Sarcoidal Granuloma Annulare-Like Dermatitis and Vasculitis With Granulomatous Features: An Atypical Case of Giant Cell Arteritis.

ABSTRACT: Giant cell arteritis (GCA) is a diagnosis that clinicians should not miss because of the accompanying risk of irreversible vision loss. GCA can present without the classic symptoms of headache and temporal artery tenderness, which may lead to a delay in diagnosis. Cutaneous findings, although rare, have been associated with GCA. Accordingly, it is imperative to be aware of the broad clinical and histological presentations of GCA, including the cutaneous findings, because they may prove to be harbingers of impending disease. We present a unique case of GCA where 2 distinct cutaneous morphologies, sarcoidal granuloma annulare-like dermatitis and leukocytoclastic vasculitis with granulomatous features, presented simultaneously before the classic symptoms of headache and unilateral vision loss.

A Rare Case of Infliximab-Induced Small Vessel Vasculitis With Renal Involvement.

There are few cases in the literature demonstrating vasculitis induced by tumor necrosis factor-α. There exist even fewer cases of systemic inflammation involving the skin, nerves, and kidneys. Here, we present a novel case of a 27-year-old man with Crohn disease refractory to multiple medications, most recently treated with infliximab. He presented with a 3-week history of non-blanching palpable petechial rash involving his bilateral extremities and right upper extremity as well as lesions with black eschar around his ankles. He was found to have refractory cutaneous small vessel vasculitis, nephrotic range proteinuria, and small fiber neuropathy. This case describes the evaluation and treatment of systemic small vessel vasculitis in the setting of infliximab therapy.

A case of hypocomplementemic urticarial vasculitis syndrome complicated by eosinophilic pneumonia: a case report and review of the literature.

The primary symptom of urticarial vasculitis (UV), which is a histopathological leukocytoclastic vasculitis disease, is an eruption that resembles urticaria. Other organs may also experience accompanying symptoms. Lung lesions with UV are mostly obstructive pulmonary disease with smoking. However, the coexistence of eosinophilic pneumonia (EP) and complicated UV remains unclear. We report a man in his 70s with chronic obstructive pulmonary disease who attended our department with ring-shaped erythema, marginal edema, and pigmentation. Additionally, a skin histological analysis showed nuclear dust and perivascular neutrophil infiltration, while a blood sample showed a decrease in C3 and C1q concentrations. Administration of prednisone temporarily improved the eruption. However, he developed a cough and a new UV eruption 1 year later. Computed tomography revealed infiltration in the right upper lobe of the lungs, and a blood sample showed a high eosinophil count. He was finally diagnosed with hypocomplementemic urticarial vasculitis syndrome and idiopathic chronic EP. A previous study showed that serum C1q concentrations in patients with EP were lower when this disease was active. Whether a decline in C1q concentrations can cause EP is unclear. However, our case is unique owing to the co-onset of EP with low complement concentrations and recurrence of UV.

Disseminated tuberculosis presenting as cutaneous leucocytoclastic vasculitis.

Cutaneous leucocytoclastic vasculitis (CLV) is a type of small vessel vasculitis, predominantly presenting with palpable purpuras and sometimes with systemic manifestations. The following report describes the case of a woman, who presented with fever, anorexia and maculopapular lesions over both lower limbs. Skin biopsy revealed CLV. CT scan demonstrated bilateral pulmonary nodules, ileocecal wall thickening and generalised lymphadenopathy. Colonoscopy guided biopsy obtained from ileocecal valve ulcer showed epitheloid cell granuloma with Langhans-type giant cells and caseous necrosis. Rapid clinical improvement was seen with anti-tubercular therapy. Among infectious causes, although rare and an uncommon presentation, Mycobacterium tuberculosis should be considered as an important cause of CLV.

Localised chronic fibrosing vasculitis versus erythema elevatum diutinum.

A woman in her 70s was referred for a painless plaque on the shin, present for 2 years and progressing in thickness. Examination revealed a large erythematous to violaceous indurated plaque with cobblestone appearance. Biopsy revealed an inflammatory infiltrate of neutrophils with scattered histiocytes, lymphocytes, eosinophils and plasma cells interspersed with areas of lamellar fibrosis and focal areas of vascular damage, suggestive of a localised chronic fibrosing vasculitis of the skin. Localised chronic fibrosing vasculitis is a rare dermatosis, typically presenting as ulcerated violet-red nodules, which can appear histologically similar to erythema elevatum diutinum (EED), which typically presents as red-brown annular plaques. EED may have a predominance of neutrophils and granulomas, while chronic fibrosing vasculitis may have a sparse infiltrate of mixed inflammatory cells without granulomas. While dapsone is a first-line treatment for EED, there are no formal guidelines on the treatment of localised chronic fibrosing vasculitis. Given the neutrophils in this sample and similarities with EED, this patient was treated with oral dapsone, resulting in plaque improvement.

IgA Vasculitis in the Setting of Biologic Therapy for Psoriasis and Recurrent Cutaneous Methicillin-Resistant Staphylococcus aureus Colonization.

IgA vasculitis is a form of cutaneous small-vessel leukocytoclastic vasculitis (LCV) that has various triggers, including anti-tumor necrosis factor (TNF) α therapy. As the use of more targeted biologic therapies such as the IL-17 inhibitor secukinumab increases, so do reports of associated adverse events. Herein, we describe an uncommon case of IgA vasculitis in a man undergoing biologic therapy with adalimumab and secukinumab for psoriasis with recurrent cutaneous methicillin-resistant Staphylococcus aureus (MRSA) colonization. A review of the current literature also is provided.

Correct approach in urticarial vasculitis made early diagnosis of lupus nephritis possible: a case report.

BACKGROUND: Urticarial vasculitis is a clinicopathologic entity defined by recurrent episodes of urticarial lesions that persist > 24 hours and demonstrate the histopathologic features of leukocytoclastic vasculitis. The most important prognostic feature is the presence of normo- or hypocomplementemia. In the latter, patients are much more likely to have systemic manifestations. Urticarial vasculitis is most often idiopathic, but it can arise in association with autoimmune connective diseases, cryoglobulinemia, infections, medications, and hematologic malignancies. CASE PRESENTATION: We present the case of a 61-year-old Caucasian woman with a skin eruption that consisted of erythematous plaques on the trunk and limbs that lasted > 24 hours but were asymptomatic. The skin eruption had an acute onset and persisted for 3 months upon initial presentation in our dermatology department. A punch biopsy showed signs of a leukocytoclastic vasculitis in the superficial dermis. On laboratory examination, signs of activation of the complement system were found with low complement C3, C4, and C1q, and with a high anti-C1q antibody titer. The clinical, histological, and lab results fit the diagnosis of hypocomplementemic urticarial vasculitis. There was also a positive antinuclear factor with elevated U1 small nuclear ribonucleoprotein and high double-stranded DNA determined by Farr method. On urinalysis, marked proteinuria and massive hematuria were found. Kidney biopsy showed focal crescentic and focal mesangial type of glomerular damage with a full-blown positivity of immunoglobulin A, immunoglobulin G, and C1q, leading to lupus nephritis class III-A (according to the International Society of Nephrology/Renal Pathology Society 2003 classification of lupus nephritis). The patient was treated with hydroxychloroquine, corticosteroids, and low-dose intravenous cyclophosphamide (Euro-Lupus regimen) as remission-inducing agent, followed by azathioprine as remission-maintaining agent. This treatment regimen gave good results, with total clearance of the skin lesions and remission of the lupus nephritis. CONCLUSION: Clinicopathologic recognition of urticarial vasculitis with correct screening for extracutaneous disease can lead to early diagnosis of serious organ involvement and thereby improve prognosis for the patient.

Granulocyte colony-stimulating factor as a cause of acute leucocytoclastic vasculitis with anti-Ro and anti-La antibodies.

Granulocyte colony-stimulating factor (G-CSF) administration is associated with a diverse range of cutaneous sequelae. Serious dermatological side effects of G-CSF include the development of Sweet's syndrome and exacerbations of pre-existing inflammatory disorders such as psoriasis. Here, we describe a report of acute leucocytoclastic vasculitis caused by G-CSF therapy associated with anti-Ro and anti-La antibodies in a patient with multiple myeloma. This case highlights the importance of having a high index of suspicion for acute leucocytoclastic vasculitis in patients with haematological malignancies undergoing G-CSF therapy.

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 (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.

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.

Anti-TNF-Related Leukocytoclastic Vasculitis in Ulcerative Colitis: A Case Report.

BACKGROUND: The development of drugs directed against tumor necrosis factor (TNF)-α has dramatically modified the therapeutic approach to inflammatory bowel diseases: a larger use of such drugs has also led to a major knowledge about their adverse effects, especially on skin. The aim of this report was to describe a rare steroid-dependent form of leukocytoclastic vasculitis induced by an anti-TNF-α agent in a young woman with ulcerative colitis. CASE PRESENTATION: A young girl with ulcerative colitis developed a form of leukocytoclastic vasculitis induced by an anti-TNF-α agent. Recurrent palpable purpuric lesions on her legs were the main cutaneous manifestation. Skin lesions were steroid-dependent, but improved after withdrawal of the anti-TNF-α agent and second-line immunosuppressant therapy. CONCLUSIONS: The need to develop specific recommendations to guide the use of medications for managing skin reactions induced by anti-TNF-α drugs is herein emphasized.

Tofacitinib Treatment of Refractory Cutaneous Leukocytoclastic Vasculitis: A Case Report.

Introduction: To date, there is no treatment with proven efficacy for cutaneous leukocytoclastic vasculitis (CLV). Several reports have suggested that CLV responds favorably to corticosteroids, colchicine, nonsteroidal anti-inflammatory drugs (NSAIDs), azathioprine, and hydroxychloroquine (HCQ). To the best of our knowledge, the oral small molecule Janus kinase inhibitor, tofacitinib, plays an important role in the treatment of autoimmune and inflammatory diseases. Therefore, tofacitinib may be a prospective therapy in patients with CLV. Case Presentation: A 29-year-old woman presented to our hospital with a 5-year history of symmetric skin lesions mainly affecting both lower extremities. The results for anti-neutrophil cytoplasmic antibodies (ANCA), anti-extracted nuclear antigens (ENA) autoantibodies, anti-double-stranded deoxyribonucleic acid (dsDNA) antibodies, and antinuclear antibodies (ANA) were all negative. The definite diagnosis of CLV was determined by a skin biopsy. However, the patient exhibited a poor response to prednisone, HCQ, methotrexate, colchicine, azathioprine, and tripterygium wilfordii polyglycoside tablets (TGTs) treatments. She was then treated with oral tofacitinib (5 mg twice daily) and oral prednisone (25 mg daily). Outcomes: Her skin lesions gradually improved over a period of 4 weeks. Two months later, the skin ulcers completely resolved. No evidence of recurrence of skin ulcers was observed during a 6-month follow-up. Conclusion: We present the first case of a female patient receiving short-term tofacitinib therapy for refractory CLV. Tofacitinib may be a promising oral alternative for patients with CLV. However, its efficacy and safety require further appraisal through clinical trials.

Mediterranean spotted fever associated with leucocytoclastic vasculitis and acute pancraeatitis.

A young healthy gardener became febrile with abdominal pain, nausea, vomiting and diarrhoea followed by palpable purpura, mostly on the legs and buttocks with associated arthralgia. Dehydration, azotemia and hyponatraemia resolved with fluid replacement. Tests demonstrated acute pancreatitis, hepatitis, thrombocytopenia, microscopic haematuria and proteinuria. He improved with doxycycline, but bipedal pitting oedema and punctate rash involving the soles/hands appeared. Microbiological tests revealed positive IgM and IgG serology for rickettsiae spotted fever. Skin biopsy of the purpura confirmed leucocytoclastic vasculitis, positive for Rickettsiae conorii by PCR amplification. Palpable purpura is a rare important manifestation of Mediterranean spotted fever (MSF), due to either secondary leucocytoclastic vasculitis or associated Henoch-Schonlein purpura (HSP), which best explains the distribution of the rash, arthralgia, gastrointestinal symptoms, and microhaematuria not usually seen in R. conorii infections. Likewise, the patient's acute pancreatitis may be interpreted as a rare presentation of HSP or a seldom-encountered feature of MSF.

Dapsone-induced DRESS after infliximab-induced vasculitis: a case of cerebral infarction in the context of multiple drug reactions.

Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome is a life-threatening condition characterised by peripheral eosinophilia, rash and multi-organ failure arising several weeks after exposure to the culprit medication. Although rare, DRESS syndrome triggered by specific agents has been associated with specific genetic polymorphisms more prevalent in different ethnic groups, including an association between dapsone-induced DRESS and Human Leukocyte Antigen (HLA)-B:13*01, a single nucleotide polymorphism more prevalent in those of Asian descent. DRESS and drug-related vasculitis may affect any organ system including the central nervous system (CNS), usually manifesting as encephalitis, meningitis or embolic cerebrovascular accidents related to eosinophilic cardiac disease and thrombosis. CNS vasculitis is a much rarer complication of drug reactions that may manifest as multifocal ischemia on neuroimaging. In circumstances of drug-related vasculitides, treatment with high-dose corticosteroids may lead to rapid improvement and, ultimately, resolution of associated focal neurologic deficits.

The Anti-inflammatory Effects of HMGB1 Blockades in a Mouse Model of Cutaneous Vasculitis.

In our previous study, we have found increased serum levels of HMGB1 in patients with Henoch- Schonlein purpura (HSP), allergic vasculitis (AV), and urticarial vasculitis (UV) and altered HMGB1 distribution in lesional skin in patients with HSP. HMGB1 plays a pro-inflammatory role in the pathogenesis of HSP. To further investigate the role of HMGB1 in the pathogenic mechanism of vasculitis, we investigated the anti-inflammatory effects of HMGB1 blockades (including anti-HMGB1 mAb and glycyrrhizin) in a mouse model of a cutaneous reverse passive Arthus (RPA) reaction. A total of 36 balb/c mice were randomly divided into four groups: the control group, IC model group, HMGB1 monoclonal antibody (anti-HMGB1-mAb) group and the glycyrrhizin group, with nine mice in each group. A cutaneous RPA reaction mouse model was established by injections of the OVA antibody and the OVA antigen. Mice of the anti-HMGB1-mAb group and glycyrrhizin group were pre-treated with anti-HMGB1 mAb or glycyrrhizin, respectively, before the RPA reaction. Our results indicated that HMGB1 blockades (anti-HMGB1 mAb and glycyrrhizin) obviously extenuated the severity of vasculitis skin damage and improved the histological evolvement of inflammatory cells infiltration, vascular fibroid necrosis, and vasodilation in a cutaneous RPA reaction mouse model. In addition, HMGB1 blockades reduced the infiltration of neutrophils, DCs, and T cells and decreased the mRNA expression of IL-6 and CCL5 in skin lesions in the cutaneous RPA reaction mouse model. We suggest that HMGB1 blockades may represent a new direction for the treatment of cutaneous vasculitis.

Leucocytoclastic vasculitis in a patient with COVID-19 with positive SARS-CoV-2 PCR in skin biopsy.

Main skin manifestations of COVID-19 have been recently classified. However, little is known about cutaneous histopathological patterns and the presence of SARS-CoV-2 in these skin lesions. We present a healthy 29-year-old man who developed a leucocytoclastic vasculitis for COVID-19 with positive SARS-CoV-2 PCR in skin biopsy.