Erythema elevatum diutinum in a patient with well-controlled Crohn disease.

Erythema elevatum diutinum is a rare, chronic cutaneous leukocytoclastic vasculitis, with prominent fibrosis at its later stage. In this article, we report a case of erythema elevatum diutinum in a 23-year-old woman with well-controlled Crohn disease. To our knowledge, erythema elevatum diutinum has been reported in only three other cases of Crohn disease, in which eruptions of erythema elevatum diutinum were associated with features of active Crohn. Our patient was in clinical remission at the time of erythema elevatum diutinum onset, making this report significant not only for its uncommon presentation, but more importantly, to aid readers. diagnosis and clinical management of similar cases.

Urticarial Vasculitis.

Urticarial vasculitis is a rare autoimmune disorder characterized by persistent edematous papules and plaques on the skin that last longer than 24 hours, often accompanied by systemic symptoms such as joint pain and fever. Unlike common urticaria, this condition involves inflammation of small blood vessels, leading to more severe and long-lasting skin lesions with a tendency to leave a bruiselike appearance. Diagnosis is challenging and may require a skin biopsy. Associated with underlying autoimmune diseases, treatment involves managing symptoms with medications such as antihistamines and corticosteroids, addressing the immune system's dysfunction, and treating any concurrent autoimmune conditions.

Cutaneous Perivascular Hemophagocytosis: A Report of 2 Cases With Emphasis on Clinical and Histological Features.

ABSTRACT: Cutaneous perivascular hemophagocytosis (CH) is a histological manifestation that manifests as systemic hemophagocytic syndrome, also known as hemophagocytic lymphohistiocytosis, when accompanied by fever, hepatosplenomegaly, liver dysfunction, and cytopenia, and may rarely manifest independently of hemophagocytic lymphohistiocytosis. CH typically presents as purpuric or brownish macules and patches on the extremities, abdomen, and trunks. Histopathologically, the hallmark of CH includes extravasated erythrocytes and karyorrhectic debris phagocytized by histiocytes, associated with dermal capillary ectasia, perivascular infiltration of neutrophils, nuclear dust, and histiocytes without atypia. In this study, we report 2 cases of CH encountered in routine diagnostic practice and elucidate their significant clinical and histologic features. Our first patient had leukocytoclastic vasculitis with CH in the setting of Yersinia enterocolitis, and the second case represents CH in association with non-Hodgkin lymphoma. This study highlights the importance of considering CH as a potential indicator of underlying systemic pathology, including infectious and hematological disorders, in clinical practice.

A rare case of phenobarbital-induced leukocytoclastic vasculitis.

Drug hypersensitivity reactions (DHRs) manifested as vasculitis are rare. Antibiotics, non-steroidal anti-inflammatory drugs (NSAIDs), sulphonamides, diuretics, immunosupressants and anticonvulsants are the most common culprits for drug-induced leukocytoclastic vasculitis (LCV) but there is scarce information about barbiturates. We present a case of 53-year-old female with severe vasculitis after phenobarbital- and NSAIDs-containing medications use. The preliminary diagnosis of drug-induced vasculitis was made based on anamnestic and clinical data. Further examinations confirmed the diagnosis of LCV and excluded other more common causes of vasculitis. The causative significance of used medications was assessed by long-term observation of the patient after the reaction, including the drug challenge series and Naranjo's Adverse Drug Reaction Probability Scale. It was concluded that phenobarbital is the most probable culprit drug. The patient's data were included in the Armenian Registry of Patients with Severe DHRs. Since then, the patient has avoided only barbiturate-containing drugs and no reactions were noted. Thus, the case indicates that even with limited diagnostic capabilities, the final diagnosis of rare drug-induced LCV and even rarer culprit drug can be established by comparing the available data. Awareness about phenobarbital and proper recording of the case are important in the management and prevention of DHRs manifested as vasculitis.

Leukocytoclastic Vasculitis Localized to the Uterine Cervix.

Patients with gynecologic vasculitis should be evaluated for systemic disease as prognosis and treatment can vary depending on systemic involvement versus isolated disease. Leukocytoclastic vasculitis is a rare, immune-mediated small-vessel vasculitis. Leukocytoclastic vasculitis of the uterine cervix with systemic involvement has not previously been reported. A 25-year-old female with abnormal cervical cancer screening presented for colposcopy. Biopsies were notable for dysplasia and concurrent leukocytoclastic vasculitis. The patient later recalled a recurrent rash of her lower extremities, suspicious for systemic disease. Patients with gynecologic vasculitis should be evaluated for systemic involvement because prognosis and treatment differ from that of isolated disease. Additionally, leukocytoclastic vasculitis of the uterine cervix may be associated with both hormonal contraception and infections such as human papillomavirus, and any resulting cervical dysplasia should be monitored for progression and treated accordingly.

Kikuchi-Fujimoto disease associated with a flare-up of a leukocytoclastic vasculitis: A rare case report and review of literature.

RATIONALE: Kikuchi-Fujimoto disease (KFD) also known as histiocytic necrotizing lymphadenopathy is an exceedingly rare cause of cervical lymphadenopathy, commonly accompanied by systemic symptoms such as fever, fatigue, night sweats, myalgia, skin rash. PATIENT CONCERNS: In this paper, we report the case of a 22-year-old female patient who experienced a flare-up of leukocytoclastic vasculitis that was complicated by the appearance of a cervical lymph node with dysphagia, fever and nausea. DIAGNOSIS: Infectious and autoimmune workup came back negative. INTERVENTIONS: Excisional lymph node biopsy was done and the pathology results were consistent with histiocytic necrotizing lymphadenitis in keeping with Kikuchi-Fujimoto disease. OUTCOMES: Patient improved on intravenous corticosteroids and was discharged on per os prednisone. Six month follow-up shows complete resolution of her symptoms. LESSONS: KFD should be ruled out in patients with autoimmune or inflammatory diseases who develop lymphadenopathies.

Clinical study on single-organ cutaneous small vessels vasculitis: a retrospective observational study.

OBJECTIVE: Single-organ cutaneous small-vessel vasculitis (SoCSVV) is an inflammatory skin-limited vascular disease affecting the dermal and/or hypodermal vessel wall. Pathogenetically, idiopathic forms are described, as well as the induction from different triggers, such as infections, drugs, and vaccines. Following the Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2) pandemic outbreak, cases of cutaneous vasculitis induced by both COVID-19 and COVID-19 vaccinations have been reported in literature. The aim of this study is to provide the most recent evidence on new etiological factors, clinical features, and management of the SoCSVV. PATIENTS AND METHODS: We included 42 patients (22 women, 20 men) with SoCSVV and no systemic involvement in the study. The mean age of the patients was 57.3 years. Palpable purpura was the most frequent clinical manifestation (38 cases-90.4%). All patients were diagnosed with leukocytoclastic vasculitis by skin biopsy. RESULTS: The etiological factors were as follows: idiopathic in 9 (21%) patients, drug-related in 19 (45%) patients, COVID-19 infection-related in 5 (12%) patients, post-COVID-19 vaccination in 5 (12%) patients, paraneoplastic in 2 (5%) patients, and drug and infection and sepsis in 1 patient each. Among the drug-related cases, 16 (84%) were antibiotic-related, and most of them were beta-lactam antibiotics. Eosinophilia was present in skin biopsy in the cases related to vaccination and drugs, while intense necrosis and vascular damage in the skin were observed in the cases related to COVID-19 infection, unlike the others. A rapid resolution was observed with the cessation of drugs and short-term steroid treatment for the precipitating factors. CONCLUSIONS: SoCSVV is usually associated with drugs, preceding infections, and vaccines. COVID-19 infection and COVID-19 vaccinations have been reported as new etiological factors. SoCSVV indicates that the disease seems to be a mild, self-limiting illness with a good clinical result.

Accelerating Healing and Relieving Pain: High-Intensity Laser Therapy for Paraneoplastic Cutaneous Vasculitis Associated with Multiple Myeloma.

BACKGROUND Leukocytoclastic vasculitis (LCV) is an atypical form of cutaneous paraneoplastic manifestation. Its association with multiple myeloma (MM) is even rarer and is associated with poor prognosis and short survival, regardless of the therapy instituted. Different treatment approaches are necessary. We present a case in which LCV was the first manifestation of MM, and high-intensity laser therapy (HILT) was used as an option to accelerate healing and control pain. CASE REPORT A 76-year-old woman presented with pain and paresthesia in her lower limbs, associated with palpable purpura. The clinical diagnosis was small-vessel vasculitis. Laboratory tests showed an elevated gamma globulin monoclonal peak on protein electrophoresis. The immunophenotypic study of bone marrow aspirates led to the diagnosis of MM. Due to pain refractory to conventional analgesics, and the progressive evolution of the lesions, despite corticosteroid therapy, we performed photo-biomodulation with a neodymium-doped yttrium aluminum garnet laser (Nd: YAG), wavelength 1064 nanometers, using a 7-mm probe and energy density 6 J/cm². After the first session, the patient was referred for pain management, and after 5 weeks, we observed complete healing in ulcerated lesions and involution of bullous lesions. CONCLUSIONS This case report shows the positive effects of the Nd: YAG laser in modulating healing and reducing pain. HILT is an innovative, non-invasive, and effective treatment and should be considered a promising technique to accelerate healing and controlling pain.

Acute neutrophilic vasculitis (leukocytoclasia) in 36 COVID-19 autopsy brains.

BACKGROUND: Hypercytokinemia, the renin-angiotensin system, hypoxia, immune dysregulation, and vasculopathy with evidence of immune-related damage are implicated in brain morbidity in COVID-19 along with a wide variety of genomic and environmental influences. There is relatively little evidence of direct SARS-CoV-2 brain infection in COVID-19 patients. METHODS: Brain histopathology of 36 consecutive autopsies of patients who were RT-PCR positive for SARS-CoV-2 was studied along with findings from contemporary and pre-pandemic historical control groups. Immunostaining for serum and blood cell proteins and for complement components was employed. Microcirculatory wall complement deposition in the COVID-19 cohort was compared to historical control cases. Comparisons also included other relevant clinicopathological and microcirculatory findings in the COVID-19 cohort and control groups. RESULTS: The COVID-19 cohort and both the contemporary and historical control groups had the same rate of hypertension, diabetes mellitus, and obesity. The COVID-19 cohort had varying amounts of acute neutrophilic vasculitis with leukocytoclasia in the microcirculation of the brain in all cases. Prominent vascular neutrophilic transmural migration was found in several cases and 25 cases had acute perivasculitis. Paravascular microhemorrhages and petechial hemorrhages (small brain parenchymal hemorrhages) had a slight tendency to be more numerous in cohort cases that displayed less acute neutrophilic vasculitis. Tissue burden of acute neutrophilic vasculitis with leukocytoclasia was the same in control cases as a group, while it was significantly higher in COVID-19 cases. Both the tissue burden of acute neutrophilic vasculitis and the activation of complement components, including membrane attack complex, were significantly higher in microcirculatory channels in COVID-19 cohort brains than in historical controls. CONCLUSIONS: Acute neutrophilic vasculitis with leukocytoclasia, acute perivasculitis, and associated paravascular blood extravasation into brain parenchyma constitute the first phase of an immune-related, acute small-vessel inflammatory condition often termed type 3 hypersensitivity vasculitis or leukocytoclastic vasculitis. There is a higher tissue burden of acute neutrophilic vasculitis and an increased level of activated complement components in microcirculatory walls in COVID-19 cases than in pre-pandemic control cases. These findings are consistent with a more extensive small-vessel immune-related vasculitis in COVID-19 cases than in control cases. The pathway(s) and mechanism for these findings are speculative.