Vasculitis cutánea paraneoplásica asociada a linfomadifuso de célulasB grandes / Paraneoplastic cutaneous vasculitis associated with diffuse large B-cell non-Hodgkin's lymphoma

Resumen Los fenómenos cutáneosparaneoplásicos son heterogéneos, infrecuentes y adquiridos, caracterizados por la presencia subyacente de una neoplasia. La manifestacióncutánea más frecuentemente descripta de la vasculitis paraneoplásica es la púrpura palpable. También puede manifestarse como urticaria, eritema y úlceras de miembros inferiores. Se presenta el caso de un paciente de 38 años con diagnóstico de linfoma difuso de células B grandes y úlceras atípicas de aparición concomitantepor vasculitis leucocitoclástica como fenómeno paraneoplásico. Estas lesiones suelentener un curso paralelo a la neoplasia por lo que al realizar tratamiento de la misma pueden remitir, no así cuando se realiza únicamente tratamiento para las lesiones cutáneas. Remarcamos la importancia de conservar un lecho vital y en estado de granulaciónque acompañeal tratamiento general, para así favorecer una rápida epitelización y prevención de infecciones intercurrentes.

Abstract Paraneoplastic cutaneous phenomena are heterogeneous, infrequent and acquired, characterized by the underlying presence of a neoplasm. The most frequently described cutaneous manifestation of paraneoplastic vasculitis is palpable purpura. It can also manifest as urticaria, erythema and ulcers of the lower limbs. We present the case of a 38-year-old patient with a diagnosis of diffuse large B-cell lymphoma and atypical ulcers of intercurrent onset due to leukocytoclastic vasculitis as a paraneoplastic phenomenon. These lesions usually have a parallel course to the neoplasm, so when the neoplasm is treated they may remit, but not when only the cutaneous lesions are treated. We emphasize the importance of preserving a vital tissue in a state of granulation that accompanies the general treatment, in order to favor a rapid epithelialization and prevention of intercurrent infections.

Tercer caso de vasculitis leucocitoclástica cutánea secundaria al uso de oxacilina: reporte de caso / Third case in the literature of cutaneous leukocytoclastic vasculitis secondary to oxacilin use

Resumen La vasculitis leucocitoclastica es una patologìa que compromete los vasos pequeños y cuya causa predominantemente se ha descrito como idiopatica. Se presenta el caso de una mujer de 78 años hipertensa, diabética y con enfermedad renal crónica en estadio 5, que presentó lesiones limitadas a la piel posterior a la administración de oxacilina para manejo de bacteremia por SAMS. La presentación clínica se basó en purpuras palpables predominantemente en miembros inferiores y lesiones dolorosas coalescentes que formaban ampollas de contenido hemorrágico. Estas lesiones resolvieron gradualmente después del cambio de la terapia mencionada anteriormente. La biopsia fue compatible con vasculitis leucocitoclástica, con paraclínicos que descartaron causas infecciosas y autoinmunes.

Abstract Leukocytoclastic vasculitis is a pathology that involves small vessels and whose cause has been predominantly described as idiopathic. The clinical case of a 78-year-old woman with hypertension, diabetic and chronic stage 5 kidney disease, who presented limited skin lesions after administration of oxacillin for management of bacteremia by MSSA. The clinical presentation consisted on palpable purpura predominantly in the lower limbs and painful coalescent lesions that formed blisters of hemorrhagic content. Lesions gradually resolved after the change of the therapy mentioned above. The biopsy was compatible with leukocytocastic vasculitis, with paraclinics who ruled out infectious and autoimmune causes.

Update on vasculitis: overview and relevant dermatological aspects for the clinical and histopathological diagnosis - Part II

Abstract Vasculitis is a group of several clinical conditions in which the main histopathological finding is fibrinoid necrosis in the walls of blood vessels. This article assesses the main dermatological aspects relevant to the clinical and laboratory diagnosis of small- and medium-vessel cutaneous and systemic vasculitis syndromes. The most important aspects of treatment are also discussed.

Varied presentations of cutaneous vasculitis: a case series

Vasculitis involves a wide spectrum of clinicopathological process with reactive damage to the involved blood vessels. There is loss of vessel integrity instigating haemorrhage & luminal compromise leading to ischemia and necrosis of the tissue supplied by the involved vessels. It may affect varied size and type of blood vessels at different locations. It may be primary or secondary to systemic disease. It may involve a single organ like skin or may involve different organ systems at the same time. This case series include six cases of cutaneous vasculitis affecting different organs with varied presentations. Skin biopsies of six patients with unusual presentations were studied. Their complete history, physical examinations, laboratory investigations including serology were analysed and correlated with histopathological findings. The patients presented with different duration of symptoms varying from as short as 15 days to 1 year. Skin lesions were present in all cases while cardiac manifestation was seen in one. Serology and autoimmune disease markers were negative in all cases except one. However, histopathological features were in concordance with the clinical diagnosis of vasculitis. They were further classified as vasculitis secondary to Churg Strauss syndrome, venous stasis, Henoch Schonlein purpura or leucocytoclastic vasculitis.Vasculitis though a rare disease may manifest as an acute or chronic condition. It needs timely diagnosis by histopathological examination to aid in further management. It is important to assess the clinical severity in primary and secondary vasculitis, as it determines morbidity and mortality.

Vasculitis sistémicas con compromiso cutáneo, en la Cátedra de Dermatología, Facultad de Ciencias Médicas, Universidad Nacional de Asunción / Systemic vasculitis with cutaneous involvement, in the Department of Dermatology, Faculty of Medical Sciences, National University of Asuncion

Introducción: La vasculitis sistémica presenta afectación múltiple y variada de órganos; abarca desde procesos benignos hasta mortales. Sus manifestaciones clínicas, tratamiento y pronóstico dependerán del vaso involucrado, y de la extensión y localización de la lesión. Objetivo: Determinar características epidemiológicas, clínicas, e histopatológicas, y el tratamiento de las vasculitis sistémicas con manifestaciones cutáneas en la Cátedra de Dermatología del Hospital de Clínicas, FCM - UNA. Material y método: estudio descriptivo, observacional, retrospectivo, de cohorte transversal, con componente analítico, de enero de 2004 a diciembre de 2015. Se confeccionó una planilla para registrar las variables obtenidas de fichas clínicas e histopatológicas. Resultados: Se hallaron 11 pacientes con vasculitis sistémica con afectación cutánea, (0,0098% de las consultas). Predominó el sexo masculino (63,63%). La edad media fue 30,18 años. El 18,18% eran niños todos masculinos. La lesión elemental más frecuente fue la pápula (72,72%), la localización predominante, miembros inferiores (72,72%). Los síntomas más frecuentes fueron dolor abdominal (63,63%) y la artralgia (54,54%). El 81,81% correspondía a vasculitis de vasos pequeños. En 72,72%, el componente inflamatorio eran neutrófilos. El 72,72% correspondía a Púrpura de Shönlein - Henoch (PSH), el 18,18% a Poliangeitis Microscópica (PAM), y el 9,09% a Granulomatosis con Poliangeitis (GPA). En el 72,72%, el tratamiento fue Prednisona. Conclusión: Dado que esta patología es poco frecuente, con escasos trabajos que hayan estudiado su epidemiología, este trabajo, con sus limitaciones, podría aportar para futuros estudios.

Introduction: Systemic vasculitis has multiple and varied organ involvement; Ranging from benign to deadly. Its clinical manifestations, treatment and prognosis will depend on the vessel involved, and on the extent and location of the lesion. Objectives: To determine epidemiological, clinical, and histopathological characteristics, and the treatment of systemic vasculitis with cutaneous manifestations in the Department of Dermatology Hospital of Clinics, FCM-UNA. Materials and Methods: A descriptive, observational, retrospective, cross-sectional study with an analytical component from January 2004 to December 2015. A spreadsheet was drawn up to record the variables obtained from clinical and histopathological records. Results: We found 11 patients with systemic vasculitis with cutaneous involvement, (0,0098% of the consultations). The male sex predominated (63,63%). The mean age was 30.18 years. 18,18% were children, all of them male. The most frequent elemental lesion was the papule (72,72%), the predominant location, lower limbs (72,72%). The most frequent symptoms were abdominal pain (63,63%) and arthralgia (54,54%). The 81.81% corresponded to vasculitis of small vessels. In 72,72%, the inflammatory component was neutrophils. 72,72% corresponded to Shönlein-Henoch purpura (PSH), 18,18% to Microscopic Polyangeitis (PAM), and 9,09% to Granulomatosis with Polyangeitis (GPA). In 72,72%, the treatment was Prednisone. Conclusion: Since this pathology is infrequent, with few studies that have studied its epidemiology, this work, with its limitations, could contribute for future studies.

Clinical features and prognostic factors of cutaneous vasculitis among dermatology patients in Johor Bahru, Malaysia

Background: Cutaneous vasculitis is common, yet the riskfactors for its chronicity have not been established.Objective: To describe the clinical spectrum and identify riskfactors for chronicity of cutaneous vasculitis.Methods: Retrospective data analysis of 275 patientsdiagnosed with cutaneous vasculitis from January 2008 toDecember 2013.Results: The mean age was 33.7 (±17.89) years, with femalepredominance. The majority of patients were Malays (67.3%).Skin biopsy was performed in 110 (40%) patients. Thecommonest sign was palpable purpura (30.6%). Theaetiology remained elusive in 51.3% of patients. Commonidentifiable causes include infection (19.7%) and connectivetissue disease (10.2%). Extracutaneous features were notedin 46.5% of patients. Erythrocyte sedimentation rate andantinuclear antibody were raised in 124 of 170 and 27 of 175patients with documented results respectively. Cutaneousvasculitis was the presenting symptom in seven patientswith newly diagnosed systemic lupus erythematosus. AntiStreptolysin O Titre was positive in 82 of 156 patients withdocumented results. Despite antibiotics, 31.7% of them hadchronic lesions. Prednisolone alone was used in 20% ofpatients while 16.4% needed steroid-sparing agents. Mostpatients who needed systemic therapy (62%) hadunidentifiable aetiology. Among the 155 patients whoremained under follow up, 36.4% had chronic disease, onepatient succumbed due to septicaemia, and the rest fullyrecovered within three months. The presence of ulcerativelesion was significantly associated with developing chronicvasculitis (p=0.003).Conclusion: The clinical spectrum of cutaneous vasculitis inour population was similar to other studies. Ulcerativelesion predicts a chronic outcome

CLINICO – HISTOPATHOLOGICAL SPECTRUM OF CUTANEOUS VASCULITIS: A RETROSPECTIVE STUDY OF 62 CASES

Context: Cutaneous Vasculitis is the inflammation of vessel walls which leads to hemorrhagic or ischemic events. The histopathological classification of cutaneous vasculitis depends on the vessel size and the dominant immune cell mediating the inflammation. Object: We studied the etiological factors and clinico-pathological spectrum of patients with cutaneous vasculitis at a tertiary referral centre of north India. Design: Skin biopsies of all patients with clinically suspected cutaneous vasculitis presenting over 5 years, between 2009-2014 were reviewed. Cutaneous vasculitis was classified on the basis of etiology (primary or secondary), on the basis of size of vessel wall as well as on the dominant inflammatory cell infiltrating the vessels. Results: Over 5 years, 62 / 103 patients evaluated for vasculitic syndromes had histologically proven vasculitis. Clinically, vasculitis was primary (77.4%) or secondary (22.5%) to drugs, infections, underlying connective tissue diseases and malignancy. Neutrophilic (n=30), lymphocytic (n=18), eosinophilic (n=10), and granulomatous (n=4) vasculitis were the major histopathological groups. Small vessel involvement was seen in 97% cases. Conclusion: Skin biopsy remains the gold standard for diagnosing cutaneous vasculitis. Small vessel vasculitis was the most common type of cutaneous vasculitis with the dominant cell type being neutrophilic. Eosinophilic infiltrate was exclusively associated with primary vasculitis.

Fenómeno de Raynaud secundario y vasculitis cutánea asociados al uso de interferón / Secondary Raynaud's phenomenon and cutaneous vasculitis associated with the use of interferon

Resumen Se presenta el caso de un hombre adulto con fenómeno de Raynaud secundario y refractario al manejo médico asociado a vasculitis cutánea, que previamente venía recibiendo interferón beta para tratamiento de esclerosis múltiple. La agresividad del proceso requiere la interrupción de la medicación,la utilización de vasodilatadores, inhibidores de endotelina 1, simpatectomía bilateral por videotoracoscópia, aplicación de toxina botulínica periarterial interdigital en manos y terapia inmunosupresora con corticoide a altas dosis y ciclofosfamida obteniendo detención del proceso isquémico, pero con pérdida anatómica asociada. (Acta Med Colomb 2014; 39: 81-84).

Abstract The case of an adult male with secondary Raynaud's phenomenon refractory to medical management and associated with cutaneous vasculitis who previously had been receiving interferon beta for multiple sclerosis treatment, is presented. The aggressiveness of the process required the interruption of the medication, the use of vasodilators, inhibitors of endothelin-1, bilateral thoracoscopic sympathectomy by video, peri-arterial interdigital botulinum toxin application in hands and immunosuppressive therapy with high dose corticosteroids and cyclophosphamide, obtaining arrest of the ischemic process, but with associated anatomical loss. (Acta Med Colomb 2014; 39: 81-84).

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...

Vasculite urticariforme com comprometimento renal glomerular: relato de caso / Urtical vasculitis with renal glomerular disease: case report

JUSTIFICATIVA E OBJETIVOS: A vasculite urticariforme (VU) corresponde entre 5% a 10% das urticárias crônicas, devendo-se distinguir suas lesões daquelas da urticária crônica idiopática, que é a forma mais comum. Apesar de comumente ser de origem idiopática, pode ocorrer em associação com doenças autoimunes, reação a drogas, infecções ou malignidade, podendo ocorrer de forma sistêmica ou limitada à pele. O diagnóstico de VU deve ser considerado na presença de urticária persistente com achados clínicos e sorológicos sugestivos, ou evidência de doença sistêmica. O objetivo deste estudo foi mostrar um caso raro de acometimento renal da forma normocomplementêmica da vasculite urticariforme. RELATO DO CASO: Paciente do sexo masculino, 38 anos, previamente hígido, que apresentava lesões papulares eritêmato-violáceas extensas. Exames laboratoriais iniciais não apresentavam alterações significativas; exame qualitativo de urina demonstrava alteração progressiva da função renal e níveis nefróticos de proteinúria. A imunofluorescência renal foi negativa. Sorologias foram todas negativas e exames imunológicos não reagentes. Dosagemde complemento sérico (C3 e C4) foi normal. Realizou-se pulsoterapia com corticoide endovenoso e seguimento com corticoide oral, obtendo-se boa resposta clínica. A lesão cutânea apresentou regressão espontânea sem o uso de medicação tópica. CONCLUSÃO: O diagnóstico da doença sistêmica a partir de uma alteração cutânea salienta a importância da investigação adicional das lesões vasculares de pele.

BACKGROUND AND OBJECTIVES: Urticarial vasculitis (UV) corresponds between 5% to 10% of chronic urticaria, and their lesions must be distinguished from those of chronic idiopathic urticaria, which is more common. Although commonly idiopathic, it may occur associated with autoimmune diseases, drug reactions, infections or malignancy, which may occur in asystemic presentation or limited to the skin. The diagnosis of UV should be considered in the presence of persistent urticaria with suggestive clinical and serologic findings, or evidence of systemic disease. The case report illustrates a rare renal complication of the normocomplementemic form of urtical vasculitis. CASE REPORT: Male patient, 38 years, previously healthy, who presented extensive erythematous-violaceous papules. Initial laboratory tests showed no significant changes, qualitative urine test showed progressive impairment of renal function and levels of nephrotic proteinuria. Renal immunofluorescence was negative. Serology and immunological tests were all negative. Dosage of serum complement (C3 and C4) was normal. We performed intravenous steroid pulse therapy and follow up with oral steroids,obtaining good clinical response. The skin lesions regressed spontaneously without the use of topical medication. CONCLUSION: The diagnosis of systemic disease from a skin change emphasizes the importance of further investigation of cutaneous vascular lesions.

A Case of Diffuse Muscular Calcification following Necrotizing Cutaneous Vasculitis in a Patient with Dermatomyositis / 대한류마티스학회지

Dermatomyositis is a systemic connective tissue disease affecting the skeletal muscles and skin. Necrotizing cutaneous vasculitis and subsequent diffuse subcutaneous and muscular calcification in patients with dermatomyositis is uncommon and has not been reported. A 23-year-old female with dermatomyositis developed necrotizing cutaneous vasculitis in the knee, elbow, buttock, toe and fingers associated with large ulcer formation. Her left 5th finger was amputated due to massive soft tissue destruction, and her right 5th finger later auto-amputated. Thereafter, she developed small subcutaneous nodules on both wrists, elbows, upper arms, neck, buttocks and thighs. X-ray, computerized tomography and gracilis muscle biopsy confirmed diffuse subcutaneous, fascia and muscular calcifications.

Cutaneous vasculitides: Clinico-pathological correlation.

Background: Cutaneous vasculitis presents as a mosaic of clinical and histological findings. Its pathogenic mechanisms and clinical manifestations are varied. Aims: To study the epidemiological spectrum of cutaneous vasculitides as seen in a dermatologic clinic and to determine the clinico-pathological correlation. Methods: A cohort study was conducted on 50 consecutive patients clinically diagnosed as cutaneous vasculitis in the dermatology outdoor; irrespective of age, sex and duration of the disease. Based on the clinical presentation, vasculitis was classified according to modified Gilliam's classification. All patients were subjected to a baseline workup consisting of complete hemogram, serum-creatinine levels, serum-urea, liver function tests, chest X-ray, urine (routine and microscopic) examination besides antistreptolysin O titer, Mantoux test, cryoglobulin levels, antineutrophilic cytoplasmic antibodies and hepatitis B and C. Histopathological examination was done in all patients while immunofluorescence was done in 23 patients. Results: Out of a total of 50 patients diagnosed clinically as cutaneous vasculitis, 41 were classified as leukocytoclastic vasculitis, 2 as Heinoch−Schonlein purpura, 2 as urticarial vasculitis and one each as nodular vasculitis, polyarteritis nodosa and pityriasis lichenoid et varioliforme acuta. Approximately 50% of the patients had a significant drug history, 10% were attributed to infection and 10% had positive collagen workup without any overt manifestations, while 2% each had Wegener granulomatosis and cryoglobulinemia. No cause was found in 26% cases. Histopathology showed features of vasculitis in 42 patients. Only 23 patients could undergo direct immunofluorescence (DIF), out of which 17 (73.9%) were positive for vasculitis. Conclusions: Leukocytoclastic vasculitis was the commonest type of vaculitis presenting to the dermatology outpatient department. The workup of patients with cutaneous vasculitis includes detailed history, clinical examination and investigations to rule out multisystem involvement followed by skin biopsy and DIF at appropriate stage of evolution of lesions. Follow up of these patients is very essential as cutaneous manifestations may be the forme fruste of serious systemic involvement.

Vasculite cutânea de pequenos vasos: etiologia, patogênese, classificação e critérios diagnósticos - Parte I / Small vessel cutaneous vasculitis: etiology, pathogenesis, classification and diagnostic criteria - Part I

Vasculite é a inflamação da parede dos vasos. Pode variar em gravidade desde doença autolimitada de um único órgão até doença grave com risco de morte por falência de múltiplos órgãos. Existem várias causas, embora só se apresente por poucos padrões histológicos de inflamação vascular. Vasos de qualquer tipo e em qualquer órgão podem ser afetados, resultando em ampla variedade de sinais e sintomas. Diferentes vasculites com apresentações clínicas indistinguíveis têm evolução e tratamento muito diferentes. Essa condição representa desafio para o médico, incluindo classificação, diagnóstico, exames laboratoriais pertinentes, tratamento e seguimento adequado. Neste artigo são revistos a classificação, a etiologia, a patogênese e os critérios diagnósticos das vasculites cutâneas.

Vasculitis is an inflammation of vessel walls. It may range in severity from a self-limited disorder in one single organ to a life-threatening disease due to multiple-organ failure. It has many causes, although they result in only a few histological patterns of vascular inflammation. Vessels of any type in any organ can be affected, a fact that results in a broad variety of signs and symptoms. Different vasculitides with indistinguishable clinical presentations have very different prognosis and treatments. This condition presents many challenges to physicians in terms of classification, diagnosis, appropriate laboratory workup, treatment, and the need for careful follow-up. This article reviews the classification, etiology, pathology and diagnostic criteria of cutaneous vasculitis.