Livedo reticularis

INTRODUCTION: Various cutaneous manifestations have been reported as symptoms of coronavirus disease 2019 (COVID-19), which may facilitate early clinical diagnosis and management. This study explored the incidence of cutaneous manifestations among hospitalised patients with COVID-19 and investigated its relationships with viral load, co-morbidities, and outcomes. METHODS: This retrospective study included adult patients admitted to a tertiary hospital for COVID-19 from July to September 2020...

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that affects almost all internal organs, among which circulatory system organs (CSO) lesions are not only among the most common but also at the top of the list of causes of mortality. The tactics of treatment of patients with SLE without and in combination with CSO lesions are fundamentally different, and therefore, improving diagnostic methods will help to enhance the effectiveness of the management of this category of patients. The aim of the study - to determine the diagnostic value of laboratory markers of syntropic lesions of the circulatory system organs in patients with systemic lupus erythematosus...

INTRODUCTION: Cryofibrinogen is an abnormal, cold-insoluble protein composed of a combination of fibrinogen, fibrin, and fibronectin. Cryofibrinogenemia can be essential (e.g. primary) or secondary to various conditions. While low levels of cryofibrinogen can be seen in asymptomatic healthy individuals without evidence of clinical features typical of cryofibrinogenemia, cryofibrinogenemia associated with clinical features is considered very rare. The clinical features of cryofibrinogenemia ranges from skin manifestations, including Raynaud's phenomenon and livedo reticularis, to more severe organ-threatening manifestations such as tissue ischemia and gangrene...

Adenosine deaminase 2 deficiency (DADA2) is a rare monogenic vasculopathy caused by loss-of-function homozygous or compound heterozygous mutations in ADA2, formerly CECR1 (cat eye syndrome chromosome region 1) gene. The DADA2 phenotype is widely heterogeneous, and patients may present with fever, weight loss, livedo reticularis/racemosa, digital ischemia, cutaneous ulceration, peripheral neuropathy, abdominal pain, bowel perforation, and portal or nephrogenic hypertension. More specific manifestations include early-onset ischemic or hemorrhagic stroke, mild immunodeficiency and hypogammaglobinemia, cytopenia, and vision disturbances...

BACKGROUND: NOTCH3, a large type I transmembrane receptor expressed on arterial smooth muscle cells and capillary pericytes, features a diverse extracellular domain with 34 epidermal growth factor-like repeats. It exhibits distinct phenotypes due to variant zygosity and type; missense mutations cause CADASIL with cerebral vasculopathy, while null mutations lead to severe congenital manifestations. METHODS: This report describes two cases with homozygous loss- of- function variants in NOTCH3 along with their clinical manifestations...

UNLABELLED: Erythema ab igne is a dermatological condition resulting from repeated low-grade heat exposure (below the burning point), which can variably manifest with reticulated erythema and skin hyperpigmentation. Not infrequently, the cause of such a skin disorder is not immediately evident or reported by patients, especially if these are children. Compared to adults, erythema ab igne is rare in children and, if the general practitioners and pediatricians are not aware of this disorder, pediatric patients are often addressed to rheumatologists and/or undergo useless immunological investigations...

No abstract text is available yet for this article.

COVID-19 is a global pandemic caused by a novel zoonotic RNA virus named SARS-CoV-2. Various cutaneous manifestations associated with COVID-19 have been described, including urticarial rash, confluent erythematous rash, papulovesicular exanthem, chilblain-like acral pattern, livedo reticularis, and purpuric vasculitis pattern. Here, we are presenting a case of a 45-year-old male with mucocutaneous features of Stevens-Johnson syndrome.

OBJECTIVES: Antiphospholipid syndrome (APS)-associated heart valve disease (HVD) is well described. Nonetheless, limited data exist on clinical parameters associated with the course of primary APS (pAPS) patients with HVD. The goal of this study was to assess clinical features and related outcomes in patients with APS associated HVD. METHODS: In this multicentre retrospective study, we identified 33 pAPS patients with HVD (pAPS-HVD group) and compared their clinical course with 128 pAPS patients with normal heart valves on echocardiography (pAPS-control group)...

BACKGROUND: Livedoid vasculopathy (LV) is a rare, disabling disease characterized by painful ulcers, livedo reticularis and atrophy blanche. Hypercoagulation, endothelial, and microcirculatory dysfunction are believed to be responsible for the pathogenesis of this difficult-to-treat disease. OBJECTIVES: This study sought to investigate the frequency of endothelial dysfunction, hypercoagulability, and nailfold capillaroscopic features in LV patients to shed light on its etiology...

Deficiency of adenosine deaminase 2 (DADA2), caused by recessive mutations in the ADA2 gene, results in cutaneous or systemic vasculitis with variable clinical manifestations. There is only one other case in literature carrying both ADA2 and MEFV gene pathogenic variants. Here we report the second case that carries both ADA2 and MEFV pathogenic variants, presenting with characteristic phenotypes of both FMF and DADA2. A male patient, currently 29 years old, was initially diagnosed with FMF and developed livedo reticularis and nodular dermal lesions compatible with cutaneous PAN a year after diagnosis...

Filler-related vascular occlusion (VO) treatment remains challenging despite established protocols, including high-dose pulsed hyaluronidase injections and ultrasound-guided targeted injections. Managing patients' pain and anxiety during treatment presents additional difficulties. Nitrous oxide (N2 O) has been found to be effective for analgesia and anxiolysis in minor procedures, with a 55% reduction in photodynamic therapy pain, and a visual analog scale reduction from 6.6 to 2.9 for aesthetic laser treatment pain...

Deficiency of adenosine deaminase 2 (DADA2) is an autoinflammatory disease caused by pathogenic variants of the ADA2 gene and has similar clinical features to polyarteritis nodosa (PAN). We, herein, report a case of DADA2 in Korea that was diagnosed in a patient with childhood-onset PAN. The patient had a truncal ataxia and facial palsy caused by thalamic infarction at 34 months of age. Livedo reticularis with Raynaud phenomenon and abdominal pain with fever were followed. Radiologic examination showed multiple infarctions in brain and kidney...

OBJECTIVE: Through a case of deficiency of adenosine deaminase 2 (DADA2) to improve domestic clinicians' understanding of the disease, and to review the literature, promote dermatologists for clinical secondary primary lesion diagnosis. METHOD: Analysis of a case diagnosed with DADA2 deficiency of clinical manifestations, laboratory, imaging examination and treatment methods, and discussion through literature analysis. RESULTS: The child with recurrent fever, limbs nodular erythema, gradually in the limbs...

We present a rare case of slow-progressing neurocutaneous vasculopathy described as Sneddon syndrome. A child presented with global developmental delay, congenital livedo racemosa, unilateral vision loss, and a past history of focal neurological deficit. Our main objective is to make physicians aware of this nature of presentation in children.

OBJECTIVE: This study investigates the positivity and relevance of non-criteria antiphospholipid antibodies (aPLs) with clinical phenotypes in patients highly suspected of or diagnosed with antiphospholipid antibody syndrome (APS). METHODS: Outpatient cases were included from a prospectively-maintained database, and patients were grouped into APS (n = 168), seronegative APS (SNAPS, n = 9), those meeting the diagnostic criteria for clinical events without laboratory results (only event, n = 15), those that had aPLs positivity without clinical manifestations (asymptomatic APA, n = 39), and healthy controls (n = 88)...

Primary systemic vasculitis can present with a wide spectrum of manifestations ranging from systemic non-specific features such as fever, malaise, arthralgia and myalgia to specific organ damage. We describe two cases of cholesterol embolisation syndrome and Kaposi sarcoma mimicking primary systemic vasculitis, both of which were characterised by features such as livedo reticularis , blue toe syndrome, a brown purpuric skin rash and positive perinuclear anti-neutrophil cytoplasmic antibodies associated with Kaposi sarcoma...

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...

BACKGROUND: Deficiency of adenosine deaminase 2 (DADA2) is an autosomal recessive autoinflammatory disease caused by mutations in the ADA2 gene. DADA2 has a broad spectrum of clinical presentations. Apart from systemic manifestations, we can categorize most of the signs and symptoms of DADA2 into the three groups of vasculitis, hematologic abnormalities, and immunologic dysregulations. The most dominant vasculitis features are skin manifestations, mostly in the form of livedo racemosa/reticularis, and early onset ischemic or hemorrhagic strokes...

The central nervous system (CNS) is considered as one of the most frequently affected organs in antiphospholipid syndrome (APS). This study investigated the prevalence of CNS manifestations in APS and associated risk factors and evaluated stroke recurrence. We carried out this retrospective study from 2009 to 2021 at Peking University People's Hospital, which enrolled 342 APS patients, and 174 neurologic events were suffered by 119 patients (34.8%). Patients with and without CNS involvement were compared regarding demographics and laboratory parameters...