A 56-Year-Old Man With Emphysema, Rash, and Arthralgia.

CASE PRESENTATION: A 56-year-old man presented to the pulmonary clinic with dyspnea and hypoxemia on exertion. He was an avid biker and skier who had noticed a significant decrease in high-level physical activity over the past 3 years. He reported dyspnea, desaturations at altitudes higher than 9,000 feet, dry cough, tachycardia, and palpitations with exercise. Review of systems was also notable for gluten-intolerance, Raynaud's phenomenon, recurrent skin lesions and joint swelling, pain, and stiffness in the areas overlying the jaw, wrists, knees, and ankles (after capsaicin exposure). He denied fever, chills, anorexia, weight loss, hair loss, ocular symptoms, jaw claudication, chest pain, or lower extremity swelling. He had a five pack-year smoking history, no history of prematurity, childhood asthma, recurrent infections, or environmental and occupational exposure. Based on pulmonary function tests from an outside provider, he had received a diagnosis of exercise-induced asthma and had been prescribed an albuterol inhaler to use on an as-needed basis, which failed to improve his symptoms. He was later prescribed a mometasone-formoterol inhaler, still with no symptomatic improvement.

Diagnóstico e terapêutica precoce de vasculite leucocitoclástica: relato de caso / Early diagnosis and treatment of Leukocytoclastic Vasculitis: case report

Resumo Paciente do sexo feminino, 46 anos de idade, procurou o pronto socorro do Hospital Municipal Universitário apresentando lesões necróticas em membros inferiores associadas à síndrome consumptiva. Após anamnese e exame físico, obteve-se o diagnóstico de vasculite leucocitoclástica de modo imediato e econômico por meio da utilização de algoritmo específico de vasculites primárias, permitindo início precoce da terapêutica adequada. A boa evolução do quadro clínico ratificou a necessidade de se obter diagnóstico definitivo e início rápido da terapêutica.

Abstract A 46-year-old female patient presented at the emergency department of a Municipal University Hospital with necrotic lesions in lower limbs associated with wasting syndrome. She was diagnosed with leukocytoclastic vasculitis after physical examination and history-taking in a fast and cost-effective manner, using an algorithm specifically for primary vasculitis, enabling early and appropriate treatment. The good clinical outcome demonstrates the need to quickly make a definitive diagnosis and start treatment.

Multiple myeloma presenting as cutaneous leukocytoclastic vasculitis and eosinophilia disclosing a T helper type 1/T helper type 2 imbalance: a case report.

BACKGROUND: Multiple myeloma is a very heterogeneous disease comprising a number of genetic entities that differ from each other in their evolution, mode of presentation, response to therapy, and prognosis. Due to its more chronic nature and cumulative toxicities that patients develop from multiple lines of treatments, a number of symptoms are associated with multiple myeloma. However, the mechanisms responsible for the relationship between these symptoms and multiple myeloma currently remain unclear. CASE PRESENTATION: An 85-year-old Japanese woman exhibited the rare presentation of multiple myeloma (immunoglobulin kappa chain type) with leukocytoclastic vasculitis and eosinophilia. The serum level of interferon-γ was decreased; however, serum levels of interleukin-4, interleukin-5, interleukin-6, interleukin-10, and tumor growth factor-ß levels were elevated. She received a bortezomib, lenalidomide, and dexamethasone regimen. After one course of the treatment, the cutaneous manifestation rapidly improved and laboratory tests showed decrease of eosinophil cell count. Serum concentrations of immunoglobulin G decreased and plasma cells in bone marrow decreased. The serum level of interferon-γ was elevated and serum levels of interleukin-4, interleukin-5, interleukin-6, interleukin-10, and tumor growth factor-ß decreased. CONCLUSIONS: It is the first case of leukocytoclastic vasculitis and eosinophilia in multiple myeloma that was associated with a T helper type 1/T helper type 2 imbalance and T regulatory cells, and was successfully treated with bortezomib, lenalidomide, and dexamethasone. The present case reinforces the value of early evaluations for paraneoplastic symptoms in order to reach a diagnosis and allow for the prompt initiation of appropriate treatments and achieve successful therapeutic management.

Cutaneous hemophagocytosis: Clinicopathologic features of 21 cases.

BACKGROUND: Hemophagocytosis is well known in cytotoxic cutaneous T-cell lymphomas (CTCLs), in which it may represent a sign of hemophagocytic lymphohistiocytosis syndrome (HLHS), and is also typical of cutaneous Rosai-Dorfman disease (cRDD) (without prognostic relevance). Only rarely, has cutaneous hemophagocytosis (CH) been described in other skin conditions. OBJECTIVE: To characterize the clinicopathologic features of CH in skin biopsy specimens from patients with conditions other than CTCL or cRDD. METHODS: Case series analyzing clinicopathologic features and follow-up data on patients presenting with histopathologic signs of CH. RESULTS: Biopsy specimens from 21 patients were included. None of the patients had HLHS. The majority (n = 11) presented with leukocytoclastic vasculitis. Other associated diseases were lupus erythematous (n = 2), arthropod bite reaction (n = 2), erysipelas (n = 1), acne conglobata (n = 1), and Sweet syndrome (n = 1). Three patients had a nonspecific rash concomitant with Chlamydia pneumonia, middle ear infection, and pharyngitis, respectively. LIMITATIONS: This was a single-center, retrospective study. CONCLUSION: Isolated CH in conditions other than CTCL and cRDD is a histopathologic finding related mostly to leukocytoclastic vasculitis. Extensive investigations should be performed only if patients have other signs or symptoms of HLHS.

Cutaneous and systemic vasculitides in dermatology: a histological perspective.

Cutaneous vasculitides encompass a wide and heterogeneous group of diseases affecting skin blood vessels that are clinically characterized by polymorphic skin lesions, particularly including palpable purpura as well as urticarial and necrotic-ulcerative lesions, with possible, albeit rare, extracutaneous involvement. Cutaneous leukocytoclastic angiitis and urticarial vasculitis, which are the two prototypic and most common variants of this group, are usually idiopathic but may also be induced by different triggers, notably drugs and infections, or may manifest in association with systemic disorders, particularly lupus erythematosus. Vasculitis skin lesions can also occur during the chronic-relapsing course of systemic vasculitides, such as granulomatosis with polyangiitis and eosinophilic granulomatosis with polyangiitis, with which cutaneous vasculitides can share clinical and histological features. In order to make the diagnosis of a specific form of vasculitis, the cutaneous picture and clinical history of the patient have to be correlated with the histological pattern, direct immunofluorescence findings, laboratory data and possible presence of extracutaneous manifestations. Histology is pivotal for the diagnosis of vasculitis and timing of the biopsy is relevant to correctly evaluate the characteristics of the inflammatory infiltrate. In this review, we will focus in particular on the histological features of cutaneous vasculitides in order to differentiate them from the systemic forms and to avoid misdiagnosis when skin involvement is the presenting sign of a multisystem vasculitis.

[Cutaneous leukocytoclastic vasculitis: about 85 cases]. / Vascularites cutanées leucocytoclasiques: à propos de 85 cas.

Clinical manifestation, etiology and outcome of leukocytoclastic vasculitis are little studied. The aim of our study was to examine epidemiological, clinical etiological, and evolutionary characteristics of this entity. We conducted a cross-sectional data collection from medical records of 85 patients with leukocytoclastic vasculitis in the Department of Dermatology at the Farhat Hached University Hospital, Sousse between January 2000 and December 2013. Epidemiological, clinical, paraclinical, etiological data sheets had been completed for each patient. The average age of patients was 47.65 years, ranging between 10 and 78 years. Fifty-three women and 32 men were registered (sex ratio = 0.6). Cutaneous manifestations were dominated by vascular purpura (88.2%). The most common causes of leukocytoclastic vasculitis were systemic diseases (51%), infection (20%) and neutrophilic dermatoses (14.5%). Other causes were drugs (9.1%) and hematologic malignancies (5.4%). The cause of leukocytoclastic vasculitis was not detected in 30 patients (35, 3%). Two predictive factors associated with the acute outcome were retained: the presence of a recent infection (p= 0.014) and drug intake before the rash (p= 0.013). Chronic evolution was positively correlated with antinuclear antibodies (p= 0.009) and cryoglobulinemia (p=0.025). Our study highlights the multitude of causes of leukocytoclastic vasculitis. The search for an underlying disease is an imperative in order to ensure better therapeutic management.

ANCA-Negative Churg-Strauss Syndrome Presenting as Acute Multiple Cerebral Infarcts: A Case Report.

Eosinophilic granulomatosis with polyangiitis (EGPA, previously named Churg-Strauss syndrome) is a form of necrotizing vasculitis occurring in patients with asthma and eosinophilia. Ischemic stroke is a relatively rare complication of the disease. We report a case of a 63-year-old woman with multiple embolic infarcts, hypereosinophilia (for >7 years), and skin rash. Elevated cardiac enzymes and cardiac magnetic resonance imaging were consistent with endomyocarditis. The simultaneous presence of history of asthma, sinusitis, hypereosinophilia, and vasculitis led to the diagnosis of EGPA. This case contributes to the recent debate of the 2 possible presentations of the disease according to the ANCA (antineutrophil cytoplasmic antibodies) status. We furthermore underscore the need for careful differential diagnosis of the "ANCA negative" cases with persistent hypereosinophilia from the idiopathic hypereosinophilic syndrome.

A not-so-sweet diagnosis - leukocytoclastic vasculitis masquerading as squamous cell carcinoma.

Leukocytoclastic vasculitis (LCV) is a rare small-vessel vasculitis characterised by neutrophilic inflammation of post-capillary venules. Incidence varies from 3 to 4.5 per 100 000 people per year. Patients typically present with painful, itchy purpura and erythema, although clinical manifestations can vary making diagnosis a challenge. We report the case of a 75-year-old man with a history of a previously completely excised and grafted squamous cell carcinoma (SCC) on the dorsum of his hand, who presented with an acutely swollen, erythematous and ulcerated lesion adjacent to the graft site. A shave biopsy failed to definitively exclude SCC recurrence. He was referred to the Plastics team who initially suspected Sweet's syndrome but could not rule out SCC recurrence. The patient underwent formal mapping incisional biopsies that later diagnosed LCV. He was managed conservatively and made an excellent recovery. We present clinical photographs and histology to illustrate disease progression. LCV is typically self-limiting with a good overall prognosis, but a minority of patients follow a protracted course, which may require treatment in the form of systemic corticosteroids or colchicine. LCV can only be confirmed histologically. We present this case in order to highlight the importance of adequate tissue biopsy when there is diagnostic uncertainty with an acute dermatosis, particularly in the context of previous skin malignancy.

Urticarial vasculitis in the childhood with C2 hypocomplementenemia: a rare case.

We report a first case of hypocomplementemic urticarial vasculitis of C2 fraction in a child, with cutaneous manifestation only, with no reports in scientific literature.

Autoinflammatory associated vasculitis.

Autoinflammatory diseases are characterized by recurrent episodes of fever and localized or systemic inflammation and are caused by monogenic defects of innate immunity. The skin is commonly involved with various manifestations including erysipelas like rash and urticaria. Although vasculitis has been described in many autoinflammatory diseases, it has not been recognized as a characteristic feature of these diseases and autoinflammatory diseases are not listed as an etiology for vasculitis associated with a systemic disease in the 2012 Revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. We describe herein 3 patients with different autoinflammatory diseases in whom leukocytoclastic vasculitis was one of the major and presenting symptoms. A review of the vast evidence in the literature for vasculitis in the spectrum of autoinflammatory diseases and a suggested pathophysiology is presented. We suggest the term autoinflammatory associated vasculitis to describe vasculitis associated with autoinflammatory diseases. Autoinflammatory diseases should be considered within the differential diagnosis of vasculitis.

Escherichia coli Urinary Infection as a Cause of Acute Hemorrhagic Edema in Infancy.

Acute hemorrhagic edema of infancy (AHEI) is a benign leukocytoclastic small-vessel vasculitis that affects infants, presenting with a classic clinical triad. Because of the self-limited progression, conservative management is the most common approach. We describe a case of AHEI apparently triggered by an Escherichia coli urinary tract infection.

Vasculitis leucocitoclástica / Leukocytoclastic vasculitis

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Drug-induced cutaneous vasculitides.

Cutaneous vasculitides (CV) can be idiopathic or secondary to several triggers, including drugs, which account for up to 30% of all the cases of CV. Several drugs can induce CV, including some medications commonly used in dermatology, including minocycline, and several new drugs, such as anti-TNF agents. Different pathomecanisms are involved in the development of drug-induced CV, including the formation and deposition of immune complexes, the induction of neutrophil apoptosis, the formation of neoantigens between the drugs and proteins from the host, the shift of the immune response, and others. Although the diagnosis is difficult, because the clinical picture of drug-induced CV is in general indistinguishable from that of other forms of CV, it is important to recognize such entities in order to correctly manage the patient. Anamnesis, diagnostic algorithms to assess the likelihood of the association between a drug and a cutaneous reaction, skin biopsy and laboratory testing (including the search for antineutrophil cytoplasmic antibodies) are useful tools to make a diagnosis of drug-induced CV. About the therapy, while in idiopathic vasculitides the treatment is usually more aggressive and long-lasting, very often requiring a maintenance therapy with immunosuppressive drugs, in drug-induced CV the discontinuation of the suspected drug alone is usually enough to achieve complete remission, making the prognosis usually very good.

Revisiting clinical differences between hypersensitivity vasculitis and Henoch-Schönlein purpura in adults from a defined population.

OBJECTIVES: Hypersensitivity vasculitis (HV) and Henoch-Schönlein purpura (HSP) are the most common entities included within the category of cutaneous vasculitis (CV). Palpable purpura and histological changes characterised by the presence of leukocytoclastic vasculitis are common in both conditions. Therefore, considerable overlap between them is often seen. It is especially true when the CV occurs in adults. To further establish clinical differences between these two conditions, in the present study we assessed the main clinical differences between HV and HSP in a wide and unselected series of adults with CV from a defined population. METHODS: We reviewed the clinical records of 297 consecutive adults (age >20 years) seen at a single centre between January 1975 and December 2012 that were classified as having HSP or HV according to the criteria proposed by Michel et al. (J Rheumatol 1992; 19: 721-8). RESULTS: Based on the inclusion criteria, 102 adult patients (71 men/31 women) were classified as HSP and 195 (104 men/91 women) as HV. The mean age was similar in both groups (55.8±16.5 years in HSP and 56.8±18.3 years in HV). Precipitating events, usually an upper respiratory tract infection and/or drug intake, were more frequently observed in HV. Both at the beginning of the disease and when the CV was established clinical manifestations were more frequent in patients with HSP than in those with HV. It was the case for gastrointestinal (57.4% vs. 6.8%; p<0.001), joint (51.5% vs. 36.6%; p=0.01) and renal involvement (86.3% vs. 18.3%; p<0.001). Corticosteroid (56.7% vs. 22%; p<0.001) and cytotoxic drug (19.4% vs. 3.2%; p<0.001) use was also more common in patients with HSP. After a median follow-up of 15.5 (interquartile range- IQR; 3-37) months in HSP and 4 (IQR; 2-12) months in HV, the outcome was better in HV than in HSP. In this regard, complete recovery (72.6% vs. 85.4%; p=0.01) was more commonly observed in HV while residual renal involvement (15.3% vs. 4.2%; p<0.001) was more common in HSP. The disease relapsed in 35.3% of patients with HSP and in 24.4% with HV (p=0.07). CONCLUSIONS: Our results confirm the claim that these two diseases presenting with similar cutaneous involvement are certainly two separate entities with greater systemic involvement and less favourable outcome in HSP.

[Exercise induced vasculitis].

Exercise induced vasculitis (or purpura) is usually misdiagnosed and ignored in the literature, although it is not uncommon. We report two female patients who developed a rash on the lower legs after walking all day long. The rash was red, itchy with a burning sensation. The lesions resolved after a few days with and without steroid treatment. Allergic etiology was suspected, therefore they were sent to the allergy clinic for evaluation. Exercise-induced vasculitis tends to occur in healthy people, especially in hot weather. Extensive investigation with blood or allergy tests is not needed.

Vasculitis cutánea leucocitoclástica y glomeruesclerosis focal y segmentaria con formación de semi lunas en una joven consumidora de cocaína/crack cortada con levamisol V / Cutaneous leukocytoclastic vasculitis and focal segmental glomeruesclerosis with crescent formation in a young consumer of cocaine / crack cut with levamisole V

Se describen los hallazgos anatomopatológicos encontrados en la autopsia de una joven de 19 años de edad, indigente, trabajadora del sexo, adicta al crack desde los 12 años de edad, quien los últimos 4 meses de su vida tuvo tres ingresos hospitalarios al Hospital México de San José‚ de Costa Rica con diagnóstico de vasculitis cutánea por crack e insuficiencia renal aguda. Los hallazgos más relevantes en la autopsia fueron: vasculitis aguda leucocitoclástica con trombosis y paniculitis glomeruloesclerosis focal y segmentaria con formación de semilunas...

Evidence-based evaluation and management of chronic urticaria in children.

Urticaria affects nearly 25% of the population at some time in their lives. In a subset of children, urticaria will develop into a chronic condition that can greatly affect quality of life. Although numerous causes and triggers are proposed for chronic urticaria (CU) in children, ranging from infections, allergens, and medications to physical factors and autoimmune disease, the exact etiology is not always identifiable. Accordingly, a large subset of cases are designated "chronic idiopathic urticaria." Because of the clinical complexities of CU, as well as the confusing literature on this topic, we have developed a conceptual framework based on the cumulative evidence to assist with the categorization, clinical evaluation, and treatment of CU in children.