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.

Cutis marmorata telangiectatica congenita is a rare congenital vascular malformation characterised by cutaneous vascular abnormalities, typically diagnosed at birth or in the early postnatal period. Although typically benign, this disease is associated with other systemic abnormalities, including rare ocular alterations, such as congenital glaucoma, cataracts and retinopathy.This manuscript describes a female infant, who presented with generalised livedo reticularis, a band of alopecia and cutaneous atrophy in the temporal region above the coronal suture. The patient was diagnosed with cutis marmorata telangiectatica congenita by a paediatrician, and an ophthalmological evaluation was requested. A funduscopy examination in both eyes showed temporal and superior retina with avascular areas with new vessels, venous dilations and shunts, and no retinal detachments. Given these findings, we performed retinal photocoagulation laser treatment with excellent results.This case report highlights the importance of early ophthalmological evaluation of children with this disease to prevent secondary complications, such as vitreous haemorrhage and tractional retinal detachment.

A previously healthy woman in late adolescence presented to the emergency department with stroke-like symptoms following a two-month history of bilateral foot pain and oedema, accompanied by a macular rash and progressive lower extremity weakness. On further investigation, she was found to have multiple cerebral emboli and a left atrial myxoma fixed to the interatrial septum. The patient subsequently underwent urgent surgical excision of the myxoma. On follow-up, her cutaneous and neurological symptoms were significantly improved. This case suggests that, in the presence of a vasculitic rash without evident or obvious cause, cardiac myxoma should be included in the differential diagnosis.

BACKGROUND: Cutaneous polyarteritis nodosa (cPAN) is a form of medium-sized vessel necrotizing vasculitis. It is a rare, skin-limited variant of polyarteritis nodosa, characterized by dermal and subcutaneous tissue involvement. The most common findings in cPAN include digital gangrene, livedo reticularis, and tender subcutaneous nodules. However, while limited to the skin, cPAN results in significant morbidity and mortality due to the accompanying skin ischemia and necrosis, such that patients are vulnerable to superinfection. Here, we describe a unique presentation of cPAN associated with pulmonary arterial hypertension (PAH).
METHODS: A 78-year-old female presented with digital ischemia and leg ulcers associated with PAH. Skin biopsy showed necrotizing fibrinoid necrosis of the small- and middle-sized vessels of the dermis. A diagnosis of cPAN and PAH was made. The patient was treated with glucocorticoids, vasodilators, and cyclophosphamide.
RESULTS: She died due to severe sepsis complications.
CONCLUSIONS: To date, this is the first case report describing the association between cPAN and PAH. In this case, PAH is a complication of the cutaneous vasculitides suggesting that vasculopathy could play a role in the pathophysiology of PAH. However, the underlying pathophysiological mechanisms still have to be firmly established.

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Cocaine, one of most prevalent illicit substances in the United States, affects a multitude of organ systems and precedes numerous negative health outcomes. Many of the consequences of cocaine are linked to induction of vasoconstriction. For this reason, cocaine users are placed at considerable risk of ischemic stroke, myocardial infarction, and cardiac arrhythmias. Furthermore, a prominent contaminant, levamisole, has been widely implicated in predisposing individuals to developing or exacerbating cutaneous vasculitides. This report details a 31-year-old woman with acute, localized necrotic skin lesions after cocaine use. Her clinical picture was complicated by a 17-year history of systemic lupus erythematosus (SLE) and Raynaud\'s phenomenon. This case examines the challenge of forming a differential diagnosis, initiating an appropriate workup, and interpreting serologic-based and immunologic-based studies to differentiate between SLE and drug-based etiologies of skin necrosis. Finally, we discuss appropriate treatment plans to mitigate symptoms and reduce future instances of drug-induced 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.

This survey study assesses the health-related quality of life outcomes in adult patients with cutaneous manifestations of vasculitis.

Wounds of the skin can have very different causes. Especially in clinically atypical or non-healing wounds, the very heterogeneous group of vasculitides is of particularly important differential diagnostic significance. Nowadays, the classification of vasculitis is based on the affected vessels according to the Chapel Hill consensus conference. Thus, potentially any part of the vascular system can be affected. It becomes clear that there is often a risk of systemic diseases with high interdisciplinary relevance.Clinically, the usually very painful wounds in cutaneous vasculitis develop from necrosis and are typically surrounded by an erythematous-livid rim in the florid phase. In addition to clinical inspection, the histopathological examination of biopsies is of particular importance in the usually extensive diagnostic work-up.Therapeutically, adequate wound treatment should always be performed with a focus on pain prevention and infection prophylaxis. In the case of edema, compression therapy also supports wound healing. In addition, it is often necessary to initiate systemic treatment with immunosuppressive or immunomodulating drugs. Whenever possible, causally relevant factors and comorbidities should be diagnosed early and avoided or treated. Otherwise, there is a risk of severe or even fatal disease progression.
UNASSIGNED: Wunden an der Haut können sehr unterschiedliche Ursachen haben. Insbesondere bei klinisch atypischen oder nicht-heilenden Wunden ist die sehr heterogene Gruppe der Vaskulitiden von besonders wichtiger differenzialdiagnostischer Bedeutung. Die Klassifikation der Vaskulitiden erfolgt heute entsprechend den betroffenen Gefäßen nach der Chapel-Hill-Konsensus-Konferenz. Von einer Vaskulitis kann potenziell jeder Teil des Gefäßsystems betroffen sein. Dadurch wird deutlich, dass oft die Gefahr von systemischen Erkrankungen mit hoher interdisziplinärer Relevanz besteht.Klinisch entwickeln sich die in der Regel sehr schmerzhaften Wunden bei kutaner Vaskulitis aus Nekrosen und sind typischerweise in der floriden Phase von einem erythematös-lividen Randsaum umgeben. In der meist umfangreichen Diagnostik hat zusätzlich zu der klinischen Inspektion die histopathologische Untersuchung von Biopsien einen besonders großen Stellenwert.Therapeutisch sollte immer eine adäquate Wundtherapie mit dem Fokus auf Schmerzvermeidung und Infektionsprophylaxe durchgeführt werden. Bei begleitenden Ödemen unterstützt die Kompressionstherapie zudem die Wundheilung. Darüber hinaus ist es oft notwendig, systemische Therapien mit immunsuppressiven oder immunmodulierenden Medikamenten einzuleiten. Wann immer möglich, sollten die ursächlich relevanten Faktoren und Komorbiditäten frühzeitig diagnostiziert und vermieden bzw. behandelt werden. Andernfalls besteht die Gefahr von schweren oder sogar tödlichen Krankheitsverläufen.

Hemimegalencephaly (HME) is a rare neurological diagnosis defined as hamartomatous overgrowth of one cerebral hemisphere. The hypothesised pathogenesis is due to an increased number or size of neural cells; however, the exact mechanism can vary widely, depending on the underlying aetiology. We report a case outlining the prenatal diagnostic process and obstetric considerations for delivering an infant with HME secondary to megalencephaly-capillary malformation syndrome. After diagnosis, our patient was induced and delivered at 37 weeks of gestation via operative vaginal delivery. To our knowledge, this is the first report describing the course from prenatal diagnosis through delivery of a fetus with HME.