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Background/Objectives: Our aim was to validate the performance of the American College of Rheumatology (ACR)/European League Against Rheumatism (EULAR) classification criteria for antiphospholipid syndrome (APS), published in 2023, in an APS cohort. Methods: A total of 193 patients, 83 with APS (secondary APS, n = 45; primary APS, n = 38) and 110 without APS (systemic lupus erythematosus (SLE), n = 100; others, n = 10), were included in this study. The performance (sensitivity, specificity and area under the curve (AUC)) of the 2023 ACR/EULAR classification criteria for APS was evaluated and the agreement with the revised Sapporo criteria was compared using the kappa test. Results: In our cohort, the sensitivity and specificity of the 2023 ACR/EULAR classification criteria for APS were 73% and 94%, respectively (AUC: 0.836, 95% CI: 0.772–0.899), while the sensitivity and specificity of the revised Sapporo criteria were 66% and 98%, respectively (95% CI: 0.756–0.888). The performance of the two sets of criteria in our cohort was significantly consistent and significant (p < 0.001). When the sensitivity, specificity and ROC curve analysis were performed again by excluding livedo racemosa, the sensitivity of the new criteria in our cohort was 62% and the specificity was 100% (AUC: 0.813, 95% CI: 0.746–0.881). Conclusions: Although the newly published criteria broaden the scope of APS classification by including clinical findings other than thrombosis and obstetric criteria, their sensitivity in our cohort was low. On the other hand, we found that the specificity of the criteria in our cohort reached 100% when livedo findings were excluded. [ABSTRACT FROM AUTHOR]

Obliterative vasculopathies in dermatology represent a heterogeneous group of diseases with different pathogenesis. These are conditions of non-inflammatory occlusion of the vascular lumen with subsequently induced ischemia of the tissue (skin). In the current nomenclature, we also encounter the term cutaneous microvascular occlusive syndromes, which better characterize the involvement of skin vessels. Diseases that are responsible for occlusive syndromes include platelet pathology, the presence of cryoglobulins, infections affecting the vessel wall, embolisation (e.g. cholesterol), coagulopathy (antiphospholipid syndrome), or diseases accompanying chronic kidney failure (calciphylaxis). Possible skin manifestations include ulceration, necrotic lesions, livedo racemosa, and retiform purpura. The work provides a comprehensive view of the complex issue of these conditions and points to skin manifestations that are considered as manifestations of vasculitis in routine practice. [ABSTRACT FROM AUTHOR]

Deficiency of adenosine deaminase 2 (DADA2) is a childhood-onset disease known with pleiotropic clinical manifestations due to a monogenic form of systemic vasculopathy. Although the clinical spectrum of DADA2, linked to biallelic mutations in the ADA2 gene, has significantly expanded since 2014, approximately 200 cases have been reported thus far. In this report, two siblings are presented to draw attention to an unreported mutation of DADA2 and different phenotypic features of the same missense type homozygous mutation, p.Ser50Leu (c.149C>T). Next-generation sequencing demonstrated an autosomal recessive inherited missense type p.Ser50Leu (c.149C>T) variant homozygous mutation in the ADA-2 gene. The index patient is a 10-year-old Iraqi citizen boy who was referred to us with a complaint of anemia. The patient still needed blood transfusions after 2 hematopoietic stem cell transplantations. His older brother was an 11-year-old boy who presented to the emergency with a history of recurrent lung infections since 7 years of age. Considering the genotype-phenotype relationship from literature data, although vasculitis and low ADA2 activity are defined in cases with missense mutations, immuno-hematological manifestations are remarkable in our patients rather than vasculitic manifestations. While bone marrow failure findings e.g., anemia, neutropenia, etc. are reported to be seen in the 5th and 6th decades of life, those were predominant clinical features in our patients despite their younger age. DADA2 should also be kept in mind in cases of otherwise unexplained cytopenia, especially when associated with panhypogammaglobulinaemia and bone marrow hypocellularity, irrespective of the age of the patient at presentation. [ABSTRACT FROM AUTHOR]

Lymphocytic thrombophilic arteritis and livedoid vasculopathy may both present with livedo racemosa and ulceration. We present 6 cases with features of both conditions, raising the possibility that they are either closely linked or are part of a spectrum of the same condition. [ABSTRACT FROM AUTHOR]

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. Hypogammaglobulinemia that is found in many cases of DADA2 brings immunodeficiencies into the differential diagnosis. Cytopenia, pure red cell aplasia (PRCA), and bone marrow failure (BMF) are the hematologic abnormalities commonly found in DADA. Case presentation: We introduce eleven patients with DADA2 diagnosis, including two brothers and sisters, one set of twin sisters, and one father and his daughter and son. Ten patients (91%) had consanguineous parents. All the patients manifested livedo racemose/reticularis. Ten patients (91%) reported febrile episodes, and seven (64%) had experienced strokes. Only one patient had hypertension. Two of the patients (11%) presented decreased immunoglobulin levels. One of the patients presented with PRCA. Except for the PRCA patient with G321E mutation, all of our patients delivered G47R mutation, the most common mutation in DADA2 patients. Except for one patient who unfortunately passed away before the diagnosis was made and proper treatment was initiated, the other patients' symptoms are currently controlled; two of the patients presented with mild symptoms and are now being treated with colchicine, and the eight others responded well to anti-TNFs. The PRCA patient still suffers from hematologic abnormalities and is a candidate for a bone marrow transplant. Conclusions: Considering the manifestations and the differential diagnoses, DADA2 is not merely a rheumatologic disease, and introducing this disease to hematologists, neurologists, and immunologists is mandatory to initiate prompt and proper treatment. The efficacy of anti-TNFs in resolving the symptoms of DADA2 patients have been proven, but not for those with hematologic manifestations. Similarly, they were effective in controlling the symptoms of our cohort of patients, except for the one patient with cytopenia. [ABSTRACT FROM AUTHOR]

Background: Livedo is a well-known skin condition in patients with systemic lupus erythematosus (SLE) which correspond to small vessels involvement. The influence of antiphospholipid antibodies (aPL) on the occurrence of livedo is controversial. The aim of our study was to estimate the risk of livedo associated with aPL in patients with SLE. Methods: We conducted a systematic review and meta-analysis of the literature from 1977 to 2021 to estimate the risk of livedo in SLE patients according to different aPL profiles. Data sources were PubMed, Embase, Cochrane Library, hand search, and reference lists of studies. Studies were selected if they included SLE patients with descriptions of the exposure to aPL and the outcome (livedo). Two independent investigators assessed study eligibility, quality, and extracted patient characteristics from each study as well as exposure (aPL) and outcome (livedo). Risk estimates were pooled using random effects models and sensitivity analyses. For all stages of the meta-analysis, we followed the PRISMA guidelines. PROSPERO registration number: CRD42015027377. Results: Of the 2,355 articles identified, 27 were included with a total of 4,810 SLE patients. The frequency of livedo was 25.5% in aPL-positive patients and 13.3% in aPL-negative patients. The overall Odds Ratio (OR) for livedo in aPL-positive patients compared to aPL-negative patients was 2.91 (95% CI; 2.17–3.90). The risk of livedo was significantly increased for most of aPL subtypes, including lupus anticoagulant (LA) (OR = 4.45 [95% CI; 2.21–8.94]), IgG anticardiolipin (OR = 3.95 [95% CI; 2.34–6.65]), and IgG anti-β2-glycoprotein 1 (OR = 3.49 [95% CI; 1.68–7.27]). Conclusions: We demonstrated in this meta-analysis an excess risk of livedo in aPL-positive SLE patients compared to aPL-negative patients. For daily practice, in patients with SLE, livedo associated with aPL could correspond to a peculiar group of patients with small vessel disease. Livedo could be a good candidate for inclusion in future classification criteria for antiphospholipid syndrome. [ABSTRACT FROM AUTHOR]

Coronavirus disease (COVID-19) skin manifestations have been increasingly reported in medical literature. Recent discussions have identified a lack of images of skin of color (SOC) patients with COVID-19 related skin findings despite people with skin of color being disproportionately affected with the disease. There have been calls to prioritize the identification of COVID-19 skin manifestations in patients with SOC and disseminate these findings. The objective of this article is to review the existing literature on COVID-19 skin manifestations and, where possible, discuss how they may present differently in patients with SOC. Further research is needed to allow primary care physicians and dermatologists to be aware of and easily identify patients with cutaneous findings that may be secondary to COVID-19. Patients presenting with idiopathic dermatologic manifestations should be considered for COVID-19 testing and follow public health guidelines for self-isolation. [ABSTRACT FROM AUTHOR]

Livedo racemosa (LRac) refers to violaceous, red, or blue netlike mottling of the skin in an irregular pattern caused by circulatory abnormalities. Central venous catheters (CVC) are widely used in neonates who require intensive care to offer steady intravenous access. CVCs are covered with a hydrophilic polymer gel that reduces friction between the sheath and the vessel wall, thereby minimizing vascular spasm, irritation, and aneurysm formation. However, the introduction of foreign material into the vein carries a risk of embolization. Recently, LRac was reported as a cutaneous manifestation of hydrophilic polymer gel embolization (HPE) in adults. We present a case of LRac in a newborn that developed following CVC insertion and resolved spontaneously after the catheter was removed. [ABSTRACT FROM AUTHOR]

Background: The abundance of publications of COVID‐19‐induced chilblains has resulted in a confusing situation. Methods: This is a prospective single‐institution study from 15 March to 13 May 2020. Thirty‐two patients received PCR nasopharyngeal swabs. Of these, 28 patients had a thoracic CT‐scan, 31 patients had blood and urine examinations, 24 patients had skin biopsies including immunohistochemical and direct immunofluorescence studies, and four patients had electron microscopy. Results: COVID‐19‐induced chilblains are clinically and histopathologically identical to chilblains from other causes. Although intravascular thrombi are sometimes observed, no patient had a systemic coagulopathy or severe clinical course. The exhaustive clinical, radiological, and laboratory work‐up in this study ruled‐out other primary and secondary causes. Electron microscopy revealed rare, probable viral particles whose core and spikes measured from 120 to 133 nm within endothelium and eccrine glands in two cases. Conclusion: This study provides further clinicopathologic evidence of COVID‐19‐related chilblains. Negative PCR and antibody tests do not rule‐out infection. Chilblains represent a good prognosis, occurring later in the disease course. No systemic coagulopathy was identified in any patient. Patients presenting with acral lesions should be isolated, and chilblains should be distinguished from thrombotic lesions (livedo racemosa, retiform purpura, or ischemic acral necrosis). [ABSTRACT FROM AUTHOR]

Herein, we report a case-based review of the Sneddon Syndrome (SS), a rare chronic condition which affects small to medium blood vessels. It is known by its skin presentation, livedo racemosa (LRC), and the relapsing cerebrovascular events. However, neither LRC nor cerebrovascular events are exclusive to SS. A 36-year-old female with history of mitral valve prolapse, hypothyroidism, Raynaud phenomenon, hypertension, migraines, and four episodes of transient ischemic attacks (TIA), presented to our clinic with new skin findings, suggestive of LRC. Based on her previous history, current presentation and skin biopsy results, she was diagnosed with SS secondary to antiphospholipid syndrome. The present report illustrates the difficulty in recognizing SS and how the heterogeneity of the disease may be contributing to the difficulty making a distinct diagnosis. [ABSTRACT FROM AUTHOR]

Lymphocytic thrombophilic arteritis and Sneddon syndrome can have very similar clinical presentations with chronic persistent widespread blanchable livedo racemosa. Lymphocytic thrombophilic arteritis has only recently been described and generally is associated with a benign prognosis. Sneddon syndrome is associated with the development of multiple cerebrovascular accidents and progressive neurological impairment. We present three cases of Sneddon syndrome and compare them with lymphocytic thrombophilic arteritis to identify patients at risk of neurological events. [ABSTRACT FROM AUTHOR]

Livedo is a net‐like violaceous skin pattern. It can be classified as physiological or pathological. The physiological livedo reticularis usually appears in cold conditions, whereas the pathological and irregular livedo, which persists in warm temperatures, is often labeled as 'livedo racemosa'. Some neurological pathologies are associated with livedo, most commonly those with an inflammatory component or those derived from systemic disorders. The present review summarizes the most important central and peripheral neurological diseases in pediatric and adult age groups associated with livedo, providing physicians with an overview of the clinical presentation, etiology, diagnosis and management of these conditions. [ABSTRACT FROM AUTHOR]

Objective: To describe the clinical features, genotype, and treatment approaches of patients with confirmed adenosine deaminase 2 (ADA2) deficiency with dissimilar phenotypes. Methods: A case series of five DADA2 patients from three families was presented. The clinical and laboratory data, treatment protocols, and outcome of the patients were recorded from the patients' medical charts. ADA2 gene was screened by next generation sequencing first and then verified by Sanger sequencing. Serum ADA2 enzyme activity was measured by modified spectrophotometric method. Results: The median (min–max) age at onset of symptoms and age at diagnosis were 11 (9–13.8) years and 15 (9–19) years, respectively. The median (min–max) follow-up period was 8 (6–45) months. There was consanguinity in two families (2/3). The main clinical manifestations are musculoskeletal (5/5), dermatological (4/5), and neurological (2/5). Homozygosity for the p.G47R mutation in ADA2 gene was detected in three patients. A homozygous mutation in ADA2 gene (c.650 T > A; p.Val217Asp) was detected in two siblings. Plasma ADA2 enzymatic activity was absent in all patients. Anti-tumor necrosis factor (TNF) therapy was commenced, and all patients became clinically inactive with normal acute-phase reactants. Conclusion: ADA2 mutations should be checked in patients with presence of inflammation and livedoid vasculitis when they have neurological findings, especially in the form of stroke; and a history suggesting for an inherited disease; or presence of resistance to conventional treatment. Besides, anti-TNF seems to be useful for treatment of DADA2. [ABSTRACT FROM AUTHOR]

Sneddon syndrome (SS) is a rare medium-vessel vasculopathy which characteristically presents with livedo racemosa (LR) and complications such as strokes. This case report describes a female presenting acutely with a stroke and, initially, no evidence of LR. Her antiphospholipid antibodies were negative, and her neuroimaging revealed multiple territory strokes with extensive vasculopathy and fragile neo-formed vessel collateralisation. She had progressive memory loss and multiple transient ischaemic attacks on a background of established infarctions. SS should be considered in any idiopathic medium-vessel vasculopathy despite the absence of LR. Medical therapy can be challenging and the importance of antiphospholipid status in risk stratifying anticoagulation against antiplatelet therapy is discussed with a proposed rheumatology management strategy. The medical option of hydroxychloroquine should be considered in all patients in view of its anti-thrombotic properties and efficacy in diseases such as systemic lupus erythematosus and antiphospholipid syndrome with the suggestion that SS may be a forme fruste of these diseases. Neurosurgical options should be considered for recurrent transient neurological symptoms. For our patient, this included an extracranial to intracranial bypass via a radial artery graft for haemodynamic stroke management confirmed on SPECT imaging. The traditional hallmark of SS has previously been LR. This case highlights an atypical presentation stressing the importance of diagnostic vigilance in a patient with an idiopathic medium-vessel vasculopathy, together with balancing the medical risk of antiplatelet therapy, anticoagulation and thrombolysis whilst revealing possible neurosurgical options in select SS patients. [ABSTRACT FROM AUTHOR]

Abstract: We describe three patients who presented with a striking erythematous non‐blanching annular eruption and features of lymphocytic thrombophilic arteritis (LTA), with a prominent lymphocytic vasculitis involving deep dermal vessels. Lymphocytic inflammation was also evident in the superficial vessels and one patient had small superficial ulcers over the ankle area resembling livedoid vasculopathy (LV). Multiple biopsies demonstrated a persistent absence of neutrophils in the infiltrate consistent with a lymphocytic process. In addition to highlighting the annular morphology as a novel presentation of LTA, these cases suggest a possible relationship between LV and LTA and support the notion that they are distinct from neutrophilic vasculitides such as cutaneous polyarteritis nodosa. [ABSTRACT FROM AUTHOR]

Impairment of the protein C pathway, detectable by reduced plasma levels of activated protein C ( APC), are risk factors for venous thrombosis. Activated protein C maintains clotting homeostasis by regulation of pro-coagulant factors Va and VIIIa. Both infection and the factor V Leiden mutation reduce the formation of APC from protein C in the blood. With low levels of APC, excess factors Va and VIIIa exist, increasing the risk of thrombus formation. Livedo racemosa is characterised by a striking, violaceous branch-like pattering of the skin. It is similar to livedo reticularis, but with a different morphology and histopathology. In this case report we present the first case of livedo racemosa, in an 89-year-old factor V Leiden-positive patient with a Pseudomonas aeruginosa urinary tract infection. The cutaneous biopsies demonstrated vasculopathy with intraluminal thrombi in subcutaneous vessels with no evidence of inflammatory vasculitis. [ABSTRACT FROM AUTHOR]

Divry van Bogaert Syndrome (DBS) is a familial juvenile-onset disorder characterized by livedo racemosa, white matter disease, dementia, epilepsy and angiographic finding of “cerebral angiomatosis”. A similar syndrome including livedo racemosa and cerebrovascular disease, often associated with anticardiolipin antibodies, has been described as Sneddon Syndrome (SS) highlighting the question whether these two conditions have to be considered different entities or indeed different features of a unique syndrome. Herein, we report the clinical, neuroradiological, histopathological findings and follow up of three cases diagnosed as Divry-van Bogaert Syndrome, including an updated review of literature of both DBS and SS cases. Our findings support the assumption that DBS and SS are different disease entities. DBS is characterized by the typical angiographic feature of angiomatosis, a hereditary trait and a juvenile onset of cognitive impairment and leukoaraiosis, whereas SS has less severe manifestations of cerebrovascular disease associated with livedo racemosa but without the characteristic cerebral angiography. The report of our cases and the literature review underline the necessity of a detailed work-up and the collection of larger series to better clarify the DBS and SS phenotype and course. [ABSTRACT FROM AUTHOR]

Abstract: Lymphocytic thrombophilic arteritis (LTA) is a recently described entity defined by primary lymphocytic vasculitis; it typically has a chronic indolent course. We describe a patient who presented with clinical and histological findings consistent with LTA and later developed bilateral focal testicular infarcts as well as an acute median nerve neuropathy. [ABSTRACT FROM AUTHOR]

The antiphospholipid syndrome is an autoimmune, hypercoagulable state and is characterized clinically with vascular thrombosis or gravid morbidity, in association with persistent presence of antiphospholipid antibodies. Even though cutaneous manifestations are not considered diagnostic criteria, skin lesions in these patients have a prevalence of 49% and in 30.5 to 45% they are the initial sign of disease. This review describes the cutaneous manifestations associated with this disorder. [ABSTRACT FROM AUTHOR]

Livedo reticularis (LR) is a cutaneous physical sign characterized by transient or persistent, blotchy, reddish-blue to purple, net-like cyanotic pattern. LR is a benign disorder affecting mainly middle-aged females, whereas livedo racemosa (LRC) is pathologic, commonly associated with antiphospholipid antibody syndrome. This article aims to review the causes of LR and LRC along with the evaluation and management. [ABSTRACT FROM AUTHOR]

A 55‑year‑old woman presented with a 5‑year history of livedo racemosa on her limbs. Histology showed vasculitis of medium‑sized arteries with a circumferential, hyalinised, intraluminal fibrin ring. Her laboratory investigations did not indicate any underlying systemic disease. The findings were consistent with lymphocytic thrombophilic arteritis (LTA), alias macular arteritis, which is a recently described entity. The importance of LTA lies in the fact that it is a close clinical and microscopic mimic of polyarteritis nodosa (PAN). LTA is believed to be a distinct entity by some and as a form of PAN by others. We have discussed this case in our report. [ABSTRACT FROM AUTHOR]

The article presents a case study of a 70-year-old male referred for management of lipodermatoscerosis and progressive pigmentation of lower limbs. Examinations showed the skin eruption as livedo racemosa and related subcutaneous nodularity and induration to be because of panniculitis ossificans. Biopsy of the small saphenous vein (SSV) revealed segmental heterotopic ossification.

The skin is a common target organ in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. Skin lesions may present as the initial manifestation or as the associated cutaneous manifestations in ANCA-associated vasculitis. Visually assessing the cutaneous manifestations and performing a biopsy of skin lesions to facilitate histopathologic confirmation are highly valuable diagnostically. Vasculitis (which affects dermal small vessels and subcutaneous muscular vessels) and nonvasculitic inflammation cause various types of skin lesions. The coexistence of different levels of vasculitis and nonvasculitis can occur in one or in several lesions or on different occasions. These puzzling clinical and histopathologic features may be closely related to the disease activity. Understanding the complicated clinical and histopathologic spectrum of skin lesions will contribute to the early diagnosis of ANCA-associated vasculitis with cutaneous complications. [ABSTRACT FROM AUTHOR]

ABSTRACT A 35-year-old Lebanese woman presented with a 3-year history of persistent, localized livedo racemosa over her feet, distal legs and forearms that was associated with the development of lower limb sensorimotor neuropathy. Investigations revealed the patient was heterozygous for prothrombin gene mutation and was also found to have a T-cell receptor gamma chain gene rearrangement. Histological examination revealed a mid-lower dermal medium vessel lymphocytic vasculitis with prominent fibrinoid ring within its wall. These findings are consistent with a recently described condition known as lymphocytic thrombophilic arteritis. This has so far been considered to be a benign clinical condition not associated with extra cutaneous manifestations. The novel findings in the present case are the associated sensorimotor neuropathy and the characteristic fibrin ring appears to be intramural rather intraluminal in location. The findings of a T cell gene rearrangement and a prothombin gene mutation suggest that both immunological and thrombophilic factors might contribute to the development of this condition. [ABSTRACT FROM AUTHOR]

Terminology concerning livedo reticularis and livedo racemosa is not uniformly determined due to inconsistent classification in the past and to the use of different terms in different countries and medical specialties. The authors give a summary of actual knowledge about the physiology and pathophysiology of skin vascularization, about the main units of vascular supply, and angiosomes which give rise to the appearance of reticulate eruptions. They review the etiopathogenetic factors as well as the relationship of reticulate eruptions, inclusive retiform purpura to underlying systemic disorders. Practical approach to the diagnotics of reticulate eruptions is presented. [ABSTRACT FROM AUTHOR]

Sneddon syndrome (SS) is rare, arterio-occlusive disorder characterized by generalized livedo racemosa of the skin and various central nervous symptoms due to occlusion of medium-sized arteries of unknown. Seizure, cognitive impairment, hypertension, and history of repetitive miscarriages are the other symptoms seen in this disease. Livedo racemosa involves persisting irreversible skin lesions red or blue in color with irregular margins. Usually, SS occurs in women of childbearing age. Protein S deficiency is an inherited or acquired disorder associated with an increased risk of thrombosis. We present a 33-year-old woman with SS with diffuse livedo racemosa, recurrent cerebrovascular diseases, migraine-type headache, sinus vein thrombosis, and protein S deficiency. Protein S deficiency and with Sneddon syndrome rarely encountered in the literature. [ABSTRACT FROM AUTHOR]

ABSTRACT Reticulate pattern is one of the most important dermatological signs of a pathological process involving the superficial vascular networks. Vascular malformations, such as cutis marmorata congenita telangiectasia and benign forms of livedo reticularis, and sinister conditions, such as meningococcal meningitis or Sneddon's syndrome, can all present with a reticulate pattern. The clinical presentation and morphology is determined by the nature and extent of the underlying pathology and the involvement of a particular vascular network. This review has been divided into four instalments. In the present paper, we discuss the anatomy and physiology of the complex network of vascular structures that support the function of the skin and subcutis. [ABSTRACT FROM AUTHOR]

Livedo is a cutaneous sign of striking violaceous netlike patterned erythema of the skin. This dermatological phenomenon is of special interest in the differential diagnosis in neurological patients. In 1907 Ehrmann distinguished two different patterns of livedo: the pathological livedo racemosa and the physiological livedo reticularis. Despite important clinical differences, in the English language literature the heading livedo reticularis is still used for all types of livedo. A literature review about the spectrum of differential diagnosis in patients with livedo reticularis (especially cutis marmorata and amantadine–induced livedo reticularis) and livedo racemosa (especially Sneddon’s syndrome, Divry–van Bogaert syndrome, systemic lupus erythematosus, antiphospholipid antibody syndrome, polyarteritis nodosa, cholesterol embolization syndrome, livedoid vasculopathy and haematological diseases) is provided. [ABSTRACT FROM AUTHOR]

A 20-year-old woman was hospitalized repeatedly because of intermittent bouts of intestinal obstruction and the symptoms usually improved with conservative treatments. One year after the first admission the patient was hospitalized in emergency and a laparotomy revealed a circular stricture with a pinhole perforation in the ileum. Histological sections of the stricture showed the characteristic features of microscopic polyangiitis varying from active to resolving stages, which were localized in the ileum. Fibrinoid necrosis, fibroblastic and fibrous proliferation of the intima and fibrous replacement of the media with a variable pan- and perivascular inflammatory cell infiltrate were characteristic in the muscular arteries and arterioles. Vascular occlusion by pale eosinophilic, fibrillar-like materials resembling livedo racemosa of the skin, was noticed in the small arterioles and capillaries. Under no prophylaxis, the postoperative course was uneventful with no recurrence of the illness at an 18-month follow up. The pathological alterations were distributed focally, occasionally segmentally, and haphazardly, and required detailed examination by stepwise sections for the histological diagnosis. [ABSTRACT FROM AUTHOR]

Summary Independent of the size of the dog and the type of injury, serious infections may follow a dog bite and these may result in the abrupt onset of multiorgan failure. Early recognition of the warning signs with regard to the underlying severity of the infection is of the utmost importance. Reticulate skin eruptions constitute a precursory phenomenon. [ABSTRACT FROM AUTHOR]