Episodic angioedema with eosinophilia (Gleich's syndrome) associated with urticarial vasculitis: a coincidence or a novel clinical entity?

Episodic angioedema with eosinophilia (EAE) is a rare condition characterized by recurrent attacks of angioedema and urticaria accompanied by a marked elevation of peripheral eosinophil count. We report the case of a young female patient diagnosed with EAE associated with urticarial vasculitis. A 40-year-old female patient was admitted to our institution due to recurrent episodes of cheek and eyelid angioedema in the previous year. Episodes of facial angioedema lasted for two months with spontaneous remission afterwards. In addition, she presented pruritic and painful skin eruptions of erythematous circles, which persisted for longer than 24 h, that were palpable, somewhat purplish, and more pronounced on the face, arms, and trunk. Laboratory investigation showed a sustained elevation of white cell counts with marked eosinophilia. Serum IgM, IgE, and IgA were normal; IgG was slightly elevated. C1-esterase inhibitor and tryptase test were normal. Reverse transcriptase-polymerase chain reaction was performed for detection of FIP1L1-PDGFRA and BCR-ABL rearrangements. None of these alterations were found. Skin biopsies were suggestive of urticarial vasculitis. The patient was submitted to esophagogastroduodenoscopy, which showed mild chronic gastritis, with no eosinophilic infiltration. Cardiac dimensions and function were normal. Abdominal ultrasound and total body CT-scan failed to show lymphadenopathy, organomegaly, and tumors. We report the first case of association between episodic angioedema with eosinophilia and urticarial vasculitis. It is possible that both conditions share a physiopathological mechanism, suggesting that it is not just a chance association.

Urticaria vasculitis como manifestación de Lupus Eritematoso Sistémico / Urticarial Vasculitis as a manifestation of Systemic Lupus Erythematosus

Resumen La urticaria vasculitis (UV) es una vasculitis de pequeños vasos caracterizada clínicamente por lesiones urticarianas que muestran en la histología una vasculitis leucocitoclástica. Puede ser normo o hipocomplementémica, y mayormente es de etiología idiopática. Presentamos un caso de una paciente de 15 años con un cuadro de urticaria vasculitis en el contexto de un lupus eritematoso sistémico (LES).

Abstract Urticarial vasculitis (UV) is a vasculitis of small vessels clinically characterized by urticarial lesion that show a leukocytoclastic vasculitis in the histology. It may be normal or hypocomplementemic, mostly of idiopathic etiology. Here we present a case of a female patient 15 years old, who presents a clinical history of urticaria vasculitis in the context of a systemic lupus erythematosus.

Hypocomplementemic Urticarial Vasculitis Syndrome with Membranous Nephropathy: Case Report

Urticarial vasculitis is a rare disorder that principally manifests with recurrent urticarial, sometimes hemorrhagic, skin lesions and/or angioedema. Its clinical presentation is not always limited to cutaneous lesions and it can potentially affect other organs, such as the joints, lungs, kidneys, and eyes. Systemic involvement can either be present at the onset of disease or develop over time. In cases with systemic manifestations, urticarial vasculitis is more likely to be associated with a low complement level. We present the case of a teenage boy with hypocomplementemic urticarial vasculitis syndrome (HUVS) that occurred shortly following swine-origin influenza A virus infection in 2009. Afterwards, HUVS was systemically complicated with myositis and membranous nephropathy that developed several months and about 2 years after its onset, respectively. A combination of glucocorticoid and immunosuppressive agents has been used to effectively control disease activity.

Urticarial Vasculitis Associated with Mycoplasma pneumoniae / 대한피부과학회지

No abstract available.

Vasculitis urticarial hipocomplementémica: aclaraciónhistórica / Hypocomplementemic urticarial vasculitis: Clarification of a historical fact

El síndrome de vasculitis urticarial hipocomplementémica ha recibido enorme interés enla literatura médica, a partir de la publicación original en 1973, en Mayo Clinic Proceedings.El caso índice fue identificado en 1968, como entidad clínica distintiva, por uno de loscoautores de la primera publicación y de la revisión actual (JEM), que cierra un ciclo demás de 45 años. Las características clínicas diferenciales del síndrome vasculitis urticarialhipocomplementémica determinaron que el Consenso 2012 sobre Nomenclatura de lasVasculitis de la American Association of Rheumatology le asignara un lugar propio dentro delas enfermedades vasculares inflamatorias. En los últimos años y, sobre todo, en los últimosmeses, se ha reconocido el síndrome de vasculitis urticarial hipocomplementémica comouna forma monogénica de lupus eritematoso, lo cual completa la secuencia histórica de laenfermedad y la coloca dentro del espectro de trastornos del complemento.E l énfasis de la publicación está centrado en los aspectos históricos iniciales del proceso,que tienen el singular mérito de ser relatados por el principal autor y testigo, y que nohabían sido publicados hasta ahora, aunque sí conocidos de manera personal o privada porreconocidos reumatólogos e inmunológos.L a revisión histórica de la evolución del síndrome se ha fundamentado en la revisión dela literatura y en la concatenación de las observaciones ulteriores hasta finales de 2013,cuando se reconoció como una entidad específica y como una forma monogénica del lupuseritematoso.

Hypocomplementemic urticarial vasculitis has received extraordinary interest in the medical literature since the original publication in 1973 in Mayo Clinic Proceedings. The index case was identified in 1968 as a distinct clinical entity, by one of the co-authors of the first publication and of the current review, which closes a cycle of more than 45 years. The differential clinical characteristics of hypocomplementemic urticarial vasculitis syndrome determined that the 2012 consensus on nomenclature of Vasculitis of the american rheumatology association, designated the syndrome as a separate entity with its own place among the inflammatory vascular diseases. In the last few years, and particularly in the last few months, hypocomplementemic urticarial vasculitis syndrome has been recognized as a monogenic form of lupus erythematosus, an observation that completes the historical sequence of the disease, and places it among the spectrum of complement disorders.The emphasis of the article is centered on the initial historical aspects of the process, that have the unique merit of being recorded by the principal author and witness, and not previously published, albeit privately known by prominent rheumatologists and immunologists.The historical review of the evolution of the syndrome is based on a review of the literature, relating subsequent observations until the end of 2013, when the syndrome was recognized as a monogenic form of lupus erythematosus.

Plasmapheresis in a Patient With "Refractory" Urticarial Vasculitis

Immune complexes are found in the circulation of 30%-75% of patients with urticarial vasculitis and much evidence supports the role of these immune complexes in the pathogenesis of urticarial vasculitis. Plasmapheresis is effective for removing these immune complexes; however, there are few reports on the use of plasmapheresis in the treatment of urticarial vasculitis. We describe a case of "refractory" urticarial vasculitis in which the symptoms improved after plasmapheresis treatment. We suggest that plasmapheresis be considered as an option in patients with severe or treatment-resistant urticarial vasculitis.

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.

Hypocomplementemic Urticarial Vasculitis in Systemic Lupus Erythematosus

Urticarial vasculitis is characterized clinically by urticarial skin lesions and histologically by leukocytoclastic vasculitis. Hypocomplementemic urticarial vasculitis is associated with connective tissue diseases such as systemic lupus erythematosus (SLE). We report a case of urticarial vasculitis that preceded manifestations of SLE.

Isotopic Response Developed on the Striae Distensae / 대한피부과학회지

Isotopic response is the occurrence of a new unrelated disease that appears at the same location as a previously already healed disease. In 1995, Wolf et al defined a this new phenomenon and reported eight new cases with fiftyeight cases corresponding to the definition for this phenomenon in literiture. We report two cases considered as isotopic response, urticarial vasculitis and erythema multiforme developed on striae distensae, respectively.

A case of hypocomplementemic urticarial vasculitis syndrome with cold urticaria / 대한피부과학회지

A 38-year-old man presented with a 2-year history of recurreit irticaria, abdominal pain and arthralgia of his finger joints. These symptoms were not con rolled in spite of taking medication and had a tendence to be aggravated in the winter. Laboratory findings revealed hvpocomlementemia, hypergammaglobulinemia and a positive rect on with an ice cube test. Skin biopsy specimen taken from the urticarial lesion of the left upper arm showed leukocytoclastic vasculitis with perivascular neutrophilic infiltrition, nuclear dusts, and extravasation of red blood cells. We thus made the diagnosis of hypocomplementemic urtirarial vasculitis syndrome with cold urticaria. The patient was treatcd ith systemic corticosteroids and antihistamines.