Crioglobulinemia: una entidad heterogénea / Cryoglobulinemia: a heterogeneous entity

La crioglobulinemia se define como la presencia de inmunoglobulinas en el suero que se precipitan reversiblemente a bajas temperaturas. Se la clasifica en tipos I, II y III, según las características de las inmunoglobulinas. La primera suele asociarse a enfermedades linfoprolife-rativas y las de tipos II y III, denominadas crioglobulinemias mixtas, a infección por el virus de la hepatitis C, seguida de las enfermedades autoinmunes. Las manifestaciones clínicas se relacionan con obstrucción intravascular en el caso de la crioglobulinemia de tipo I, mientras que las de tipos II y III se manifiestan con vasculitis por depósito de inmunocomplejos. El compromiso cutáneo es el hallazgo principal, seguido del articular, el neurológico y el renal. Se presentan 3 casos de crioglobulinemia que, por los datos de laboratorio y las enfermedades asociadas, difieren de la descripción clásica publicada en los textos.

Cryoglobulinemia is defined as the presence of immunoglobulins in serum that reversibly precipitate at low temperatures. It is classified into types I, II and III on the basis of immunoglobulin characteristics. Type I is associated with lymphoproliferative disorders, type II and III known as mixed cryoglobulinemia, are associated with hepatitis C virus infection and autoimmune diseases. Clinical manifestations are related with occlusion of small and medium blood vessels common in type I cryoglobulinemia while immune-mediated vasculitis is frequent in mixed cryoglobulinemia. Cutaneous damage is the main manifestation, followed by joint, peripheral nerves and renal involvement. We present three cases of cryoglobulinemia that differ from the literature due to their laboratory findings and associated diseases.

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.

Vasculitis crioglobulinémica con compromiso renal y síndrome de Sjögren / Cryoglobulinemic vasculitis with renal involment and Sjögren's syndrome

La vasculitis crioglobulinémica es una vasculitis de vasos pequeños que se da en presencia de crioglobulinas séricas. Las crioglobulinemias mixtas son las más frecuentes y se asocian a infecciones crónicas, típicamente al virus de la hepatitis C, así como a enfermedades linfoproliferativas y autoinmunes, más a menudo al síndrome de Sjögren. Las manifestaciones clínicas incluyen púrpura de los miembros inferiores, neuropatía periférica, artralgias y glomerulonefritis. La presencia de vasculitis crioglobulinémica en el contexto de un síndrome de Sjögren es marcadora de peor pronóstico. Presentamos el caso de una paciente con vasculitis crioglobulinémica con compromiso cutáneo y renal, que condujo al diagnóstico de un síndrome de Sjögren primario. (AU)

Cryoglobulinemic vasculitis is a small-sized vasculitis that occurs in the context of serum cryoglobulins. Mixed cryoglobulinemias are the most frequent and are associated with chronic infections, typically hepatitis C and autoimmune diseases, most commonly Sjögren's syndrome. Clinical manifestations include purpura of lower limbs, peripheral neuropathy, arthralgias and glomerulonephritis. The presence of cryoglobulinemic vasculitis in the context of Sjögren's syndrome is a marker of poor prognosis. We present the case of a patient with cryoglobulinemic vasculitis associated to cutaneous and renal involvement that led us to the diagnosis of primary Sjögren's syndrome. (AU)

Síndrome POEMS: um diagnóstico desafiador / POEMS Syndrome: a challenging diagnosis

A síndrome POEMS é um distúrbio multissistêmico. Sua patogênese não está totalmente estabelecida, mas sabe-se que tem relação com fator de crescimento vascular endotelial, interleucinas e fator de necrose tumoral alfa. A idade média de incidência é 50 anos, com maior prevalência em homens. Neuropatia periférica e gamopatia monoclonal estão presentes em todos os pacientes e são consideradas critérios maiores; quando associadas a pelo menos um critério menor, estabelecem diagnóstico da síndrome. As opções de tratamento são radioterapia, corticosteroides e quimioterapia, além de transplante autólogo de células-tronco hematopoiéticas. (AU)

POEMS syndrome is a multisystem disorder. Its pathogenesis isn't fully established, but it is known to be related to endothelial vascular growth factor, interleukins, and tumoral necrosis factor alpha (TNF-α). The mean age at incidence is 50 years, with a higher prevalence in men. Peripheral neuropathy and monoclonal gammopathy are present in all patients, and are considered major criteria; when associated with at least one minor criterium, they establish the diagnosis of the syndrome. Treatment options are radiotherapy, corticosteroids, chemotherapy, as well as autologous hematopoietic stem cell transplantation. (AU)

Edema agudo hemorrágico de la infancia: Lesiones alarmantes de un cuadro benigno. Reporte de caso / Acute hemorrhagic edema of infancy: alarming lesions of a benign condition. Case report

Se reporta un caso de edema agudo hemorrágico de la infancia, en un lactante de 18 meses, después de un episodio de otitis media. El cuadro comenzó con máculas eritematosas en los muslos, seguidas de lesiones purpúricas en los brazos, las piernas y edema en los tobillos. Se interpretó, inicialmente, como urticaria, por lo que recibió esteroides. Sin embargo, las características clínicas fueron de edema agudo hemorrágico de la infancia, una vasculitis leucocitoclástica benigna que se presenta en niños de entre 4 y 24 meses y que se caracteriza por fiebre, máculas y lesiones purpúricas. Estas se ubican, principalmente, en la cara, los lóbulos de las orejas y las extremidades, y se asocian, muchas veces, a edema. Los diagnósticos diferenciales son eritema multiforme, urticaria, vasculitis inducida por droga, enfermedad de Kawasaki, eccema infectado, meningococcemia y maltrato infantil, algunas de ellas, con riesgo de mortalidad. El manejo es conservador, sin embargo, los esteroides podrían ser una opción terapéutica.

We report a case of acute hemorrhagic edema of infancy in an 18-month-old boy after an episode of otitis media. The clinical presentation begins with skin erythematous macules on the thighs, followed by purpuric lesions in arms, legs, and ankle edema. It was initially interpreted as urticaria, whereby steroids were indicated. However, the clinical feature was acute hemorrhagic edema of infancy, a benign leukocytoclastic vasculitis that occurs in children between 4 and 24 months of age and is characterized by fever, large purpuric palpable target-like skin lesions affecting the face, lobes of the ears, limbs and frequently associated with edema. Differential diagnosis includes erythema multiforme, hemorrhagic urticaria, drug-induced vasculitis, Kawasaki disease, infected eczema, sepsis (either meningococcal or non-meningococcal) and child abuse. Some of them have risk of mortality. Management is conservative, however, steroids may be a therapeutic option.

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)

Clinicopathological study of cutaneous vasculitides

To study the different patterns of cutaneous vasculitides along with their underlying etiologic factors and to assess the clinicopathological correlation. A cross sectional study was conducted on 30 consecutive patients, histologically diagnosed as cutaneous vasculitis in the department of dermatology and venereology, BSMMU, Dhaka. All patients were subjected to a baseline workup consisting of complete hemogram, serum-creatinine levels, liver function tests, chest X-ray, urine [routine and microscopic] examination besides antistreptolysin O titer, antinuclear antibody assay, rheumatoid factor assay, antineutrophilic cytoplasmic antibodies and hepatitis B and C. Histopathological examination was done in all patients while immunofluorescence was done in 9 patients. Out of a total of 30 patients diagnosed histologically as cutaneous vasculitis, 22 were classified as cutaneous small vessel vasculitis [CSVV], 6 as Heinoch-Schonlein purpura, and one each as urticarial vasculitis, and Behcet's syndrome. Approximately 30% of the patients had a significant drug history, 23.3% were attributed to infection. No cause was found in 46.7% cases. Only 9 patients could undergo direct immunofluorescence [DIF], out of which 4 were positive for vasculitis. Cutaneous small vessel 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 formc fruste of serious systemic involvement

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...

An aetiological & clinicopathological study on cutaneous vasculitis.

Background & objectives: Cutaneous vasculitis has protean clinical manifestations. It may be idiopathic or associated with a spectrum of conditions such as infections, drugs, etc. Skin is involved in both small vessel vasculitis (SVV) and medium vessel vasculitis (MVV). Overlapping features are seen between SVV and MVV. The histopathological features may not always relate with the clinical lesions. The aim of the present study was to evaluate the aetiological factors and clinicopathological association in patients with cutaneous vasculitis. Methods: In this cross-sectional study, detailed history and clinical examination were done on patients with biopsy proven cutaneous vasculitis. Two skin biopsies were taken from each patient for routine histopathology and direct immunofluorescence. Results: Of the 61 patients studied, hypersensitivity vasculitis (HSV) [23 (37.7%)] and Henoch Schonlein purpura (HSP) [16 (26.2%)] were the two most common forms. Systemic involvement was seen in 32 (52.45%) patients. Drugs were implicated in 12 (19.7%) cases, infections in 7 (11.4%) and connective tissue disorders in 4 (6.5%) cases. Histologically SVV was the most common pattern, seen in all the clinically diagnosed patients with SVV (47), and in 12 of the 14 clinically diagnosed patients with MVV. Direct immunofluorescence showed positivity for at least one immunoreactant in 62 per cent of the patients and the most common deposit was C3 followed by IgG, IgA and IgM. Interpretation & conclusions: Majority of our patients with cutaneous vasculitis were idiopathic. Histologically, SVV was seen in most of our patients. No association was seen between history of drug intake and tissue eosinophilia and also between histologically severe vasculitis and clinical severity. The presence of immunoreactant IgA was not specific for HSP.

Leukocytoclastic Vasculitis Associated with Influenza A Virus Infection

Leukocytoclastic vasculitis (LCV) usually presents palpable purpura characterized by inflammation of vessel walls and fragmentation of nuclei. Various conditions can cause LCV, and it can be induced by influenza A virus infection. We report a 2-yr-old Korean girl who presented palpable purpuric and hemorrhagic lesions with fever. She was diagnosed as LCV by skin biopsy, and influenza A virus was isolated from nasopharyngeal swab. She was treated with oseltamivir (Tamiflu(R)) and prednisolone with dramatic effect of vasculitis and fever.

Vasculite leucocitoclástica: mais uma "imitação" da sífilis / Leukocytoclastic vasculitis: another condition that mimics syphilis

A sífilis, doença de importante morbiletalidade no passado, tem ressurgido nos últimos anos, graças, sobretudo, às alterações nos comportamentos de risco. Um grupo epidemiológico, frequentemente, acometido é a população com infecção pelo HIV: estes pacientes podem apresentar características peculiares nas manifestações e evolução da doença. Relatamos o caso de um paciente masculino, HIV-positivo, que desenvolveu um quadro florido de secundarismo: além da roséola sifilítica, apresentou pan-uveíte bilateral e acometimento do sistema nervoso central. A investigação, apresentou fenomeno pro-zona e no estudo histologico, mostrou a presenca de vasculite leucocitoclastica achado este extremamente raro e pouco documentado.

Syphilis, a disease that in the past was associated with significant morbidity and lethality rates, has resurged in recent years principally as a consequence of changes in risk behavior. An epidemiological group that is commonly affected is the HIV-infected population. The characteristics of the disease and its progression may differ in these patients. The present report describes a case of an HIV-positive male patient, who developed florid secondary syphilis: in addition to syphilitic roseola, he also presented with bilateral panuveitis and involvement of the central nervous system. Investigation revealed the prozone phenomenon and histological examination of the skin lesions showed the presence of leukocytoclastic vasculitis. This finding is extremely rare and few cases have been documented.

Eritema elevatum diutinum: reporte de dos casos y revisión de la literatura / Erythema elevatum diutinum: report of two cases and literature review

Eritema elevatum diutinum es una enfermedad cutánea poco frecuente y de curso crónico recurrente. Se caracteriza por pápulas, placas y nódulos de color rojo-violáceo de distribución acral y simétrica sobre las superficies extensoras. Un hallazgo histopatológico característico es la vasculitis leucacitoclástica. El tratamiento de elección es la dapsona, A continuación se describen dos casos de Eritema Elevatum Diutinum, el primero en un paciente adulto y el segundo en una niña de 15 años, ambos con estudio histopatológico compatible y buena respuesta al uso de difenildiazona (dapsona).

Erithema elevatum diutinum is a rare cutaneous disease. It usually has a chronic course and is characterized by purple red papules, plaques o nodules with an acral and symmetrical distribution. The main histological feature is o leucocytoclostic vasculitis. Treatment of choice is dapsone. We describe two cases of erithema elevatum diutinum, a male adult patient and a 15 year old girl. Both cases present characteristic histological findings and good response to dapsone.

Vasculite leucocitoclástica: associação com infecção pelo vírus da hepatite C / Leucocitoclastic vasculitis: association with hepatitis C virus infection

A vasculite leucocitoclástica pode estar associada a infecções, drogas ou neoplasias malignas. Os autores relatam o caso clínico de um paciente que apresentou quadro de lesões petequiais e púrpura palpável em membros inferiores, sendo diagnosticado histologicamente como vasculite leucocitoclástica. Na investigação etiológica foi evidenciada infecção pelo vírus da hepatite C, já com hepatopatia crônica (AU)

Leucocitoclastic Vasculitis may be associated with infections, drugs, or malignant neoplasms. Here the authors report the case of a patient with palpable, petechial purple lesions in the lower limbs, which were histologically diagnosed as leucocitoclastic vasculitis. An etiological investigation evidenced hepatitis c virus infection with chronic hepatopathy (AU)

Acute hemorrhagic edema of infancy.

The authors report a case of acute hemorrhagic edema of infancy in an 8-month-old boy with a history of recent vaccination prior to the illness. He was well in general, apart from mild fever diarrhea, and the rash compatible with acute hemorrhagic edema of infancy; though, he had unusual trunk and mucosal involvement. The diagnosis was confirmed by the histopathological findings and direct immunofluorescence of the lesion. Complete resolution spontaneously occurred within 3 weeks.

Eritema elevatum diutinum e infección por HIV / Erythema elevatum diutinun and HIV infection

El eritema elevatum diutinum (EED) es una forma localizada, crónica e infrecuente de vasculitis leucocitoclástica cutánea, caracterizada clínicamente por lesiones papulonodulares de color eritematovioláceo, pardas o amarillentas. Histopatológicamente evoluciona hacia una fibrosis concéntrica. Su causa es desconocida, pero se presume que se debe al depósito de inmunocomplejos en los vasos. Se lo asocia a múltiples enfermedades y también a HIV. Describimos el primer paciente con esta asociación en la Argentina, actualizamos el tema efectuando una revisión bibliográfica y confrontamos las características clínicas, histopatológicas, inmunológicas y terapéuticas de nuestra paciente con los pacientes de la literatura.

Typhoid fever complicated by leukocytoclastic vasculitis

We report a case of typhoid fryer with an unusual presentation: prolonged fever with painful purpuric skin lesions of the legs, splenic abscess and pancreatitis, The diagnosis was made upon isolation of S. typhi in blood cultures, and ruling out other causes of leukocytoclastic vasculitis. The outcome was favorable with antibiotics alone without surgery

Edema hemorrágico agudo del lactante / Infant acute hemorrhagic edema

El edema hemorrágico agudo del lactante (EHAL) es una variante de vasculitis leucocitoclástica confinada a la piel que se caracteriza por placas purpúricas y edema de las extremidades, en un niño en buenas condiciones generales. Durante una o dos semanas previas al inicio del cuadro se describen infecciones, uso de drogas o inmunizaciones los que gatillarían un proceso inmunológico mediado por inmunocomplejos. Su principal diagnóstico diferencial es el púrpura de Schõnlein-Henoch. Es de evolución benigna y autolimitada. Objetivo: Presentar un caso de EHAL, dando a conocer la enfermedad y entregar herramientas de diagnóstico. Caso clínico: Lactante de 8 meses que presenta edema y lesiones purpúricas extensas en cara y extremidades asociadas un cuadro respiratorio intercurrente. No había compromiso del estado general, los exámenes de laboratorio fueron normales y la biopsia de piel fue compatible con EHAL. Se descartaron los diagnósticos diferenciales más importantes. No se realizó ningún tratamiento específico y el cuadro remitió en forma espontánea sin recurrencias posteriores. Este caso representa el cuadro típico del edema hemorrágico agudo del lactante, el cual es poco frecuente, pero sus manifestaciones cutáneas son muy alarmantes y pueden originar confusión en el diagnóstico.