Cutaneous lesions and finger clubbing uncovering hypocomplementemic urticarial vasculitis and hepatitis C with mixed cryoglobulinemia / Lesoes cutaneas e baqueteamento digital reveladores de vasculite urticariforme hipocomplementemica e hepatite C com crioglobulinemia mista

Urticarial vasculitis is a rare clinicopathologic entity characterized by urticarial lesions that persist for more than 24 hours and histologic features of leukocytoclastic vasculitis. Patients can be divided into normocomplementemic or hypocomplementemic. The authors report the case of a healthy 49-year-old woman with a 1-year history of highly pruritic generalized cutaneous lesions and finger clubbing. Laboratory tests together with histopathologic examination allowed the diagnosis of hypocomplementemic urticarial vasculitis, chronic hepatitis C and type II mixed cryoglobulinemia. The patient started symptomatic treatment and was referred to a gastroenterologist for management of the hepatitis C, with progressive improvement of the skin condition. The development of hypocomplementemic urticarial vasculitis in the context of chronic hepatitis C is exceedingly rare and possible pathogenic mechanisms are discussed.

A vasculite urticariforme é uma entidade clinico-patológica rara caracterizada por lesões urticariformes com duração superior a 24 horas e uma vasculite leucocitoclásica na histologia. É dividida em normo e hipocomplementêmica. Os autores relatam o caso de uma mulher saudável de 49 anos, com lesões cutâneas intensamente pruriginosas e baqueteamento digital com 1 ano de evolução. O estudo efectuado permitiu efectuar os diagnósticos de vasculite urticariforme hipocomplementêmica, hepatite C crônica e crioglobulinêmia mista tipo II. A doente iniciou tratamento sintomático e foi referenciada para a Gastroenterologia para orientação da hepatite, com melhoria progressiva das lesões cutâneas. O desenvolvimento de vasculite urticariforme hipocomplementêmica no contexto de hepatite C crónica é raro e os possíveis mecanismos patogênicos são discutidos.

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.

Edema agudo hemorrágico da infância: relato de três casos / Acute hemorrhagic edema of infancy: report of three cases

O Edema Agudo Hemorrágico da Infância é uma vasculite leucocitoclástica pouco frequente, que ocorre, quase exclusivamente, em crianças entre 4 meses e 2 anos de idade. Caracteriza-se, clinicamente, pela tríade febre, lesões purpúricas na face, pavilhões auriculares e extremidades e edema. Embora os achados cutâneos sejam dramáticos e de surgimento rápido, o prognóstico é favorável, com resolução espontânea dentro de 1 a 3 semanas. Descrevem-se três casos cujos achados clínicos e histopatológicos são característicos de edema agudo hemorrágico da infância.

Acute Hemorrhagic Edema of Infancy is an infrequent leukocytoclastic vasculitis which occurs almost exclusively in children between 4 months and 2 years of age. It is clinically characterized by the triad fever, purpuric lesions on the face, auricular pinna and extremities, and edema. Although the cutaneous findings are dramatic and of rapid onset, the prognosis is favorable, with spontaneous resolution within 1 to 3 weeks. Three cases are described in which clinical and histopathological findings are characteristic of acute hemorrhagic edema of infancy.

Vasculitis de vaso pequeño asociada a un producto oral usado para la caída del pelo / Small vessels vasculitis associated with a oral medication for loss hair

Se presenta el caso de una mujer de 36 años que consultó por lesiones vasculíticas en extremidades inferiores que aparecieron con relación al uso de un producto oral comercializado para la caída del pelo que contiene prosilium, vitaminas B6, B5, B8, hierro, zinc y vitamina E. Algunos elementos evolucionaron hacia la necrosis, especialmente en la pierna derecha. Los análisis de laboratorio permitieron excluir otras patologías asociadas con vasculitis, diagnosticándose una vasculitis cutánea de vaso pequeño posiblemente asociado a alguno de los componentes de dicho producto.

A 36 year old woman went for a consultation because of vasculitic lesions in her lower extremities that appeared after the use of a commercial oral product for hair loss which contains prosilium, vitamin B6, B5, B8, E, iron, and zinc. Some elements evolved towards necrosis, especially in the right leg. Laboratory analysis excluded other causes of vasculitis. The final diagnosis was a cutaneous vasculitis possible associated with some of the components of the hair loss product.

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)

Vasculite leucocitoclástica cutânea associada à tuberculose pulmonar / Cutaneous leukocytoclastic vasculitis accompanied by pulmonary tuberculosis

Relatamos o caso de um homem de 50 anos com uma rara associação: tuberculose pulmonar e vasculite leucocitoclástica cutânea. O paciente procurou o pronto atendimento em razão do quadro de cefaléia, artralgia, rash cutâneo e perda ponderal (4 kg) nos últimos 20 dias. A radiografia de tórax, solicitada em consulta ambulatorial prévia, demonstrava cavitação nos lobos médio e superior do pulmão direito, confirmada por tomografia computadorizada. Apresentou baciloscopia de escarro (coloração de Ziehl-Neelsen) positiva em três amostras consecutivas e biópsia da lesão de pele compatível com vasculite leucocitoclástica cutânea. Foi, então, realizado o diagnóstico de vaculite leucocitoclástica cutânea associada à tuberculose pulmonar. Nosso objetivo é descrever uma associação pouco relatada na literatura médica e discutir seus possíveis mecanismos patogênicos.

We report the case of a 50-year-old male with a rare combination: pulmonary tuberculosis and cutaneous leukocytoclastic vasculitis. The patient sought emergency treatment presenting with headache, arthralgia, cutaneous rash, and weight loss (4 kg) in the last 20 days. A chest X-ray, performed in a previous outpatient visit, revealed cavitation in the middle and upper lobes of the right lung, as confirmed by computed tomography. Sputum smear microscopy (Ziehl-Neelsen staining) was positive in three consecutive samples, and the result of the skin lesion biopsy was consistent with cutaneous leukocytoclastic vasculitis. The patient was therefore diagnosed with cutaneous leukocytoclastic vasculitis accompanied by pulmonary tuberculosis. Our objective was to describe a combination rarely reported in the medical literature and to discuss the possible pathogenic mechanisms of this combination.

Cutaneous allergic vasculitis due to Solenopsis geminata (Hymenoptera: Formicidae) envenomation in Indonesia.

Severe cutaneous allergic vasculitis in a 60 year-old Caucasian male following the bite of the tropical fire ant, Solenopsis geminata (F.) is reported. Over the course of 8 weeks, the pathology progressed from an extensive red papular urticaria to vasculitis with peri-vascular inflammation and ulceration of the skin on the feet, ankles and lower limbs. Many of the affected areas of the skin eventually became covered with black eschar associated with further tissue breakdown and ulcer formation. After debridement, compression dressings, antimicrobial ointment and corticosteroids, complete healing eventually took place with only residual scarring. An awareness of the severe dermatologic reactions caused by a bite of S. geminata, albeit rare, is clinically important. Recognizing the characteristic skin lesions caused by the bite of S. geminata, treated with prompt administration of appropriate chemotherapy will speed recuperation of the patient and reduce possible secondary complications.

Paraneoplastic leukocytoclastic vasculitis in chronic lymphoid leukemia.

A 50-year-old female who was a known case of chronic lymphoid leukemia (CLL) developed ecchymoses, purpuric spots with papules, some nodules (1-3 mm) and crusts all over the body associated with severe burning and itching along with exaggeration of CLL. The lesions were more prominent on lower limbs and face. Skin biopsy was reported as leukocytoclastic vasculitis. These lesions regressed after treatment with leukeran and glucocorticoids.

Edema hemorrágico agudo del lactante: reporte de dos casos / Acute infantile hemorrhagic edema: two case report

El Edema Hemorrágico Agudo del Lactante (EHAL) es una vasculitis leucocitoclásica de curso benigno, generalmente sin compromiso sistémico, que se presenta entre los 4 y 24 meses de edad. Clínicamente se caracteriza por la súbita aparición de placas o pápulas purpúricas, edematosas, que forman lesiones en escarapela, localizadas principalmente en mejillas, pabellones auriculares y porción distal de extremidades. Su patogenia es desconocida: se postula que el mecanismo sería mediado por inmunocomplejos, probablemente precipitado por infecciones virales, ingesta de medicamentos y vacunas recientes. En la histopatología se encuentra una vasculitis leucocitoclástica. Algunos plantean que se trataría de una variante del púrpura de Schõnlein-Henoch. El cuadro se resuelve espontáneamente en una a tres semanas, por lo que no requiere tratamiento específico. Se presentan dos casos clínicos característicos, destacando en uno de ellos la presencia de PCR positiva para parvovirus B19, cuya asociación es relativamente infrecuente.

Leucocytoclastic vasculitis as a presentation of adenocarcinoma rectum.

Vasculitis has been linked to several processes, like infections, drugs and allergic, rheumatologic and neoplastic diseases. Neoplasm-associated vasculitis described in the medical literature has mostly been reported in association with haemotological neoplasms. Adenocarcinoma of rectum presenting as leucocytoclastic vasculitis is rare. We present a case of a 43-year-old male with paraneoplastic leucocytoclastic vasculitis preceding the manifestation of adenocarcinoma rectum. The vasculitis subsided on resection of the rectal malignancy and the patient did not require steroid therapy thereafter.

Erythema elevatum diutinum as a first clinical manifestation for diagnosing HIV infection: case history

CONTEXTO: O eritema elevatum diutinum é uma dermatose crônica, rara, variante clínica da vasculite leucocitoclástica, provavelmente mediada por imunocomplexos. Está sendo incluído no grupo das dermatoses específicas associadas à infecção pelo HIV. Em geral, associa-se a outros processos infecciosos, auto-imunes e neoplásicos. RELATO DE CASO: Relatamos o caso de um paciente em que a manifestação cutânea de eritema elevatum diutinum foi a primeira evidência clínica para diagnóstico da infecção pelo HIV. O tratamento foi feito com dapsona e se obteve regressão parcial das lesões após 15 dias, mesmo antes de o esquema anti-retroviral ser prescrito. CONCLUSÃO: Frente ao diagnóstico de eritema elevatum diutinum, deve-se investigar a infecção pelo HIV, principalmente nas apresentações clínicas atípicas e exacerbadas.

Vasculite leucocitoclástica crônica: relato de um caso e revisão bibliografica / Chronic leukocytoclastic vasculitis: case report and literature review

Os autores apresentam um caso de vasculite cutânealeucocitoclástica secundária a medicamentos, com lesões segmentares na pele. Uma paciente de 39 anos, branca, sexo feminino, com antecedente de uso de penicilina nos sete dias anteriores à consulta, apresentou lesões cutâneas multissegmentares e simétricas em quase todoo corpo, com predomínio nos membros inferiores, abaixo do joelho,e sem incidência apenas no pólo cefálico e nas regiões palmares e plantales. A avaliação da paciente baseou-se no estudo clínico, nos exames laboratoriais, na documentação fotográfica e na análise histopatológica,que revelaram elementos compatíveis com o diagnóstico de vasculite leucocitoclástica. A paciente foi submetida a tratamento clínico com imunossupressores e corticóides, sem resposta clínica adequada. De acordo com a literatura médica, supõe-se que essas lesões necrotizantes da pele estejam imunologicamente relacionadas, na maioria das vezes, ao uso de drogas e geralmente respondem bem ao tratamento clínico.

Eritema elevatum et diutinum / Erythema elevatum diutinum

Eritema elevatum et diutinum es una extraña enfermedad que se presenta con persistentes pápulos o plaquetas de color rojo a amarillo/café, de superficie suave que se encuentra sistemáticamente ubicados principalmente sobre aspectos extensores de las manos y desos, codos, rodillas, tobillos o glúteos. Estudios hisiológicos muestran una vasculitis leucositoclásica en lesiones tempranas y posteriormente una fibrosis. Presentamos tres casos y un resumen de la literatura existente

Cutaneous vasculitis in a patient with primary Sjogren's syndrome.

Sjogren's syndrome (SS) is an uncommonly described disorder from India. In this report we describe a patient with SS and who developed cutaneous vasculitis.

Vasculitis inducida por medicamentos. Estudio de 13 casos

Se estudiaron trece casos de vasculitis medicamentosa comprobada clínica e histopatológicamente. La mayoría fueron linfomonocíticas (siete casos) y leucocitoclástica (un caso). Clínicamente las lesiones cutáneas eritemato-papulares se observaron en seis casos, la púrpura palpable en cuatro, la urticaria crónica, las lesiones ulcero-necróticas en tres y el prurito generalizado en seis. Algunos tenían más de una manifestación clínica. Dos pacientes presentaron miopatía fibrosa secundaria al uso intramuscular crónico de D-propoxifeno. La patogénesis de las vasculitis por drogas no es conocida completamente. Se cree que en ella están involucrados fenómenos inmunológicos de tipo humoral y celular. La miopatía fibrosa posiblemente es secundaria a un proceso inflamatorio estimulado por la irritación mecánica de la aguja o por algunos medicamentos. Esta es la serie más grande publicada en la literatura de vasculitis medicamentosa y los primeros casos informados de miopatía fibrosa secundaria a D-Propoxifeno.

Cutaneous leukocytoclastic vasculitis: clinical and laboratory features of 45 patients seen in Ramathibodi Hospital.

Forty-five patients with histologically proved cutaneous leukocytoclastic vasculitis were studied with regard to the clinical features, laboratory findings and etiology. There were 12 males and 33 females, with an age range of 13 to 64 years. The most common skin lesions were palpable purpura which appeared mostly on the lower part of the legs. Renal involvement was the most common systemic manifestation, which occurred in 45 per cent of the patients. Abdominal pain occurred in 42 per cent of the male patients while none of the female patients had this symptom. Arthralgia occurred in 20 per cent of the patients. The most common laboratory abnormalities were elevation of ESR, which was significantly more common in females than in males (P = 0.047). The possible etiology of leukocytoclastic vasculitis was identified in 5 patients, these included streptococcal infection in 2 patients, in the other patients the possible causes were penicillin hypersensitivity, systemic lupus erythematosus and livedoid vasculitis, respectively.