Direct immunofluorescence demystified: Essential insights and recent advances for dermatologists.

Direct immunofluorescence (DIF) is widely used in dermatopathology for the diagnosis of autoimmune blistering diseases (AIBDs), cutaneous vasculitis, and connective tissue disorders. Although it is easy and useful to perform, it needs technical expertise and experience for proper interpretation. The yield of DIF depends on multiple factors including the adequacy, transportation, storage, processing, and interpretation of the biopsy specimen. Effective collaboration between the dermatologist and dermatopathologist along with meticulous clinico-pathological correlation is crucial for accurately interpreting DIF in the appropriate clinical context. In this narrative review of DIF in dermatology, we discuss the indications of DIF, recent updates on the selection of optimum biopsy sites, basic techniques of DIF including the classical transport medium and its alternatives, processing and staining technique, patterns in various diseases, advancements such as serration pattern analysis, and latest recommendations on the use of DIF in cutaneous disorders.

Atypical presentation of varicella-zoster virus reactivation in a lung transplant patient: a case report.

Background. Varicella-zoster virus (VZV) is a human neurotropic virus which commonly causes infection during childhood, presenting as chickenpox. Later in life it may reactivate as herpes zoster. We report a rare manifestation of reactivation of VZV infection presenting as cutaneous vasculitis and varicella pneumonia in a lung transplant recipient. Case presentation. A 65-year-old man was lung transplanted bilaterally for emphysema and had repeated posttransplant chest infections and colonization with Pseudomonas aeruginosa. Nine months post-transplant he presented with dyspnoea and a cutaneous vasculitis-like eruption with a predilection over face, thorax and distal extremities. Initially, VZV reactivation was not suspected due to absence of the typical vesicular eruptions. The diagnosis was confirmed by VZV PCR from the swabs of the ulcer after skin punch biopsy of a lesion and from bronchoalveolar lavage (BAL). The histology of skin biopsy demonstrated epithelial damage and vascular damage but no typical epithelial virus associated changes. The patient responded to antiviral therapy with total remission of rash and VZV DNA was finally not detectable from repeated BAL after 29 days of therapy. However, the pulmonary radiological features and dyspnoea persisted due to reasons possibly unrelated to the VZV infection. Conclusion. Had it not been for the patient to mention the resemblance of the vasculitic rash with his primary VZV infection, the diagnosis would easily have been overlooked. In this case, the biopsy did not show typical histopathologic findings of VZV-vasculitis. What led the diagnosis was a PCR from the wound swab taken after the punch biopsy. This case serves as a reminder for atypical presentation of common conditions in immunosuppressed patients and that extensive diagnostic sampling may be warranted in this group.

Infectious Endocarditis Accompanied by Leukocytoclastic Vasculitis.

Bacterial endocarditis is a rare infection that can present with variable clinical manifestations. Rarely, it can present as cutaneous vasculitis characterized by a purpuric rash mimicking immune-mediated vasculitis. There have been a few case reports of leukocytoclastic vasculitis (LCV) due to infectious endocarditis. It is important to recognize endocarditis as a potential cause of vasculitis because treatment with immunosuppressive agents can have devastating consequences. We report a case of a 53-year-old male with endocarditis who developed a palpable purpura of the bilateral lower extremities. A skin biopsy was performed, and histopathologic and immunofluorescence studies demonstrated LCV.

The role of split-thickness skin grafting in the treatment of vasculitic and pyoderma gangrenosum ulcers in a multidisciplinary wound centre.

Vasculitic and pyoderma gangrenosum ulcers are traditionally treated with immunosuppressants, and the role of surgery in the treatment of these atypical ulcers remains unclear. This study aimed to investigate the need for surgical intervention as well as the outcome and safety of skin grafting in the treatment of 46 patients with vasculitic ulcers and 34 with pyoderma gangrenosum ulcers using data recorded in the validated Wound Registry. Of the 80 patients with atypical ulcers, 14% (n = 11) were treated surgically; these patients were older (p = 0.039), had lower mobility status (p = 0.002), and more often pulmonary diseases, rheumatoid arthritis, and previous arterial procedures (p = 0.007; p = 0.031; p = 0.031, respectively) than those treated conservatively. Of 181 ulcers, 15% (n = 27) were surgically treated, 78% once and 22% multiple times. During follow-up, 92.3% of both surgically and conservatively treated ulcers with available data healed. Of the surgically treated ulcers, median healing time after first surgical procedure was 96 days, and post-surgical complications were considered mild or unrelated to surgery. Our results suggest that if surgery is indicated, skin grafting is a safe and efficient treatment method provided that multidisciplinary approach is applied.

Juvenile dermatomyositis with central nervous system involvement: two case reports from a retrospective single-center cohort, with literature review.

Background: Juvenile dermatomyositis (JDM) is a systemic autoimmune disease primarily involving the muscles and skin; it can also affect the central nervous system (CNS). The relevant literature provides limited information regarding the characteristics of JDM with CNS involvement. Method: We reviewed patients with JDM who were hospitalized at our center between January 2016 and August 2023, with a focus on those with CNS involvement. The aim was to provide detailed case reports on these patients, and to summarize the relevant literature about the characteristics of similar cases. Results: Among 193 hospitalized patients with JDM, two (1.03%) had CNS involvement. Two patients, a 5.5-year-old girl and an 11-year-old boy, were admitted with severe proximal muscle weakness and seizures, and presented with active cutaneous vasculitis. Both were ultimately diagnosed with JDM, with CNS involvement. Both patients had confirmed presence of anti-NXP2 antibody through myositis-specific antibody analysis. Additionally, they all exhibited hyperferritinemia and thrombocytopenia. Salvage therapies like intravenous methylprednisolone (IVMP) pulse therapy and/or plasma exchange were administered successfully. At final follow-up, both patients had achieved complete clinical response and full neurological recovery. Our literature review identified nine similar case studies. CNS involvement usually occurred within the first 10 months of the disease course, and most of these patients had fatal outcomes, with a mortality rate of 66.6% (6/9). Including the two patients described herein, the median age for disease onset is 10.5 years (range 4-17 years), and the male: female ratio is 6:5. Seizures are the most common neurological symptom, accompanied by active cutaneous vasculitis. The brain biopsies showed two distinct pathological presentations: one was central nervous system vasculitis, and the other was cerebral macrophage activation syndrome. Conclusions: CNS involvement is a rare but life-threatening JDM complication. Herein, our cases and the literature indicate that it typically occurs within the first 10 months of the disease course and manifests as seizures, often accompanied by active cutaneous vasculitis, with fatal outcomes. Timely implementation of salvage therapies, like IVMP pulse therapy and plasma exchange, may significantly impact patient outcomes.

Semaglutide-Induced Lupus Erythematosus With Multiorgan Involvement.

We report the case of a 76-year-old female who presented with a new onset of petechial rash in her lower extremities after the introduction of a new agent, semaglutide. She started taking this medication three months before her presentation at an initial dosage of 0.5 mg subcutaneously every week. She noticed a 15-pound weight loss and debilitating fatigue within that timeframe. She stopped taking the medication due to nontolerance and GI upset (nausea and vomiting) about a week before her hospitalization. She denied the use of any other agents. Initial lab work revealed elevated transaminases, alkaline phosphatase, total bilirubin, and inflammatory markers. A CT of the abdomen revealed mild cirrhosis and hepatosplenomegaly. Other causes for cirrhosis were effectively ruled out with negative viral hepatitis, ceruloplasmin levels, and the HFE gene. An autoimmune panel was conducted, yielding positive antinuclear antibody (ANA), anti-histone antibodies, elevated double-stranded DNA, as well as low complement levels supporting evidence of drug-induced lupus (DIL). Anti-mitochondrial M2 and anti-smooth antibodies were also detected, indicating a possible overlap syndrome with autoimmune hepatitis. Perinuclear anti-neutrophil cytoplasmic antibodies (P-ANCA) and anti-neutrophil cytoplasmic autoantibodies (C-ANCA) were negative and ruled out the possibility of ANCA-associated vasculitis. The patient's condition improved with pulse-dose steroids, leading to an improvement in liver function tests. Consequently, the decision to perform skin and liver biopsies was deferred. She was discharged with a tapering dose of steroids and scheduled for outpatient follow-up to monitor her progress. This case report can offer insights to healthcare providers regarding the potential side effects of GLP-1 RAs in their patient population.

Leukocytoclastic vasculitis in a patient with ankylosing spondylitis: A case report.

Key Clinical Message: Although the concurrent occurrence of vasculitis with AS is uncommon, when patients diagnosed with AS exhibit symptoms including skin petechiae, purpura, abdominal discomfort, malaise, elevated ESR, and reduced complement levels, vigilant monitoring for vasculitis is advisable following the exclusion of secondary vasculitis triggers such as malignancies, infections, and pharmaceutical agents. Abstract: The primary characteristic of ankylosing spondylitis (AS) involves inflammation occurring within the sacroiliac joint and the spine, leading to destruction and eventual ankylosis. A notably infrequent complication associated with AS is vasculitis, with limited reports linking AS to vasculitis. This case study documents a 48-year-old male, diagnosed with HLA-B27-positive AS for the past 15 years, who developed abdominal pain and skin lesions following the cessation of his medication on his own. Subsequent clinical evaluations identified leukocytoclastic vasculitis (LCV) related to AS after excluding all other potential causes of LCV, including drug-related sources, cancer, hepatitis B and C viruses, Henoch-Schönlein purpura (HSP), and IgA nephropathy.

Lupus Vasculitis.

We present the case of a 30-year-old woman who presented with 8-month history of intermittent fever, joint pains with morning stiffness, recurrent oral ulcers, photosensitivity, weight loss and hair fall. For the last 2 months, she had developed a dry cough with progressive shortening of breath. On examination, a cachexic lady with malar hyperpigmentation, alopecia, pallor, nail dystrophy and erythema over her hands and feet were noted. There were multiple punched-out skin ulcers of variable size over legs, arms and abdomen usually round in shape with well-defined even wound margins and scant serous discharge. Musculoskeletal examination revealed synovitis of both elbows and a few metacarpophalangeal and proximal interphalangeal joints. Chest X-ray and HRCT showed bilateral ground-glass opacification. Anti-Nuclear Antibody (ANA) was positive, 1:320, homogenous nuclear pattern. Anti-Ro antibody was highly positive and serum complement (C3, C4) levels were reduced. She was diagnosed with Lupus Vasculitis and started on steroids, mycophenolate mofetil and hydroxychloroquine.

The Lack of a Durable Response to Tofacitinib in Anti-PL-12 Anti-synthetase Syndrome With Progressive Interstitial Pneumonitis.

We discuss a case of a 48-year-old female with anti-PL-12 anti-synthetase syndrome. She presented with dermatitis and myositis and developed rapidly progressive interstitial lung disease (ILD) while on prednisone and azathioprine, which responded dramatically to the addition of tofacitinib. However, the patient later developed arthritis, worsening skin disease, cutaneous vasculitis, and worsening ILD with corticosteroid reduction. The response to a standard dose of tofacitinib, although dramatic in the subacute phase of ILD, was not durable in terms of preventing a flare-up of skin disease, cutaneous vasculitis, arthritis, and the slow progression of pulmonary fibrosis as corticosteroids were tapered. Rituximab and nintedanib were then sequentially added to her therapy. Our experience suggests that rather than sequential addition, targeted triple therapy with tofacitinib, rituximab, and an antifibrotic should be considered early in the disease course in patients with dermatomyositis and severe ILD.

SARS-COV-2 MRNA vaccine-associated cutaneous vasculitis.

During the coronavirus disease 2019 pandemic, global approach was to isolate populations with quarantine procedures to reduce the spread of this deadly virus until effective treatments are found or vaccines are developed. mRNA-based vaccines became available in the United States in March 2020. The Food and Drug Administration even issued an Emergency Use Authorization for individuals 16 years and older in December 2020. However, these rapid developments have brought along other problems such as possible side effects. As we develop and test a new treatment, it became clear how important side-effect management is. Here, we present a case of cutaneous vasculitis that developed on the fourth day of SARS-CoV-2 mRNA vaccination. The patient was successfully treated with medium-dose methylprednisolone.

Vasculitis-Like Hemorrhagic Herpes Zoster and HIV Infections: An Intricate Association.

Herein, we report the case of a 69-year-old patient who presented to our dermatology clinic for a skin eruption characterized by grouped hemorrhagic vesicles and erosions covered by hemorrhagic crusts on an erythematous background located on the lower right limb. The lesions were small, clustered, and variable in size (diameters between one and 10 mm) and located at the level of the L4-L5 dermatomes. The rash had started three to five days after the complete COVID-19 vaccination scheme with the BNT162b2 Pfizer BioNTech vaccine and had been accompanied by a flu-like syndrome. The histopathological examination established the diagnosis of leukocytoclastic vasculitis potentially in the context of a cytopathic zoster phenomenon. The atypical aspect of the zosterian eruption required additional laboratory work-up to identify possible causes of immunosuppression, i.e., screening for the presence of the human immunodeficiency virus (HIV) infection, solid cancers, as well as measurement of serum immunoglobulin concentrations, which revealed that the subject was HIV-positive. Antiviral treatment was started, with a favorable evolution of the lesions, and the patient was referred to an infectious diseases clinic for initiation of antiretroviral therapy (ART).

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.

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

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.