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.

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.

Cutaneous Small Vessel Vasculitis: A Practical Guide to Diagnosis and Management.

Cutaneous vasculitis may be a cutaneous manifestation of systemic vasculitis, a skin-limited variant of systemic vasculitis, or a type of single-organ vasculitis limited to the skin. Careful evaluation is necessary to distinguish these possibilities, differentiate skin-limited from systemic disease, and identify important underlying conditions, if present. Appropriate management depends on disease type and severity. In the case of cutaneous small vessel vasculitis (CSVV), treatment is primarily based on case reports and expert opinion, using a range of immunomodulatory or immunosuppressive therapies. This manuscript presents a systematic and stepwise approach to the evaluation of patients presenting with cutaneous vasculitis, as well as the management of those diagnosed with CSVV, using best available evidence. Studies relevant to the classification, evaluation, and management of skin vasculitis are ongoing.

Cutaneous vasculitis occurring in the setting of systemic lupus erythematosus: a multicentre cohort study.

OBJECTIVES: To describe the clinical and pathological features of biopsy-proven cutaneous vasculitis (CV) associated with SLE, focusing on diagnosis classification and impact on overall SLE activity. METHODS: Retrospective multicentric cohort study including SLE patients with biopsy-proven CV identified by (i) data from pathology departments of three university hospitals and (ii) a national call for cases. SLE was defined according to 1997 revised ACR and/or 2019 ACR/EULAR criteria. CV diagnosis was confirmed histologically and classified by using the dermatological addendum of the Chapel Hill classification. SLE activity and flare severity at the time of CV diagnosis were assessed independently of vasculitis items with the SELENA-SLEDAI and SELENA-SLEDAI Flare Index. RESULTS: Overall, 39 patients were included; 35 (90%) were female. Cutaneous manifestations included mostly palpable purpura (n = 21; 54%) and urticarial lesions (n = 18; 46%); lower limbs were the most common location (n = 33; 85%). Eleven (28%) patients exhibited extracutaneous vasculitis. A higher prevalence of Sjögren's syndrome (51%) was found compared with SLE patients without CV from the French referral centre group (12%, P < 0.0001) and the Swiss SLE Cohort (11%, P < 0.0001). CV was mostly classified as urticarial vasculitis (n = 14, 36%) and cryoglobulinaemia (n = 13, 33%). Only 2 (5%) patients had no other cause than SLE to explain the CV. Sixty-one percent of patients had inactive SLE. CONCLUSION: SLE-related vasculitis seems very rare and other causes of vasculitis should be ruled out before considering this diagnosis. Moreover, in more than half of patients, CV was not associated with another sign of active SLE.

Cutaneous small vessel vasculitis.

This review discusses the clinical manifestations of cutaneous small vessel vasculitis. The etiologies and work up will be explored as well as the treatment considerations. This entity is multifactorial and usually involves multiple specialties. The presentation can range from self-limited to life threatening, multi-organ failure. It is essential to be able to diagnose vasculitis and proceed with the appropriate laboratory studies and work-up. Finally, investigation of associated etiologies such as infection and drugs will guide additional diagnostic studies.

Manifestaciones cutáneas asociadas a enfermedad inflamatoria intestinal pediátrica. Serie de casos / Cutaneous manifestations associated with paediatric inflammatory bowel disease. Case series

Introducción. Las manifestaciones extraintestinales en la enfermedad inflamatoria intestinal tienen una prevalencia variable de 6 a 47%, dentro de las cuales las manifestaciones cutáneas en la edad pediátrica suponen un 10-15%, siendo las más frecuentes el pioderma gangrenoso y el eritema nodoso. Suelen presentar una adecuada evolución clínica, a pesar de ello es importante realizar un correcto diagnóstico con tratamiento precoz. Presentamos tres casos clínicos de enfermedad inflamatoria intestinal con manifestaciones dermatológicas asociadas. Caso 1. Vasculitis leucocitoclástica asociada a colitis ulcerosa en paciente varón de 11 años. Presenta buena respuesta al tratamiento con corticoterapia e inmunosupresores. Caso 2. Varón de 8 años con eritema nodoso asociado a enfermedad de Crohn. Buena respuesta clínica con nutrición enteral exclusiva e inmunosupresores. Caso 3. Mujer 15 años con psoriasis en gotas asociada a tratamiento con anti-TNF en enfermedad de Crohn. Resolución tras retirada de agente desencadenante. Conclusiones. Las manifestaciones dermatológicas en la edad pediátrica son menos frecuentes que en adultos. De manera habitual tienen un curso leve y autolimitado como es el caso de nuestros dos primeros pacientes. El tercer caso lo presentamos como causa de manifestación cutánea secundaria a tratamiento de mantenimiento de la enfermedad de base (AU)

Introduction. Extraintestinal manifestations in inflammatory bowel disease have a variable prevalence of 6-47%, within which cutaneous manifestations in pediatric age account for 10-15%, the most frequent being pyoderma gangrenosum and erythema nodosum. They usually present an adequate clinical evolution, in spite of this it is important to make a correct diagnosis with early treatment. Our objective is to present three clinical cases of dermatologic manifestations of inflammatory bowel disease from our practice. Case 1. Leukocytoclastic vasculitis associated with ulcerative colitis in an 11-year-old male patient. He presented good response to treatment with corticotherapy and immunomodulator. Case 2. 8-year-old male with erythema nodosum associated with Crohn´s disease. Good clinical response with exclusive enteral nutrition and immunomodulator. Case 3. 15-year-old woman with guttate psoriasis associated with anti-TNF treatment for Crohn´s disease. Resolution after withdrawal of the triggering agent. Conclusions. Dermatological manifestations in pediatric age are less frequent than in adults. They usually have a mild and self-limited course as in the case of our first two patients. The third case is presented as a cause of cutaneous manifestation secondary to maintenance treatment of the underlying disease (AU)

[Cutaneous Vasculitides - Clinical Manifestations, Diagnosis, and Aetiology]. / Kutane Vaskulitiden.

Cutaneous Vasculitides - Clinical Manifestations, Diagnosis, and Aetiology Abstract. Vasculitides are a heterogeneous group of diseases that are classified differently, for example according to the size of the affected vessel or according to primary and secondary causes. The skin is most frequently affected; it can be involved both as single organ vasculitis and in the context of systemic forms. The combination of skin lesions, their anatomical location and information on the time course provide clues for a differential diagnosis. Purpura, blisters, necrosis, ulcerations and possibly a livedo are characteristic manifestations. Constitutional symptoms such as weight loss, exhaustion, fever, and arthralgias are indicative of a systemic form. It is important to differentiate vasculitides from vasculopathies, which can manifest similarly. The most common form in adults is cutaneous leukocytoclastic angiitis, in children IgA vasculitis (Schönlein-Henoch purpura). Various triggers are possible: infections, drugs, autoimmune diseases, and malignancies, whereby up to 50% remain etiologically unexplained. Skin biopsies and laboratory parameters, if necessary supplemented with imaging, are important steps in the clarification process. Treatment is primarily directed at the elimination of a possible triggering cause. Idiopathic cutaneous leukocytoclastic angiitis usually resolves spontaneously; treatment is symptomatic. In more severe cases, topical corticosteroids or calcineurin antagonists are primarily used. In case of therapeutic resistance, systemic immunosuppressants are recommended.

Cutaneous Manifestations of COVID-19 in the Inpatient Setting.

Cutaneous findings have increasingly been reported in patients with coronavirus disease 2019 (COVID-19). This review discusses associated skin findings in patients with COVID-19 in the inpatient setting, ranging from vasculopathy-related lesions associated with high hospitalization rate and poor prognosis to inflammatory vesicular and urticarial eruptions that are rarely associated with prolonged hospitalization. We also discuss other reported COVID-19 cutaneous manifestations such as Sweet's syndrome, purpuric eruptions, and Multisystem Inflammatory Syndrome in Children. Although the relationship between dermatologic changes and COVID-19 disease progression is not fully elucidated, familiarity with cutaneous manifestations is valuable for physicians caring for patients hospitalized with COVID-19 and may help improve disease recognition and care.

Development of sacral/buttock retiform purpura as an ominous presenting sign of COVID-19 and clinical and histopathologic evolution during severe disease course.

Retiform purpura has been described as a relatively frequent cutaneous finding in patients with coronavirus disease 2019 (COVID-19). The etiology is hypothesized to be related to thrombotic vasculopathy based on lesional biopsy specimen findings, but the pathogenesis of the vasculopathy is not completely understood. Here, we present a case of a retiform purpuric patch on the sacrum/buttocks in a hospitalized patient prior to subsequent diagnosis of COVID-19 and an eventual fatal disease course. Two lesional biopsy specimens at different time points in the disease course revealed thrombotic vasculopathy, despite therapeutic anticoagulation. Detailed histopathologic evaluation using immunohistochemical markers suggest the etiology of the vasculopathy involves both persistent complement activation and platelet aggregation, which possibly promote ongoing thrombus formation. This case highlights that sacral/buttock retiform purpuric patches may be a presenting sign of infection with SARS-CoV-2 virus and may represent an ominous sign supporting a future severe disease course. In addition, biopsy specimen findings at separate time points demonstrate that cutaneous vasculopathy may persist despite adequate systemic anticoagulation, possibly due to the combination of persistent complement and platelet activation. Finally, occlusive thrombi in sacral/buttock retiform purpuric patches may contribute to future ulceration and significant cutaneous morbidity in patients who survive COVID-19.

Leucocytoclastic vasculitis due to acute bacterial endocarditis resolves with antibiotics.

Infective endocarditis is associated with a variety of clinical signs, but its association with multisystem vasculitis is rarely reported. A high index of suspicion is necessary to differentiate a primary autoimmune vasculitis from an infectious cause as the wrong treatment can lead to significant morbidity and mortality. We present a 71-year-old female patient with negative blood cultures, on antibiotics for recent bacteraemia, who presented with cutaneous and renal leucocytoclastic vasculitis. Workup revealed a vegetation adjacent to her right atrial pacemaker lead consistent with infective endocarditis and her vasculitis completely resolved with appropriate antibiotics.

Clinical and Histopathologic Characteristics of the Main Causes of Vascular Occusion - Part II: Coagulation Disorders, Emboli, and Other. / Dermatopatología de la oclusión intraluminal vascular: parteII (coagulopatías, émbolos y miscelánea).

Vascular occlusion has multiple, diverse clinical manifestations, some of which can have grave consequences for patients. It also has a wide variety of causes, including thrombi, which we recently addressed in partI of this review. In this second part, we look at additional causes of vascular occlusion.

Lessons From the First Wave of the Pandemic: Skin Features of COVID-19 Can Be Divided Into Inflammatory and Vascular Patterns [Formula: see text].

This review examines the clinical, morphological, and systemic factors related to coronavirus disease 2019 (COVID-19) cutaneous manifestations. The EMBASE, Medline, and Pubmed Central databases were searched from February 1, 2020 until April 25, 2020, using the search words "(COVID-19 OR SARS-CoV-2 OR coronavirus-19) AND (skin OR cutaneous OR dermatologic)". Cutaneous manifestations of COVID-19 were included. The cutaneous manifestations can be classified into 2 types. Patients with inflammatory reactions consisted of morbilliform, varicella-like, urticarial eruptions, and vesiculobullous manifestations. These manifestations were mainly found on the trunk, limbs, and faces of patients and had mainly positive COVID-19 polymerase chain reaction findings (97.7%). Furthermore, there were 516 patients with acral vascular lesions: chilblains, livedo lesions, cutaneous small-vessel vasculitis, and other noninflammatory purpura. These were often nonpruritic (88%) and not seen in severe disease (88.7%). The cutaneous lesions have potential for early diagnosis of COVID-19 and prevention of disease transmission. The implications of COVID-19 in the field of dermatology continue to evolve as more clinical data becomes available.

Intravascular histiocytosis: case report of a rare disease probably associated with silicone breast implant

Abstract Intravascular histiocytosis is a rare condition characterized by the aggregate of histiocytes within dilated dermal vessels. The diagnosis is mainly histophatological and immunohistochemical. We describe a case of a 55 year-old female patient presenting erythematous/purple patches on the breasts, back and limbs. She previously presented ductal carcinoma in the right breast in 2006 which was treated with mastectomy and proceeded to silicone breast implant in 2009. Clinical hypothesis was telangiectatic metastatic carcinoma. Histopathology showed vascular ectasia, thrombosis and recanalization of upper dermis small vessels. On immunohistochemistry, intravascular cells were CD 68+ and negative for estrogen and progesterone receptors, CK7, EMA and AE1/AE3 and endothelial cells were CD64+, leading to the diagnosis of intravascular histiocytosis.