ABSTRACT
A vasculite leucocitoclástica é uma patologia cujos mecanismos estão associados ao processo de inflamação vascular. Estima-se que até 24% dos casos de vasculite estão relacionados ao uso de fármacos, sendo os antimicrobianos beta-lactâmicos um dos grupos farmacológicos comumente associados a este desfecho adverso. A oxacilina, uma penicilina semissintética, possui um anel beta-lactâmico que confere atividade biológica e está associada com maior frequência a relatos de vasculite leucocitoclástica. No entanto, casos semelhantes relacionados a esse antimicrobiano são raros, sendo identificados apenas três casos na literatura. Diante desse contexto, relatamos um quarto caso de vasculite leucocitoclástica em um homem de 56 anos, em tratamento com oxacilina, que desenvolveu a vasculite no 3º dia de uso do antimicrobiano. Além da suspensão da oxacilina, ele foi tratado com 125 mg/dia de metilprednisolona endovenosa por sete dias, seguido de 20 mg/dia de prednisona oral por quatro dias, resultan-do em remissão satisfatória das lesões cutâneas e ausência de novos desfechos adversos. Este caso corrobora a possível relação causal entre o uso de oxacilina e o desenvolvimento da vasculite leucocitoclástica, apesar de sua ocorrência ser rara. A resposta favorável às intervenções terapêuticas, incluindo a suspensão da oxacilina e o uso de corticosteroides, destaca a eficácia dessas abordagens no tratamento dessa complicação (AU).
Leukocytoclastic vasculitis is a pathology whose mechanisms are associated with the process of vascular inflammation. It is estimated that up to 24% of the cases of vasculitis are drug-related, with beta-lactam antimicrobials be-ing one of the pharmacological groups commonly associated with this adverse outcome. Oxacillin, a semisynthetic penicillin, has a beta-lactam ring that confers biological activity and is most frequently associated with reports of leukocytoclastic vasculitis. However, similar cases related to this antimicrobial are rare, with only three cases identified in the literature. Against this background, we report a fourth case of leukocytoclastic vasculitis in a 56-year-old man, on oxacillin treatment, who developed the vasculitis on the 3rd day of antimicrobial use. In addition to oxacillin suspension, he was treated with 125 mg/day of intravenous methylprednisolone for seven days, followed by 20 mg/day of oral prednisone for four days, resulting in satisfactory remission of the skin lesions and no new adverse outcomes. This case provides further evidence supporting the potential causal relationship between the use of oxacillin and the development of leukocytoclastic vasculitis, albeit a rare occurrence. The positive response to therapeutic interventions, such as oxacillin suspension and corticosteroid treatment, underscores the effectiveness of these approaches in addressing this complication (AU),
Subject(s)
Humans , Male , Middle Aged , Oxacillin/adverse effects , Vasculitis, Leukocytoclastic, Cutaneous , beta-LactamsABSTRACT
Abstract Background Direct immunofluorescence (DIF) panels are usually ordered for clinically suspected cutaneous vasculitis, but their positivity rate is variable, and their prognostic significance is not clear to date. Objective The study aims to investigate the systemic involvement rate in leukocytoclastic vasculitis (LCV) patients and the potential clinical and laboratory associations with systemic involvement, including DIF findings. Methods A retrospective study of patients with histopathologically proven cutaneous LCV examined in the dermatology department between 2013 and 2017 was performed. Results Of the 81 patients (mean age, 50.6 years), 42 (52%) were male. The mean time between the appearance of skin lesions and biopsy was 23.1 days, ranging from 2 to 180 days. DIF showed overall positivity of 90.1%, and C3 was the most frequent immunoreactant (82.7%). Any kind of extracutaneous involvement was present in 47 (58%) of patients, with renal involvement being the most frequent (53.1%), followed by articular (18.5%) and gastrointestinal (11.1%) involvement. The presence of renal disease was associated with the detection of IgG in the lesional skin (p = 0.017), and with the absence of IgM in the lesional skin (p = 0.032). There was a significant association between C3 deposition and joint involvement (p = 0.05). Study limitations This is a single-center study with a retrospective design. Conclusion DIF seems to be a useful ancillary diagnostic tool in the evaluation of cutaneous vasculitis, but the relationship between DIF findings and systemic involvement needs to be further elucidated due to contradictory data in the current literature.
ABSTRACT
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.
ABSTRACT
El fenómeno de Lucio es un estado reaccional grave, poco frecuente, mediado por inmunocomplejos que se observa típicamente en los casos de lepra lepromatosa difusa. En Paraguay, corresponde aproximadamente al 2% de los casos de lepra diagnosticados en la mayoría de los casos, se presenta como debut de la enfermedad. Se manifiesta con lesiones en la piel de tipo máculas y placas purpúricas poligonales, generalmente sin compromiso visceral. El diagnóstico lo confirma el estudio histopatológico y es fundamental realizar el diagnóstico diferencial con vasculitis de otras etiologías.
Lucio's phenomenon is a rare and severe reactional state mediated by immune complexes that is commonly seen in cases of diffuse lepromatous leprosy. In Paraguay, it represents approximately 2% of leprosy diagnosed cases, mainly as a debut of the disease. It manifests with macule-type skin lesions and polygonal purpuric plaques, generally without visceral involvement. The diagnosis is confirmed by the histopathological study and it is essential to consider differential diagnosis such as vasculitis of other etiologies.
Subject(s)
Leprosy , Leprosy, LepromatousABSTRACT
Abstract: Leukocytoclastic vasculitis (LCV) is a skin condition that is a result of unregulated immune activation. The exact causes have to date not been established. The studied causes tend to have a higher probability of causing LCV. This raises concerns about a deep-seated causal relationship and the tendency of an individual for the development of LCV. Antipsychotics are a class of drug mainly used for psychiatric disorders including schizophrenia, schizophreniform disorder, or even depressive disorder with psychotic features. These drugs target the dopamine receptors in the central nervous system to exert their effects. They are classified as typical or the older antipsychotics and atypical or the newer antipsychotics. Prevalent in the current literature are the reported cases of LCV with antipsychotic medications. We carried out a systematic review following the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-analysis) protocol to find out previously reported cases on LCV due to antipsychotic medication administration from inception till current date. Our study aims to check and in turn, discuss the causal relationship of antipsychotics with LCV.
ABSTRACT
Resumen Las vasculitis leucocitoclásticas se definen como el daño e inflamación de las paredes vasculares, son aquellas vasculitis de pequeños vasos que anatomopatológicamente presentan leucocitoclasia y puede observarse como una manifestación extraintestinal de la enfermedad inflamatoria intestinal. En la colitis ulcerativa se presentan en menor frecuencia, por inmunocomplejos generados en la mucosa intestinal debido a la exposición del tejido linfoide submucoso a antígenos fecales; podrían precipitarse en las paredes de los pequeños vasos. Se pueden asociar con Clostridium difficile, que es un bacilo grampositivo esporulado, anaerobio estricto, que se encuentra normalmente en el medio ambiente y produce colitis, que se manifiesta como un cuadro diarreico presentado después de la ingesta de antibióticos y altera la flora bacteriana común de este órgano. El caso se trata de un paciente 36 años de edad con cuadro de diarreas líquidas con moco y escaso sangrado; se realizó un estudio endoscópico y anatomopatológico en el que se observó colitis ulcerativa con coproparasitario positivo para antígeno de C. difficile, y en su hospitalización presentó lesiones dérmicas petequiales y necróticas en el cuarto dedo de la mano izquierda, que en la biopsia dio como resultado vasculitis de pequeños vasos. En este artículo se revisan de forma práctica los aspectos relacionados con la fisiopatología, histología, tratamiento y diagnósticos de la manifestación extraintestinal dermatológica rara, como la vasculitis leucocitoclástica en pacientes con colitis ulcerativas asociadas con Clostridium.
Abstract Leukocytoclastic vasculitis is defined as the damage and inflammation of the vascular walls. The term refers to vasculitis of the small vessels that anatomopathologically present leukocytoclasia and it can be seen as an extra-intestinal manifestation of inflammatory bowel disease. In ulcerative colitis, it occurs less frequently due to immune complexes produced in the intestinal mucosa by exposure of the submucosal lymphoid tissue to fecal antigens, which could precipitate in the walls of the small vessels. This condition can be associated with Clostridium difficile, which is a gram-positive, sporulated, strict anaerobic bacillus, normally found in the environment. It causes colitis that manifests as a diarrheal disease following the ingestion of antibiotics that alter the common bacterial flora of this organ. This is the case report of a 36-year-old patient with liquid diarrhea with mucus and scarce bleeding. Endoscopic and anatomopathological studies were performed, finding ulcerative colitis with positive coproparasite for Clostridium difficile antigen. The patient was hospitalized, and during his stay, he presented with petechiae and necrotic skin lesions on the fourth finger of the left hand. Skin biopsy showed small vessel vasculitis. This article is a practical review of the pathophysiology, histology, treatment, and diagnosis of a rare dermatologic extraintestinal manifestation, namely, leukocytoclastic vasculitis, in patients with C. difficile-associated ulcerative colitis.
Subject(s)
Humans , Male , Adult , Vasculitis , Inflammatory Bowel Diseases , Colitis, Ulcerative , Clostridioides difficile , Skin , Therapeutics , Diarrhea , Fingers , HistologyABSTRACT
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.
Subject(s)
Humans , Male , Aged , Vasculitis, Leukocytoclastic, Cutaneous , Oxacillin , Blister , Renal Insufficiency, Chronic , Kidney DiseasesABSTRACT
RESUMEN Las vasculitis leucocitoclásticas son grupos de procesos clinicopatológicos con heterogeneidad etiopatogénica y que requieren diagnóstico diferencial. En este reporte se presenta un caso de vasculitis leucocitoclástica de etiología no infecciosa sin germen aislado. Se revisan las posibles causas de este cuadro fundamentalmente cutáneo, así como su diagnóstico y tratamiento.
ABSTRACT Leukocytoclastic vasculitis is a group of clinicopathological processes with etiopathogenic heterogeneity that require differential diagnosis. This report presents a case of leukocytoclastic vasculitis of non-infectious etiology without an isolated germ. The possible causes of this fundamentally skin condition are reviewed, as well as its diagnosis and treatment.
ABSTRACT
RESUMEN La púrpura de Henoch-Schönlein en el adulto es un reto diagnóstico. Su baja incidencia y su sintomatología poco específica configuran un cuadro clínico que puede pasar desapercibido en diversas ocasiones o solaparse bajo el peso de diferentes sospechas diagnósticas. La púrpura de Henoch-Schönlein no es un cuadro de espectro único. Se considera un grupo de enfermedades de manifestación heterogénea con un eje patogénico común dado por el hallazgo de inflamación de la pared en vasos de pequeño calibre mediada por complejos inmunes. Este es el caso de un paciente de 70 arios quien cursa con un cuadro compatible con púrpura de Henoch-Schönlein, de inicio tardío, caracterizada por su difícil manejo y constantes recaídas. a pesar del uso cuidadoso de las pautas terapéuticas establecidas por los consensos actuales. En este paciente se documentó, de forma concomitante, una infección por citomegalovirus que al recibir tratamiento permitió el control adecuado de síntomas. Adicionalmente, este paciente presentaba una linfocitopenia que parecía ser secundaria a la infección viral.
ABSTRACT Henoch-Schönlein purpura in the adult is a diagnostic challenge. Its low incidence and its unspecific symptomatology in this age group, establish a clinical chart that can be ignored on several occasions. Henoch-Schönlein purpura is considered a group of diseases of heterogeneous manifestation with a common pathogenic axis: the finding of inflammation of the wall of the small calibre vessels, mediated by immune complexes. The case is presented of a 70-year-old patient with a difficult to treat Henoch-Schönlein purpura, with constant relapses despite the use of the therapeutic guidelines established in the current guidelines. In this patient, a concomitant cytomegalovirus infection was documented that, after receiving treatment, allowed adequate control of symptoms. Additionally, this patient also had a lymphocytopenia that was secondary to cytomegalovirus.
Subject(s)
Humans , Male , Aged , IgA Vasculitis , Cytomegalovirus , Diagnosis , Therapeutics , Cytomegalovirus InfectionsABSTRACT
RESUMEN La vasculitis IgA, también conocida como púrpura de Schönlein-Henoch, es una vasculitis leucocitoclástica que involucra pequeños vasos con depósito de inmunocomplejos IgA. Puede abarcar piel, articulaciones, riñones y tracto gastrointestinal. Su presentación en adultos es rara, y las formas clínicas suelen ser más agresivas. Es objetivo del presente trabajo describir el curso y evolución de vasculitis IgA, en un paciente de 59 años, con púrpuras en miembros inferiores y tronco, hematuria macroscópica, y edema de miembros inferiores. Los complementarios mostraron creatininas elevadas, proteinuria de rango nefrótico, elevación de la IgA y anticuerpos contra el citoplasma de los neutrófilos negativos. Se descartaron causas neoplásicas. El estudio anatomo-patológico del riñón concluyó una vasculitis IgA.
ABSTRACT IgA vasculitis, also known as Henoch-Schönlein purpura, is a leukocytoclastic vasculitis that involves small vessels with deposition of IgA immune complexes. It can include skin, joints, kidneys, and gastrointestinal tract. Its presentation in adults is rare, and the clinical forms are usually more aggressive. The objective of this study is to describe the course and evolution of IgA vasculitis, in a 59-years-old patient, with purples in the lower limbs and trunk, macroscopic hematuria, and lower limb edema. The complementary ones showed elevated creatinines, nephrotic range proteinuria, elevated IgA and negative antibodies against the cytoplasm of neutrophils. Neoplastic causes were dismissed. The anatomical-pathological study of the kidney concluded IgA vasculitis.
Subject(s)
Male , Middle Aged , IgA Vasculitis/physiopathology , IgA Vasculitis/diagnostic imagingABSTRACT
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.
ABSTRACT
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.
Subject(s)
Humans , Vasculitis , SkinABSTRACT
Abstract The term vasculitis refers to the 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 and 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 quite different prognosis and treatments. This condition presents many challenges to physicians in terms of classification, diagnosis, appropriate laboratory workup, and treatment. Moreover, it compels a careful follow-up. This article reviews the Chapel-Hill 2012 classification, etiology, recent insights in pathophysiology, some important dermatological clues for the diagnosis and summarizes treatment of some of these complex vasculitis syndromes.
Subject(s)
Humans , Male , Female , Vasculitis/diagnosis , Vasculitis/pathology , Skin Diseases, Vascular/diagnosis , Skin Diseases, Vascular/pathology , Syndrome , Vasculitis/classification , Skin Diseases, Vascular/classification , NecrosisABSTRACT
Abstract Erythema elevatum diutinum is a small vessel vasculitis which is benign, rare, and chronic. It is clinically characterized by violaceous, brown, or yellowish plaques, nodules, and papules. It has been associated with autoimmune, infectious, and neoplastic processes. The following case describes a patient with hepatitis B virus and human immunodeficiency virus with CD4 count < 200 mm3, HIV-seropositive for 16 years, and diagnosed with hepatitis B virus at the hospital. The patient was treated with oral dapsone 100 mg/day, showing regression after seven months of treatment. The authors found three cases in the literature of association of erythema elevatum diutinum, human immunodeficiency virus, and hepatitis B virus.
Subject(s)
Humans , Male , Adult , HIV Infections/complications , Vasculitis, Leukocytoclastic, Cutaneous/pathology , Hepatitis B/complications , Biopsy , Hepatitis B virus/pathogenicity , HIV/pathogenicity , Vasculitis, Leukocytoclastic, Cutaneous/virologyABSTRACT
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.
Subject(s)
Humans , Female , Middle Aged , Algorithms , Vasculitis, Leukocytoclastic, Cutaneous/diagnosis , Vasculitis, Leukocytoclastic, Cutaneous/physiopathology , Vasculitis, Leukocytoclastic, Cutaneous/therapy , Lower ExtremityABSTRACT
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)
Subject(s)
Humans , Female , Aged , Glomerulonephritis, Membranoproliferative/diagnosis , Sjogren's Syndrome/diagnosis , Vasculitis, Leukocytoclastic, Cutaneous/diagnosis , Cryoglobulinemia/diagnosis , Glomerulonephritis, Membranoproliferative/complications , Sjogren's Syndrome/complications , Vasculitis, Leukocytoclastic, Cutaneous/complications , Vasculitis, Leukocytoclastic, Cutaneous/pathology , Cryoglobulinemia/complications , Lower ExtremityABSTRACT
Resumen La vasculitis leucocitoclástica es un trastorno autoinmunitario que afecta pequeños vasos y provoca inflamación, destrucción y necrosis de los mismos; con frecuencia es subdiagnosticada. La causa es multifactorial, la fisiopatología es compleja y los inmunomoduladores son los medicamentos más importantes en su tratamiento. Este artículo tiene por objetivo revisar el estado actual del conocimiento en vasculitis leucocitocástica con insistencia en el diagnóstico y tratamiento. Se revisaron artículos publicados en el periodo comprendido entre 1990 y 2017. Los navegadores fueron Google Crome y Firefox y el motor de búsqueda fue Scholar google. Las bases de datos consultadas fueron: MEDLINE, RIMA Astra-Zeneca y las guías de práctica clínica del sistema de salud mexicano (CENETEC). Se revisaron 108 publicaciones relevantes para el tema, priorizando las pertenecientes a revistas indizadas en MEDLINE y Science Citation Index-JCR. Se requieren estudios para integrar subgrupos clínicos y de tratamiento e investigar los mecanismos de daño tisular en cada subgrupo. La inmunomodulación juega un papel central en el tratamiento.
Abstract Leukocytoclastic vasculitis is an autoimmune disorder that affects small vessels, and causes inflammation, destruction and necrosis of the same. It is often underdiagnosed. The etiology is multifactorial, the pathophysiology is complex and immunomodulators are the most important medications in their treatment. This article aims to review the current state of knowledge in leukocytoclastic vasculitis with emphasis on diagnosis and treatment. Articles published in the period from 1990 to 2017 were reviewed. The browsers were Google Crome and Firefox and the search engine was Scholar google. The database consulted was: MEDLINE, RIMA Astra-Zeneca and the clinical practice guidelines of the Mexican health system (CENETEC); 108 relevant publications to the subject were reviewed, prioritizing those belonging to journals indexed in MEDLINE and Science Citation Index-JCR. Studies are required to integrate clinical and treatment subgroups and investigate the mechanisms of tissue damage in each subgroup. Immunomodulation plays a central role in the treatment.
ABSTRACT
SUMMARY The authors report a case of a 69-year-old man with idiopathic leukocytoclastic cutaneous vasculitis. For three years, the lesions recurred with progressive worsening and were associated with systemic manifestations of low-grade fever, weight loss and raised inflammatory markers. The patient latter presented a 6th cranial nerve involvement, raising the concern of a possible systemic vasculitis, which was latter evidenced by the development of deep vein thrombosis and angina pectoris. The treatment of the patient witch based on the decreasing of inflammatory activity, by using effective immunosuppressive therapy, with lower toxicity is more important than identifying the type of the vasculitis. This case illustrates the importance of awareness for the systemic involvement that can occur in up to 50% of patients with leukocytoclastic cutaneous vasculitis.
RESUMO Os autores reportam um caso de vasculite leucocitoclástica recidivante num homem de 69 anos. Durante cerca de três anos as lesões cutáneas de vasculite leucocitoclástica reapareceram periodicamente, acompanhando-se sempre de um quadro sistêmico caracterizado por febrícula, perda de peso e astenia, assim como aumento de novo dos parâmetros inflamatórios. O aparecimento de parésia do sexto par craniano no decurso de uma dessas recorrências cutâneas levantou a hipótese de estarmos perante uma vasculite mais agressiva, com envolvimento extracutâneo. Esse envolvimento sistêmico foi novamente evidente com aparecimento de angina pectoris e trombose venosa profunda. Atualmente, mais do que a identificação do tipo de vasculite, a abordagem dos doentes com essa patologia assenta na cessação da atividade inflamatória recorrendo a terapêutica imunossupressora eficaz, com a menor toxicidade possível. Destacamos a importância da vigilância do componente sistêmico, que pode ocorrer até 50% na vasculite leucocitoclástica cutânea.
Subject(s)
Humans , Male , Aged , Vasculitis, Leukocytoclastic, Cutaneous/pathology , Recurrence , Biopsy , Anti-Inflammatory Agents, Non-Steroidal/therapeutic use , Naproxen/therapeutic use , Vasculitis, Leukocytoclastic, Cutaneous/drug therapy , Disease ProgressionABSTRACT
Se presenta un caso, portadora de artritis reumatoide de varios años de evolución, que tras abandonar el tratamiento presentó una exacerbación de la actividad de la enfermedad, acompañada de un síndrome general y lesiones dérmicas y neurológicas producidas por una vasculitis leucocitoclastica, fue necesario actualizar el tratamiento con metotrexate y bolos de metilprednisolona obteniendo una respuesta favorable demostrada al desaparecer la actividad de la enfermedad y cicatrización de las lesiones ulcerosas.
present a case, with rheumatoid arthritis of several years of evolution, that after leaving the treatment presented an exacerbation of the activity of the disease, accompanied by a general syndrome and dermal and neurological lesions caused by a leukocytoclastic vasculitis, it was necessary to update the treatment with methotrexate and boluses of methylprednisolone obtaining a favorable response demonstrated when the activity of the disease disappeared and cicatrization of the ulcerous lesions.
ABSTRACT
Resumen El edema agudo hemorrágico de la infancia es una vasculitis leucocito-clástica aguda que afecta a niños hasta los dos años y está caracterizado por la aparición de lesiones purpúricas tipo pápulas y placas en forma de diana, edema en miembros inferiores y fiebre de bajo grado. Se trata de un cuadro benigno y autolimitado, por lo cual su manejo es sintomático. El objetivo de este trabajo es describir un caso de edema agudo hemorrágico y discutir su presentación con la literatura actual. Presentamos un caso clínico de una niña de dos años quien es llevada a la consulta por un cuadro de edema y lesiones equimóticas en maléolos, asociado a síntomas respiratorios de dos meses de evolución. Posteriormente, aparecieron lesiones cutáneas, purpúricas, simétricas, de predominio en miembros inferiores, tipo rosetas, sobreelevados y confluyentes, asociadas a prurito y lesiones similares en brazos, sin compromiso del estado general. Los exámenes de laboratorio mostraron una velocidad de sedimentación glomerular y proteína C reactiva elevadas e IgM positiva para Mycoplasma, así como vas-culitis leucocitoclástica en una biopsia de piel. Se realizó tratamiento para el cuadro respiratorio y el prurito. El cuadro remitió en forma espontánea sin recurrencias posteriores. El diagnóstico correcto del edema agudo hemorrágico de la infancia es fundamental para disminuir el subdiagnóstico, evitar gastos médicos y antibioticoterapia innecesaria.
Abstract Acute hemorrhagic edema of infancy is an acute leukocytoclastic vasculi-tis, that affects children up to 2 years old, characterized by the appearance of purpura papules and plaques in the form of a bulls-eye, lower limb edema and low-grade fever; It is a benign and self-limiting disease so its management is symptomatic. The aim of this report is to describe a case acute hemorrhagic edema of infancy and to discuss their presentation with the current scientific literature. We report the clinical case of a 2 years-old girl, who consulted for edema and ecchymotic lesions on the malleolus, with a 2-month history of respiratory symptoms associated. Thereafter, coalesced and symmetrical purpuric skin lesions predominantly in lower limbs, rosettes type and raised associated with itching and similar injuries in arms, without effecting general wellbeing. Laboratory tests showed an elevated sedimentation rate and C-reactive protein, IgM positive for Mycoplasma, and leukocytoclastic vasculitis in the skin biopsy. Differential diagnoses were discarded. Treatment for respiratory symptoms and pruritus was performed. The clinical manifestation disappeared spontaneously without further recurrences. The correct diagnosis of the disease is essential to reduce sub-diagnosis, avoiding unnecessary medical expenses and antibiotic therapy.