Cutaneous polyarteritis nodosa: An atypical simulator. Report of 3 cases and a narrative review of the literature

Objectives: To report the clinical manifestations and therapeutic schemes established in three patients with cutaneous polyarteritis nodosa, as well as to describe the pathophysiology, clinical features, diagnostic criteria, and therapeutic options. Methods: A literature review was performed using Google scholar and PubMed and MeSH terms. There was no limit on the publication date or language for the selection of the articles. Results: Cutaneous polyarteritis nodosa is an uncommon small and medium-sized vessel vasculitis, and is rarely reported in Colombia. Although its pathophysiology is unknown, it is considered to be mediated by the deposition of immunocomplexes in the walls of blood vessels. It can be triggered by infectious agents and be associated with autoimmune diseases. The cutaneous manifestations mainly include subcutaneous painful nodules, livedo reticularis, and ulcers. Myalgia, arthralgia, peripheral neuropathy, and fever can also be present. It represents a diagnostic challenge. Treatment is not standardized and is guided according to the severity. Conclusions: Cutaneous polyarteritis nodosa is a rare entity, with a wide variety of cutaneous manifestations. There is still no specific diagnostic test. Its diagnosis represents a challenge for the dermatologist, and requires multidisciplinary management, in which the rheumatologist plays a fundamental role.

Objetivos: Reportar las diferentes manifestaciones clínicas y los esquemas terapéuticos instaurados en 3 pacientes con poliarteritis nudosa cutánea. Así mismo, describir la fisiopatología, el cuadro clínico, los criterios diagnósticos y las opciones terapéuticas. Métodos: Se realizó una búsqueda de la literatura en Google Académico y PubMed utilizando términos MeSH. En la selección de la bibliografía, la búsqueda no se limitó por fecha de publicación ni por idioma, debido al escaso número de reportes. Resultados: La poliarteritis nudosa cutánea es una vasculitis de pequeño y mediano vaso, infrecuente, poco reportada en Colombia. Su fisiopatología no es del todo conocida, se considera mediada por el depósito de inmunocomplejos en las paredes de vasos sanguíneos; puede ser desencadenada por agentes infecciosos y asociarse con enfermedades autoinmunes. Las manifestaciones cutáneas incluyen principalmente nódulos subcutáneos dolorosos, livedo reticular y úlceras. A nivel extracutáneo podrían encontrarse mialgias, artralgias, neuropatía periférica y fiebre. El diagnóstico representa un desafío. El tratamiento no está estandarizado y es guiado según la severidad de la patología. Conclusión: La poliarteritis nudosa cutánea es una entidad poco frecuente, con una amplia variedad de manifestaciones cutáneas. Hasta el momento, no contamos con una prueba diagnóstica específica. Su diagnóstico representa un reto para el dermatólogo y requiere un manejo multidisciplinario, en el cual el reumatólogo desempeña un papel fundamental.

Polyarteritis nodosa / Periarteritis nodosa

Abstract Polyarteritis nodosa was first described in 1866 by Zarco. Histologically, it is characterized by necrosis of the medium-sized arteries. Clinical case: A 63-year-old patient presented with myalgia, blurred vision, paresthesias and loss of muscle strength. On physical exam, he had hypesthesia in the left foot. Paraclinical studies ruled out small vessel vasculitis, and, in the end, the biopsy was compatible with polyarteritis nodosa. Discussion: this is a low prevalence entity with widely variable clinical manifestations. Therefore, the American College of Rheumatology criteria must be used for diagnosis, keeping in mind that the gold standard is histopathology. It is treated with immunosuppressants and the patient's prognosis is determined through the assessment of four criteria.(Acta Med Colomb 2020; 45. DOI:https://doi.org/10.36104/amc.2020.1388).

Resumen La poliarteritis nodosa fue descrita por primera vez en 1866 por Zarco. Histológicamente se caracteriza por la presencia de necrosis de las arterias de mediano calibre. Caso clínico: paciente de 63 años con mialgias, visión borrosa, parestesias y pérdida de fuerza muscular. Al examen físico con hipoestesia en pie izquierdo. Los paraclínicos descartaron vasculitis de pequeño vasos, finalmente la biopsia era compatible con poliarteritis nodosa. Discusión: es una entidad de baja prevalencia, la cual tiene una amplia variabilidad en cuanto a manifestaciones clínicas, por lo cual es necesario usar los criterios del Colegio Americano de Reumatología para el diagnóstico, teniendo en cuenta que el gold estándar es el estudio histopatológico. El tratamiento se da con medicamentos inmunosupresores y el pronóstico del paciente se establece mediante la evaluación de cuatro criterios.(Acta Med Colomb 2020; 45. DOI:http://doi.org/10.36104/amc.2020.1388).

Poliarterite nodosa e osteonecrose em paciente feminino como afecção rara em atenção primária à saúde / Nodosa polyarteritis and osteonecrosis in a female patient as a rare condition in primary health care

A poliartrite nodosa (PAN) faz parte de um grupo de patologias autoimunes caracterizadas pela vasculite. Quando associada à osteonecrose, tal comorbidade torna-se ainda mais rara, sendo mais comum em indivíduos do sexo masculino e com manifestações clínicas variadas. O presente estudo trata-se de um relato de caso de uma paciente de 40 anos do sexo feminino que apresentou PAN, a qual se agravou desenvolvendo osteonecrose na articulação coxofemoral bilateral. O tratamento por meio da terapia com corticoides associados a ciclofosfamida, apesar de necessitar monitorização frequente, tem se mostrado eficaz na redução do impacto da morbidade sobre o bem estar do paciente. O quadro apresenta um prognóstico complexo e de difícil reconhecimento visto sua raridade, sintomatologia variada e diagnóstico histopatológico.

Polyarteritis nodosa (PAN) is part of a group of autoimmune disorders characterized by vasculitis. When associated with osteonecrosis, such comorbidity becomes even rarer, being more common in males. Its clinical manifestations are varied. The present study is a case report of a 40-year-old female patient who presented with PAN, which was aggravated by developing osteonecrosis on the bilateral hip joint. Treatment with corticosteroid therapy associated with cyclophosphamide, despite requiring frequent monitoring, has been shown to be effective in reducing the impact of morbidity on patient's well-being. The condition has a complex prognosis and is difficult to recognize due to its rarity, varied symptoms, and histopathological diagnosis.

Compromiso vesical en paciente pediátrico con poliarteritis nodosa: primera descripción / Bladder involvement in a paediatric patient with polyarteritis nodosa: First description

RESUMEN La poliarteritis nudosa (PAN) es una vasculitis necrosante, rara en la infancia, caracterizada por el compromiso de vasos pequeños/medianos y de múltiples órganos. Presentamos a una paciente que inició a los 4 arios con síndrome febril prolongado, dolor abdominal crónico, mialgias incapacitantes y compromiso en la piel, quien luego de 2 años de cuadro clínico completa criterios clínicos para PAN. Recibió tratamiento con corticoide sistêmico por vía oral e intravenosa, 6 meses de ciclofosfamida por vía intravenosa y manejo de mantenimiento con inmunosupresores convencionales sin respuesta adecuada, logrando control de la enfermedad únicamente con ciclofosfamida por vía oral y corticoide a largo plazo. Luego de 5 años y de recibir una dosis alta acumulada de ciclofosfamida, inicia con cuadros de hematuria macroscópica. Se evaluaron, entre otras causas, la toxicidad por ciclofosfamida y la actividad de la enfermedad. El estudio incluyó biopsia vesical, con hallazgo de vas-culitis necrosante de paredes vesicales. La vasculitis vesical es raramente reportada en la literatura (3-5 casos en adultos) y en lo consultado no hay reportes en niños. Se describe, en nuestro conocimiento, el primer caso de compromiso vesical asociado a vasculitis sistêmica reportado en la edad pediátrica.

A B S T R A C T Polyarteritis nodosa (PAN) is a necrotising vasculitis, rare in childhood, and characterized by the inflammation of small and medium vessels and multiple organ involvement. The case is presented of a 4 year old girl with prolonged febrile syndrome, chronic abdominal pain, disabling myalgia, and skin involvement. After 2years of symptoms, she met clinical criteria for PAN. She received treatment with oral and intravenous systemic corticosteroids, 6 months of intravenous cyclophosphamide and maintenance with conventional immuno-suppressants without an adequate response. However, she showed clinical improvement with oral cyclophosphamide and long-term corticosteroids. She had several relapses during follow-up visits due to irregular treatment requiring a high cumulative dose of cyclophosphamide. Five years later she presented with macroscopic haematuria, and was assessed for, among other causes, cyclophosphamide toxicity and disease activity. The workup included cystoscopy and bladder biopsy with findingof necrotising vasculitis of bladder wall. Bladder vasculitis is rarely reported in the literature (3-5 cases in adults) and in that consulted there are no reports in children. To our knowledge, this is the first case of bladder involvement associated with systemic vasculitis reported in the paediatric age.

Is macular lymphocytic arteritis limited to the skin? Long-term follow-up of seven patients

Abstract Background: Macular lymphocytic arteritis most commonly presents as hyperpigmented macules on the lower limbs. The pathogenesis of this disease is still unclear and there is an ongoing debate regarding whether it represents a new form of cutaneous vasculitis or an indolent form of cutaneous polyarteritis nodosa. Objective: To describe clinical, histopathological, and laboratory findings of patients with the diagnosis of macular lymphocytic arteritis. Methods: A retrospective search was conducted by reviewing cases followed at the Vasculitis Clinic of the Dermatology Department, School of Medicine, University of São Paulo, between 2005 and 2017. Seven patients were included. Results: All cases were female, aged 9-46 years, and had hyperpigmented macules mainly on the legs. Three patients reported symptoms. Skin biopsies evidencing a predominantly lymphocytic infiltrate affecting arterioles at the dermal subcutaneous junction were found, as well as a typical luminal fibrin ring. None of the patients developed necrotic ulcers, neurological damage, or systemic manifestations. The follow-up ranged from 18 to 151 months, with a mean duration of 79 months. Study limitations: This study is subject to a number of limitations: small sample of patients, besides having a retrospective and uncontrolled study design. Conclusions: To the best of the authors' knowledge, this series presents the longest duration of follow-up reported to date. During this period, none of the patients showed resolution of the lesions despite treatment, nor did any progress to systemic vasculitis. Similarities between clinical and skin biopsy findings support the hypothesis that macular lymphocytic arteritis is a benign, incomplete, and less aggressive form of cutaneous polyarteritis nodosa.

Report of a case of probable overlap between systemic erythematous lupus and polyarteritis nodosa / Reporte de un caso de probable overlap de lupus eritematoso sistémico con panarteritis nudosa

ABSTRACT Systemic lupus erythematosus (SLE) is a heterogeneous autoimmune disease with a wide range of clinical manifestations that may affect any organ. Polyarteritis nodosa (PAN) is defined as necrotizing inflammatory changes in the medium and small vessels, a rare form of systemic necrotizing vasculitis in childhood. This article discusses the case of a patient with a history of deep venous thrombosis of the left leg, who presented with erythematosus purple lesions in her right hand, associated with pain, intermittent claudication, progressive limping and generalized edema. While in hospital, she was diagnosed with SLE with renal involvement and medium vessel vasculitis mainly of the upper limbs. She also met the criteria for PAN, a rare association that is seldom described in the medical literature.

RESUMEN El lupus eritematoso sistémico (LES) es una enfermedad autoinmune heterogénea con una amplia variedad de manifestaciones clínicas que pueden afectar cualquier órgano. La panarteritis nudosa (PAN) se define como cambios inflamatorios necrotizantes en arterias medianas o pequenas, siendo una vasculitis necrotizante sistêmica rara en la infancia. Presentamos el caso de una paciente con antecedente de trombosis venosa profunda del miembro inferior izquierdo, que presenta lesiones violáceas eritematosas en la mano derecha, asociadas a dolor, claudicación intermitente, limitación funcional progresiva, así como edema generalizado. Durante la hospitalización se llega al diagnóstico de LES con compromiso renal y vasculitis de vasos medianos con predominio de miembros superiores que cumple criterios de PAN, asociación rara muy poco descrita en la bibliografía.

Manifestaciones imagenológicas pulmonares y abdominales de las vasculitis primarias / Imaging of Pulmonary and Abdominal Manifestations of Primary Vasculitis

Las vasculitis son un grupo de enfermedades que consisten en la inflamación dentro y alrededor de la pared de los vasos sanguíneos, lo que produce necrosis, y su extensión depende del tamaño y ubicación del vaso afectado. Se pueden manifestar en cualquier órgano, por lo que se consideran enfermedades sistémicas. Las vasculitis primarias son patologías poco comunes y difíciles de diagnosticar, porque sus signos y síntomas son inespecíficos y pueden confundirse con muchas otras patologías ­como enfermedades infecciosas, neoplásicas o del tejido conjuntivo­. Por esta razón, para su diagnóstico es importante combinar los hallazgos clínicos, radiológicos, de estudios histopatológicos y de laboratorio. Esta revisión se enfocará en el papel de los hallazgos imagenológicos en el diagnóstico y monitoreo de las manifestaciones pulmonares y abdominales de las vasculitis más frecuentes.

Vasculitis are a group of diseases that are characterized by the presence of inflammation in and around the wall of the blood vessels. This produces necrosis and inflammation where the extension of the injury depends on the size and location of the affected vessel. They can have manifestations in any organ, so they are considered systemic diseases. Primary vasculitis are uncommon pathologies and difficult to diagnose because its signs and symptoms are nonspecific and are easily confused with many other disease processes such as infectious, neoplastic or connective tissue. Because of this, it is important to combine clinical, radiological, histopathological and laboratory findings to achieve a correct diagnosis. In this review, we will focus on the role of imaging findings on the diagnosis and monitoring for pulmonary and abdominal manifestations of the most frequent vasculitis.

Trastorno de excoriación en una mujer con panarteritis nudosa. Reporte de caso / Skin Picking Disorder in a Woman with Polyarteritis Nodosa. Case Report

RESUMEN Introducción: El trastorno por excoriación está incluido en el DSM - 5 dentro de la categoría Recibido el 4 de abril de 2017 de trastorno obsesivo compulsivo y trastornos relacionados. Se define como la urgencia Aceptado el 18 de marzo de 2018 de tocar, rascar, frotar, restregar, friccionar, apretar, morder o excavar la piel de forma On-line el 7 de mayo de 2018 recurrente hasta producirse lesiones cutáneas. Es un trastorno poco frecuente (1.4 - 5.4% de la población) y se presenta principalmente en mujeres. Presentación de caso: Se presenta el caso de una mujer de 31 años quien fue valorada por dermatología y ortopedia por presencia de lesiones ulceradas e infectadas en miembros inferiores, junto con otras lesiones superficiales por rascado en tórax, brazos, antebrazos, espalda y cabeza; además reportando síntomas ansiosos, razón por la cual es valorada por el servicio de Psiquiatría de enlace Discusión: El rascado cutáneo, conducta normal en los mamíferos, cobra valor patológico desde el punto de vista psiquiátrico al ser un acto repetitivo y persistente, como la conducta que se presenta en el trastorno por excoriación. Dada la relación descrita con el espectro obsesivo - compulsivo, se recomienda el uso de inhibidores selectivos de la recaptación de serotonina y la terapia cognitivo conductual.

ABSTRACT Introduction: Excoriation (skin picking) disorder is included in the DSM-5 in the obsessive compulsive and related disorders category. It is defined as the recurrent urge to touch, scratch, scrape, scrub, rub, squeeze, bite or dig in the skin, leading to skin lesions. It is a rare disorder (1.4-5.4% of the population) and occurs mainly in women. Case report: this article reports the case of a 31-year-old female patient, initially assessed by dermatology and orthopaedics for the presence of infected ulcerated lesions on her lower limbs, with other superficial lesions from scratching on her chest, arms, forearms, back and head. The patient also reported symptoms of anxiety, so was assessed by consultation-liaison psychiatry. Discussion: skin picking, normal behaviour in mammals, becomes pathological from a psychiatric point of view when it is repetitive and persistent, as in the case of excoriation disorder. In view of the reported relationship with the obsessive-compulsive spectrum, use of selective serotonin reuptake inhibitors and cognitive behavioural therapy are recommended.

Multifocal myopathy in a patient with polyarteritis nodosa; usefulness of magnetic nuclear resonance as a diagnostic test / Miopatia multifocal en paciente con poliarteritis nodosa: utilidad de la resonancia nuclear magnética como prueba diagnóstica

ABSTRACT Polyarteritis nodosa is part of the primary systemic vasculitis that specifically compromises vessels of medium caliber, and can affect virtually any organ. The diagnosis of this disease is based on clinical criteria, such as pain and weakness in the lower limbs, as well as laboratory results and the histology report that shows necrotizing, segmental and focal inflammation of the vessels involved. The case is presented of a 42 year-old woman with a previous diagnosis of polyarteritis nodosa, who, 12 years later, presented with an episode of activation of the disease associated with venous thrombosis and multifocal myopathy in the lower limbs. This is a rare presentation that should be suspected in this type of patients.

RESUMEN La poliarteritis nodosa hace parte de las vasculitis sistémicas primarias, específicamente compromete vasos de mediano calibre pudiendo afectar virtualmente a cualquier órgano. El diagnóstico de esta enfermedad se basa en criterios clínicos, como el dolor y la debilidad en los miembros inferiores, además de reportes paraclínicos y el compromiso histológico, que evidencia inflamación necrosante, segmentaria y focal de los vasos involucrados. A continuación, presentamos el caso clínico de una mujer de 42 arios, con diagnóstico previo de poliarteritis nodosa, que presenta, 12 años después, un episodio de activación de la enfermedad asociado a trombosis venosa y miopatía multifocal en miembros inferiores, una presentación poco frecuente pero que debe sospecharse en este tipo de pacientes.

Panniculitides of particular interest to the rheumatologist

Abstract The panniculitides remain as one of the most challenging areas for clinicians, as they comprise a heterogeneous group of inflammatory diseases involving the subcutaneous fat with potentially-shared clinical and histopathological features. Clinically, most panniculitides present as red edematous nodules or plaques. Therefore, in addition to a detailed clinical history, a large scalpel biopsy of a recent-stage lesion with adequate representation of the subcutaneous tissue is essential to specific diagnosis and appropriate clinical management. Herein we review the panniculitides of particular interest to the rheumatologist.

Vasculitic Neuropathy in Cutaneous Polyarteritis Nodosa

No abstract available.

Hybrid Surgery to Treat Multiple Visceral Aneurysms Secondary to Polyarteritis Nodosa

A 57-year-old woman presented to vascular surgery clinic with visceral artery aneurysms that were incidentally detected during regular check-up. Imaging studies revealed occlusion of the celiac axis and severe stenosis of the superior mesenteric artery and 3 aneurysms along the posterior and inferior pancreaticoduodenal arteries, as well as the right gastroepiploic artery. Endovascular embolization of all aneurysms was rejected because of the risk of hepatic ischemia. These complicated lesion caused by polyarteritis nodosa were successfully treated using a hybrid operation with coil embolization, aneurysm resection, and antegrade aorto-celiac-superior mesentery artery bypass.

Successful Treatment of Refractory Cutaneous Polyarteritis Nodosa with Adalimumab

Cutaneous polyarteritis nodosa (CPAN) is a form of necrotizing vasculitis of the medium and small-sized arteries. The condition is limited to the skin and there is a lack of visceral involvement. Treatment with systemic glucocorticoids alone or in combination with azathioprine, methotrexate or cyclophosphamide, depending on the disease severity, has been shown to be effective. This paper reports the clinical case of a 53-year-old female patient with CPAN refractory to treatment with high dose glucocorticoid, methotrexate, azathioprine, and cyclophosphamide, who was treated successfully with anti-tumor necrosis factor-α therapy (adalimumab).

Poliarteritis nodosa cutánea / CUTANEOUS POLYARTERITIS NODOSA

La poliarteritis nodosa es una vasculitis de los vasos de mediano calibre que puede afectar solamente la piel o tener compromiso sistémico. Los signos cutáneos más frecuentes son livedo reticularis, nódulos y ulceras dolorosas. Se presenta un paciente masculino de 40 años de edad que consultó por nódulos eritematovioláceos dolorosos en miembros de un mes de evolución. Se realizó biopsia sugestiva de poliarteristis nodosa, se detectó serología para VIH positiva, cavitaciones pulmonares en TAC de tórax y lavado broncoalveolar con baciloscopía positiva para tuberculosis (TBC). Se inicia tratamiento antifímico y antiretroviral con mejoría de la clínica respiratoria y resolución a los 2 meses de las lesiones en piel

Polyarteritis nodosa is a vasculitis of medium sized vessels that can affect only skin or have systemic involvement. The most frequent cutaneous signs are livedo reticularis, nodules and painful ulcers. We present a 40 year-old male patient who consulted for painful erythematouspelvic nodules in a month of evolution. A biopsy suggestive of polyarteritis nodosa was detected, positive HIV serology, pulmonary cavitations in chest CT and bronchoalveolar lavage with tuberculosis-positive smear microscopy (TB). Antimicrobial and antiretroviral treatment with respiratory clinic improvement and resolution at 2 months of skin lesions are started

Poliarteritis nudosa asociada a una infección por citomegalovirus en una fiebre de origen desconocido / Polyarteritis nodosa associated with a cytomegalovirus infection in a fever of unknown origin

Un paciente de 26 años de edad fue ingresado por fiebre de origen desconocido y lesiones nodulares en la piel, malestar general, artritis simétrica y bilateral, dolor testicular y debilidad muscular. Una biopsia de piel reveló necrosis fibrinoide con infiltrado de neutrófilos en la pared de medianas y pequeñas arterias. La serología para citomegalovirus fue positiva; el antígeno de superficie al virus de la hepatitis B y el anticuerpo contra el virus de la hepatitis C resultaron negativos. Se diagnosticó una poliarteritis nudosa asociada a una infección por citomegalovirus. Sus síntomas desaparecieron rápidamente con tratamiento de prednisolona (60 mg oral) y ganciclovir endovenoso, lo que enfatiza la importancia de tener en cuenta la infección por citomegalovirus en la patogenia de la poliarteritis.

Manifestaciones clínicas de la esclerosis de Monckeberg. Reporte de caso y revisión de la literatura / Clinical manifestations of Monckeberg's sclerosis. Report of case and literature review

Resumen La esclerosis de Monckeberg es una enfermedad de baja prevalencia y causa desconocida, en la cual arterias de mediano y pequeño calibre sufren calcificación de la capa media generándose una reducción de la luz. Suele manifestarse por la aparición de úlceras de tipo arterial en la piel de extremidades superiores e inferiores. Su diagnóstico requiere de estudios histopatológicos, con diagnósticos diferenciales como la poliarteritis nodosa y la arteritis de células gigantes. No existen guías de tratamiento, se han propuesto quelantes de calcio como el tiosulfato de sodio, pero los pocos estudios disponibles no han logrado demostrar resultados significativos.

Abstract Monckeberg's sclerosis is a rare and low prevalence disease of unknown cause in which small and medium size arteries suffer calcification of the middle layer, leading to a reduction in calibre. Clinically, the disease manifests by the appearance of arterial type ulcers on the skin of upper and lower extremities. Its diagnosis is difficult, and requires histopathological studies. Some rheumatic conditions, such as polyarteritis nodosa and giant cell arteritis may mimic this process. Treatment of the disease is not well established. Although the use of calcium chelators such as sodium thiosulfate has been proposed, the few available studies have failed to show significant results.

Chronic polyarthritis as the first manifestation of childhood systemic polyarteritis nodosa / Poliartrite crônica como primeira manifestação de poliarterite nodosa sistêmica pediátrica

ABSTRACT Arthritis has been reported as an acute pattern, generally evanescent with oligoarthritis, mostly affecting knees and ankles in childhood systemic polyarteritis nodosa. However, chronic polyarthritis with morning stiffness mimicking juvenile idiopathic arthritis has not been reported. We describe the case of a 4-year old girl who had additive and chronic polyarthritis with edema, tenderness, pain on motion and morning stiffness for 2 months. After 45 days, she also presented painful subcutaneous nodules and erythematous-violaceous lesions in the extensor region of upper and lower limbs. She was admitted to university hospital due to high fever, malaise, myalgia, anorexia, loss of weight (1kg), painful skin lesions and severe functional disability. She was bedridden by chronic polyarthritis with limitation on motion. Systolic and diastolic blood pressures were greater than 95th percentile for height. Urine protein/creatinine ratio was 0.39g/day, and immunological tests were negative. Anti-streptolysin O was 1,687UI/mL. Skin biopsy revealed necrotizing vasculitis in medium- and small-sized vessels compatible with polyarteritis nodosa. Therefore, we had the diagnosis of systemic polyarteritis nodosa. Prednisone 2mg/kg/day was administered with complete resolution of skin lesions and arthritis, and improvement of proteinuria (0.26g/day) after 15 days. The diagnosis of childhood systemic polyarteritis nodosa should be considered for patients with chronic polyarthritis associated to cutaneous vasculitis triggered by streptococcal infection.

RESUMO Na poliarterite nodosa sistêmica pediátrica, a artrite caracteriza-se pelo padrão agudo, geralmente evanescente, com oligoartrite, e afeta principalmente joelhos e tornozelos. No entanto, a poliartrite crônica com rigidez matinal e simulando artrite idiopática juvenil ainda não foi relatada. Descrevemos o caso de uma menina de 4 anos que apresentou poliartrite crônica aditiva com edema, dor à palpação e movimento, e rigidez matinal por 2 meses. Após 45 dias, também apresentou nódulos subcutâneos dolorosos e lesões eritêmato-violáceas na região extensora dos membros superiores e inferiores. Foi internada no hospital universitário por conta de febre alta, mal-estar, mialgia, anorexia, perda de peso (1kg), lesões de pele muito dolorosas e incapacidade funcional grave. Estava restrita ao leito devido à poliartrite crônica com limitação do movimento. Pressões sistólica e diastólica foram maiores que percentil 95 para altura. Relação proteína/creatinina urinária estava 0,39g/dia, e os testes imunológicos foram negativos. Antiestreptolisina O era 1.687UI/mL. A biópsia de pele revelou vasculite necrosante de vasos de pequeno e médio calibre, compatível com poliarterite nodosa. Portanto, foi realizado o diagnóstico de poliarterite nodosa sistêmica. Foi administrada prednisona 2mg/kg/dia com resolução completa das lesões de pele e da artrite, além de melhora da proteinúria (0,26g/dia) após 15 dias. O diagnóstico de poliarterite nodosa sistêmica pediátrica deve ser considerado em pacientes com poliartrite crônica associado a lesões cutâneas vasculíticas, sendo a infecção estreptocócica um importante fator desencadeante.

Cutaneous polyarteritis nodosa causing refractory skin deformation and pigmentation as sequel

Abstract: A 39-year-old woman presented with prominent and painful livedo reticularis lesions spreading on her upper and lower extremities. Histopathologically, the small-to medium-sized arteries in the deep dermis and subcutis showed necrotizing vasculitis with cellular infiltration, suggesting cutaneous polyarteritis nodosa. The serum levels of inflammatory markers normalized with aspirin 100mg/day and prednisolone 10mg/day within 2 months, and there was no other skin or organ involvement over 18 months of follow up. However, serious refractory skin depressions and pigmentation remained after two years of treatment. This suggests the importance of early and aggressive therapy for cutaneous polyarteritis nodosa to prevent unsightly skin sequel, as well as control of disease activity.

Panarteritis nodosa cutánea, presentación de un caso con lesiones en localización atípica / Cutaneous polyarteritis nodosa. Case presentation with lesions in an atypical location

La Panarteritis Nodosa Cutánea es una rara entidad caracterizada por nódulos subcutáneos, de curso crónico, benigno y recurrente. La mayoría de los pacientes presentan lesiones en miembros inferiores, y un tercio en los miembros superiores. Además existen manifestaciones extracutáneas como miositis o artritis. Presentamos el caso de una paciente que fue internada por tumefacción, debilidad muscular y dolor incapacitante en ambos hombros y nódulos subcutáneos en hombros y cara, a quien le habían diagnosticado panarteritis nodosa en la infancia, pero que siempre presentó lesiones en localizaciones atípicas, respetando los miembros inferiores

Four Cases of Polyarteritis Nodosa Presenting Initially as Pain and Pitting Edema in Both Lower Extremities

Polyarteritis nodosa (PAN) has a broad spectrum of clinical presentation, since it affects small and medium-sized muscular arteries with microaneurysm formation, aneurysmal rupture with hemorrhage, thrombosis, and, consequently, organ ischemia or infarction. Although skeletal muscle involvement is well documented in patients with PAN, it can mimic more common diseases, and cause confusion and delays in diagnosis. PAN muscular involvement may have limited or early systemic forms with a benign course and excellent clinical response to corticosteroid therapy. Herein, we describe the clinical course and outcome of four unusual cases of PAN manifested by acute onset of pain and pitting edema in both lower extremities; in addition, we reviewed the relevant literature.