Reporte de caso: vasculitis leucocitoclastica como forma de presentación de hepatitis b crónica / Case report: leukocytoclastic vasculitis as presenting manifestation of chronic hepatitis b virus infection

La vasculitis leucocitoclástica, también denominada angeitis cutánea leucocitoclástica, es la forma más común de vasculitis. Si bien la mayoría de los casos son idiopáticos, entre los agentes etiológicos que podemos nombrar se encuentran los agentes infecciosos, las enfermedades del tejido conectivos, las reacciones de hipersensibilidad a medicamentos y las neoplasias solidas o hematológicas. Si bien los procesos infecciosos son una causa conocida de vasculitis leucocitoclástica, la infección por virus de Virus de hepatitis B (VHB) es muy infrecuente. Presentamos una mujer de 47 años, sin antecedentes patológicos previos, que consultó por artralgias en rodillas y tobillos, mialgias en gemelos y rash purpúrico con leve prurito en ambos miembros inferiores, de un mes de evolución. La biopsia cutánea de las lesiones de miembros inferiores fue compatible con vasculitis leucocitoclástica. La serología de hepatitis B fue positiva por lo que inició tratamiento antiviral con Tenofovir y Prednisona con buena evolución de sus lesiones cutáneas

Leukocytoclastic vasculitis, also called leukocytoclastic cutaneous angiitis, is the most common form of vasculitis. Although most cases are idiopathic, etiologic agents include infectious agents, connective tissue diseases, drug hypersensitivity reactions, and solid or hematologic malignancies. Although infectious processes are a known cause of leukocytoclastic vasculitis, hepatitis B virus (HBV) infection is very rare. We present a 47-year-old woman, with no previous pathologic history, who consulted for arthralgias in the knees and ankles, myalgia's and purpuric rash with mild pruritus in both lower limbs, of one month evolution. Skin biopsy of lower extremity lesions was compatible with leukocytoclastic vasculitis. Hepatitis B serology was positive, so she started antiviral treatment with tenofovir and prednisone with good evolution of her skin lesions

[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.

Palpable purpura: is it associated with vasculitis or not? A single-center experience.

BACKGROUND AND AIM: The most common cutaneous manifestation of small vessel vasculitis is palpable purpura. Etiology includes various causes such as infections, malignancies, drugs, and systemic vasculitides. The number of studies that evaluated the etiology of patients presenting with palpable purpura in the adult age group is minimal. This study aimed to determine the etiology in patients presenting with palpable purpura and analyze the clinical features associated with this pathology. MATERIALS AND METHODS: We included 85 patients over 18 years old who presented with palpable purpura in the study. The presenting demographic characteristics, medical history, systemic examination findings, laboratory, imaging, and histopathological results, and initial treatment of the patients were recorded. At the end of data collection, statistical analyses were performed to determine the patients' final diagnoses and organ involvement. RESULTS: Etiological evaluation revealed Ig A vasculitis (IgAV) in 58.8% (n = 50) of the cases, cutaneous leukocytoclastic vasculitis (CLV) in 23.5% (n = 20), and ANCA-associated vasculitis (AAV) in 3.5% (n = 3). Rheumatologic disease-associated vasculitis (RDaV) was detected in 7.1% (n = 6) of the patients. In 7.1% (n = 6) of the patients, the biopsy results were not compatible with vasculitis (NVH). DISCUSSION: Palpable purpura can occur due to many reasons. Ig A vasculitis was the most common cause of palpable purpura in our study. KEY POINTS: • All clinicians should recognize and know palpable purpura and its differential diagnosis. • IgA vasculitis is the most common cause of palpable purpura in adult patients. • In our study, Anca-associated vasculitis (AAV) was found in 3 (3%) of 85 adult patients with palpable purpura.

Autologous platelet-rich plasma in the treatment of refractory wounds in cutaneous leukocytoclastic vasculitis complicated with hypertension (grade 2 moderate risk): A case report.

INTRODUCTION: Cutaneous leukocytoclastic vasculitis is an inflammatory variant of vasculitis with a variety of causes that only affects the skin. Its pathological manifestations include neutrophil infiltration and nuclear fragmentation. Clinically, it is characterised by a pleomorphic rash, including erythema, purpuric skin lesions, reticulocytosis, necrosis and ulceration. Once formed, local ulcerations are very difficult to heal. CASE PRESENTATION: A 46-year-old female was diagnosed with cutaneous leukocytoclastic vasculitis. The patient's legs exhibited ulcers with a black eschar on the surface. The largest wound was 4.5 × 4.0 cm and the deepest wound was 1.7 × 1.8 × 1.0 cm. The ulcers had been present for 6 months and did not exhibit signs of healing. Treatment was commenced with platelet-rich plasma, and the wounds healed within 1 month. CONCLUSION: Topical application of autologous platelet-rich plasma gel exerts beneficial effects in cutaneous leukocytoclastic vasculitis with regard to wound size reduction, and it induces granulation tissue formation. Platelet-rich plasma may represent a safe and cost-effective treatment for managing cutaneous wound healing to reduce the length of the recovery period.

Diagnosis and management of leukocytoclastic vasculitis.

Leukocytoclastic vasculitis (LCV) is a histopathologic description of a common form of small vessel vasculitis (SVV), that can be found in various types of vasculitis affecting the skin and internal organs. The leading clinical presentation of LCV is palpable purpura and the diagnosis relies on histopathological examination, in which the inflammatory infiltrate is composed of neutrophils with fibrinoid necrosis and disintegration of nuclei into fragments ("leukocytoclasia"). Several medications can cause LCV, as well as infections, or malignancy. Among systemic diseases, the most frequently associated with LCV are ANCA-associated vasculitides, connective tissue diseases, cryoglobulinemic vasculitis, IgA vasculitis (formerly known as Henoch-Schonlein purpura) and hypocomplementemic urticarial vasculitis (HUV). When LCV is suspected, an extensive workout is usually necessary to determine whether the process is skin-limited, or expression of a systemic vasculitis or disease. A comprehensive history and detailed physical examination must be performed; platelet count, renal function and urinalysis, serological tests for hepatitis B and C viruses, autoantibodies (anti-nuclear antibodies and anti-neutrophil cytoplasmic antibodies), complement fractions and IgA staining in biopsy specimens are part of the usual workout of LCV. The treatment is mainly focused on symptom management, based on rest (avoiding standing or walking), low dose corticosteroids, colchicine or different unproven therapies, if skin-limited. When a medication is the cause, the prognosis is favorable and the discontinuation of the culprit drug is usually resolutive. Conversely, when a systemic vasculitis is the cause of LCV, higher doses of corticosteroids or immunosuppressive agents are required, according to the severity of organ involvement and the underlying associated disease.

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.

Colloidal Silver Ingestion Associated with Leukocytoclastic Vasculitis in an Adolescent Female.

BACKGROUND Leukocytoclastic vasculitis is a disease of the small vessels and is uncommon in children. In this case report, we present an adolescent case of leukocytoclastic vasculitis associated with the ingestion of colloidal silver, a naturopathic drug. This report highlights the rarity of the patient's presentation and inducing agent. CASE REPORT A 19-year-old female presented in the Emergency Department with severe rash on the face, and neck, and then continued to spread in a craniocaudal fashion during the day of presentation to involve trunk, back, upper and lower extremities. There was no recent travel, no pets and a negative family history for rheumatologic or autoimmune diseases. Her home medications included colloidal silver for "internal cleansing" for 4 weeks prior to Emergency Department presentation. Once the clinicians were aware of the continued ingestion of colloidal silver, the patient was advised to discontinue the drug. The patient was started on methylprednisolone with preliminary diagnosis of vasculitis, as well as concurrent therapy with colchicine. The rash was noted to be receding from the face within 24 hours. Over a hospital course of 5 days, the patient's rash and pruritus continued to slowly improve. CONCLUSIONS The ingestion of a naturopathic drug, colloidal silver, caused vast leukocytoclastic vasculitis in our patient warranting hospitalization due to the extent of the disease. The symptoms resolved after discontinuation of colloidal silver ingestion. Due to unknown safe ingestion concentrations and potential side effects, use of colloidal silver should be discouraged.

Acute hemorrhagic edema of infancy: the experience of a large tertiary pediatric center in Israel.

BACKGROUND: Acute hemorrhagic edema of infancy (AHEI) is a rare leukocytoclastic vasculitis of the small vessels occurring at a young age and considered as a benign self-limited disease. Due to its low prevalence, there are limited data on the presentation and complications of this disease. METHODS: All computerized files of children who were hospitalized at a tertiary pediatric center due to AHEI over a 10 year period were reviewed. Clinical, laboratory and histopathological data were collected. RESULTS: Twenty-six patients were included in our study, accounting for 0.7 cases per 1000 admissions of children aged 2 years or less. Mean age was 12.9 months. More than two thirds of the children had preceding symptoms compatible with a viral infection. Upon admission, all patients presented with typical findings of a rash and edema. Edema was most profound over the lower extremities (73%). Concomitant viral or bacterial infections were found in six children. Skin biopsy was performed in six patients revealing leukocytoclastic vasculitis. Thirteen children (50%) had systemic involvement including joint involvement (n=9), gastrointestinal hemorrhage (n=4), microscopic hematuria (n=1) and compartment syndrome of the limb (n=1). The latter was diagnosed in a patient with familial Mediterranean fever. CONCLUSIONS: Our largest data series highlighted what is known regarding clinical and histological findings in children with AHEI. However, contrary to what was previously reported, we found a higher rate of systemic involvement. Although AHEI is a rare entity, pediatricians should be familiar with its presentation, management and our reported complications.

Clinical study on single-organ cutaneous small vessels vasculitis (SoCSVV).

Leukocytoclastic vasculitis (LCV) is a heterogenous group of disorders that may manifest as a mild disease isolated to the skin or be a part of life-threatening systemic vasculitis. According to the 2012 Chapel Hill Consensus Conference nomenclature, patients presenting symptoms of LCV confined only to the skin should be defined as suffering from a single-organ cutaneous small vessel vasculitis (SoCSVV). SoCSVV is a benign disease with a good clinical outcome but with a significant risk of relapse and skin ulcer formation.The aim of the current study was to characterize SoCSVV and to identify factors that may be associated with the risk of recurrence and skin ulcers.Medical records of patients with LCV hospitalized at the Department of Dermatology at University Hospital in Cracow in the years 2010 to 2015 were analyzed.A total of 24 patients fulfilled criteria of SoCSVV. Drugs and preceding infections were identified as precipitating factors in 40% and 20% of cases, respectively. Skin lesions other than palpable purpura (i.e., macules, urticarial vasculitis, or ulcers) were identified in almost half of the patients. Interestingly, the presence of macules independently increased the risk of skin ulcer formation (odds ratio = 16; 95% confidence interval: 1.5-176.6; P = 0.0075) in the multivariate logistic regression analysis. One-quarter of patients with SoCSVV experienced relapse during the 6-month follow-up. The greater number of affected skin areas was an independent risk factor of recurrence (odds ratio = 5; 95% confidence interval: 2-45; P = 0.02).SoCSVV was usually associated with drugs and preceding infections. The disease relapses in approximately one-quarter of the patients. The more severe the skin involvement in the course of SoCSVV, the higher is the risk of recurrence.

Chronic Localized Fibrosing Leukocytoclastic Vasculitis Associated With Lymphedema, Intralymphatic and Intravascular Lymphocytosis, and Chronic Myelogenous Leukemia: A Case Report of Unilateral Erythema Elevatum Diutinum.

One of the pathogenic causes of cutaneous inflammatory pseudotumors is chronic localized fibrosing leukocytoclastic vasculitis (CLFLCV), a vasculitic reaction pattern seen in granuloma faciale (GF), a localized vasculitis, and erythema elevatum diutinum (EED), a generalized vasculitis. Patients with myelodysplastic syndromes (MDSs) are at risk for a diverse spectrum of cutaneous neutrophilic dermatoses such as EED. Herein, we report a 74-year-old man who presented with a large ulcerative, fungating tumor affecting the right flexor ankle caused by CLFLCV. During his workup and management, MDS and Philadelphia chromosome-negative chronic myeloid leukemia was diagnosed. Surgical excision of the inflammatory mass promptly triggered tumor recurrence, whereas antineutrophil therapy (dapsone coupled with hydroxyurea, and prednisone) lead to tumor regression. Histopathologic examination revealed an eosinophilic-rich small-vessel neutrophilic vasculitis associated with storiform and angiocentric fibrosis (GF-like). In the regions of fibrosis, dilated lymphatic and vascular spaces were numerous, some of which were congested with small CD3-positive lymphocytes (intralymphatic and intravascular lymphocytosis). These findings indicate coexisting localized lymphedema. By direct immunofluorescence, IgM and C4d vessel deposits were detected. The pathogenesis of the fibrotic nodules and plaques of CLFLCV is suspected to be due to recurring bouts of immune-complex vasculitis, creating a cycle of vessel damage followed by reparative granulation tissue. Poor lymphatic drainage may be the underlying factor initiating and maintaining recurrent, localized episodes of immune-complex vasculitis and progressive fibrosis. Although his tumor histopathology resembled GF-eosinophilic rich CLFLCV-the clinical context points to a solitary and paraneoplastic case of EED.

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.

Exercise-Induced Vasculitis: A Review with Illustrated Cases.

Although exercise-induced vasculitis (EIV) is usually misdiagnosed, it is not uncommon. Occurring mostly after prolonged exercise, especially in hot weather, EIV is an isolated cutaneous vasculitis with stereotypical presentation. This article reviews the clinical characteristics, treatments, and outcomes of EIV based on the published literature. We report 99 patients who developed EIV after walking, dancing, swimming, or hiking especially during hot weather, including the records of 16 patients with EIV treated at our hospital from 2007 to 2015. Erythematous or purpuric plaques arise on the lower legs, without the involvement of compression socks or stockings. Symptoms include itch, pain, and a burning sensation. EIV is an isolated cutaneous vasculitis. Lesions resolve spontaneously after 10 days. When triggering conditions persist, relapses are frequent (77.5 %). Histopathology demonstrates leukocytoclastic vasculitis in 95 % of cases with C3 or immunoglobulin M deposits in 88 and 46 % of cases, respectively. Blood investigations are negative. EIV appears to be a consequence of venous stasis induced by an acute failure of the muscle pump of the calf and thermoregulation decompensation. Both appear after prolonged and unusual exercise in hot weather. Treatment is not codified; topical corticosteroids may reduce symptoms and wearing light clothes might limit lesion occurrence.

[Cutaneous and mucosal manifestations associated with cocaine use]. / Manifestaciones cutáneas y mucosas asociadas al consumo de cocaína.

Complications due to cocaine are a public health problem. The typical cutaneous disease is leukocytoclastic vasculitis and/or thrombotic vasculopathy affecting mainly the ears. No intense systemic involvement is usually present, but there may be several cutaneous, mucosal and systemic manifestations. Other findings associated as arthralgia, neutropaenia or agranulocytosis, low titer positive antinuclear antibodies, antiphospholipid antibody positivity and neutrophil cytoplasmic antibodies against multiple antigens help the diagnosis. This disease requires a clinical suspicion with a clinical history, a complete physical examination and a broad differential diagnosis for an early and correct diagnosis. The course is usually self-limited. In most cases the only treatment is to discontinue the use of cocaine associated with symptomatic treatment, no proven benefit of systemic corticosteroids.

Vasculitis leucocitoclástica / Leukocytoclastic Vasculitis

Introducción: la vasculitis leucocitoclástica constituye un proceso clínico patológico caracterizado por inflamación y necrosis de los vasos sanguíneos. Objetivo: describir las características clínicas de la vasculitis leucocitoclástica y el tratamiento utilizado. Resultados: se indicaron estudios de laboratorios: eritrosedimentación, células LE I, II y III, factor reumatoide, exudado faríngeo, U.S.A y biopsia de piel; arrojando este último vasculitis leucocitoclástica. Conclusiones: la vasculitis leucocitoclástica se conoce con el nombre de venulitis cutánea necrotizante y ocurre en asociación a enfermedades crónicas, puede ser precipitada por infecciones o drogas y desarrollarse por causa desconocida. Los fármacos y agentes terapéuticos que se describen son: penicilina, sulfonamida, tiamina, hidantoina, alopurinol, hidralazina propiltiuracilo y después de la estreptoquinaza recombinante, anticuerpos monoclonales y radioterapia(AU)

Introduction: Leukocytoclastic vasculitis is a clinicopathologic process characterized by inflammation and necrosis of blood vessels. Objective: Describe the clinical features of leukocytoclastic vasculitis and the treatment used. Results: Laboratory studies were initiated: ESR, LE cellsI, II and III, rheumatoid factor, throat swab, and skin biopsy; the latter showed leukocyte clastic vasculitis. Conclusions: leukocytoclastic vasculitis is known also as cutaneous necrotizing venulitis and it occurs in association with chronic diseases. Infections or drugs can precipitated it andit can be developed by unknown causes.The drugs and therapeutic agents described are penicillin, sulfonamide, thiamine, hydantoin, allopurinol, propylthiouracil and hydralazine after recombinantestreptoquinaza, monoclonal antibodies and radiotherapy(AU)

Vasculitis leucocitoclástica / Leukocytoclastic Vasculitis

Introducción: la vasculitis leucocitoclástica constituye un proceso clínico patológico caracterizado por inflamación y necrosis de los vasos sanguíneos.Objetivo: describir las características clínicas de la vasculitis leucocitoclástica y el tratamiento utilizado.Resultados: se indicaron estudios de laboratorios: eritrosedimentación, células LE I, II y III, factor reumatoide, exudado faríngeo, U.S.A y biopsia de piel; arrojando este último vasculitis leucocitoclástica.Conclusiones: la vasculitis leucocitoclástica se conoce con el nombre de venulitis cutánea necrotizante y ocurre en asociación a enfermedades crónicas, puede ser precipitada por infecciones o drogas y desarrollarse por causa desconocida. Los fármacos y agentes terapéuticos que se describen son: penicilina, sulfonamida, tiamina, hidantoina, alopurinol, hidralazina propiltiuracilo y después de la estreptoquinaza recombinante, anticuerpos monoclonales y radioterapia(AU)