features of complex purpura are nonbranching (round) versus branching (retiform) morphology, dependent versus acral or generalized distribution, and leukocytoclastic vasculitis versus microvascular occlusion (with emphasis on depth of involvement). Dependent nonbranching purpura with only superficial vessels involved by leukocytoclastic vasculitis was most often due to IgA vasculitis or cutaneous single-organ small-vessel vasculitis. In contrast, deeper involvement by leukocytoclastic vasculitis was suggestive of systemic disease (eg, antineutrophil cytoplasmic antibody-associated vasculitis). Branching purpura was concerning, with greater than 90% sensitivity and specificity for microvascular occlusion and associated high mortality (≈50%). The majority of patients who died had acral branching lesions. LIMITATIONS: Small sample size, inpatients at a tertiary care center, and retrospective nature are some limitations. CONCLUSION: Nonbranching dependent purpura corresponded to leukocytoclastic vasculitis, with the most common diagnoses being IgA vasculitis or skin-limited small-vessel vasculitis; patients with deep involvement often had systemic diseases. In this series, branching purpura was due to microvascular occlusion rather than medium-vessel vasculitis, and had associated high mortality.

Leukocytoclastic vasculitis as a cutaneous manifestation of ChAdOx1 nCoV-19 corona virus vaccine (recombinant).

With the present COVID-19 vaccination drive across the world, adverse skin reactions post COVID-19 vaccine is expected. Majority of these reactions seen were transient or local injection site reactions. However, as the larger population is being vaccinated, certain uncommon dermatological presentations including leukocytoclastic vasculitis, pityriasis rosea, and exacerbation of pre-existing autoimmune diseases are now being reported. Among all the COVID-19 vaccines, most of these reactions are seen with messenger ribonucleic acid-based Pfizer/BioNTech (BNT162b2) and Moderna (mRNA-1273) vaccine. We report two cases of leukocytoclastic vasculitis following ChAdOx1 nCoV-19 corona virus vaccine (recombinant) that bring out potential new dermatological manifestations of recombinant corona virus vaccine being administered across the European, South American, and Asian countries. It is important for all health care workers and patients to be aware of the corona virus vaccine associated adverse cutaneous reactions.

Dermatologic comorbidities of the patients with severe COVID-19: A case-control study.

The current studies focus on the association between COVID-19 and certain comorbidities. To the best of our knowledge, the association between severe COVID-19 and dermatologic comorbidities has not been reported yet. In this study, we aimed to describe the dermatologic comorbidities of patients with severe COVID-19 and compare it with the control group. Patients who have died at Usak Training and Research Hospital due to COVID-19 and other diseases in the COVID-19 Intensive Care Units and Internal Medicine Intensive Care Units were recruited into the study. Two groups were compared with each other regarding the most common dermatologic comorbidities. A total of 198 patients including 111 patients with COVID-19 and 87 age and sex-matched patients with other diseases were enrolled in the study. The most common dermatologic comorbidities were pruritus (8.1%), eczema (6.3%), skin infections (3.6%), leukocytoclastic vasculitis (1.8%), and urticaria (0.9%) in the COVID-19 group while they were skin infections (9.2%), eczema (3.4%), pruritus (2.3%), and urticaria (1.1%) in the control group. None of patients in the control group had leukocytoclastic vasculitis. There were no significant differences between COVID-19 and control groups in terms of pruritus, eczema, skin infections, and urticaria (P values were .117, .517, .181, .505, and 1.000, respectively). In conclusion, although it is not statistically significant, it appears that pruritus and leukocytoclastic vasculitis are more common in severe COVID-19 patients. These cytokines-related diseases in the immuno-cutaneous systems may give some clues on the COVID-19 severity. Further studies are required to elucidate the relationship between the immuno-cutaneous system and COVID-19 severity.