Acute sarcoidosis Löfgren Syndrome treated by Chinese medicinal formula based on Syndrome Differentiation: a case - based review / Tratamiento de síndrome de Löfgren de sarcoidosis con fórmula medicinal china basada en diferenciación de síndrome: una revisión basada en casos

Löfgren syndrome (LS) is a unique acute manifestation of sarcoidosis and characterized by erythema nodosum, bilateral hilar lymphadenectasis, and/or bilateral ankle arthritis or periarthritis. A 37 - year - old female patient with LS presented with fever accompanied by multiple joint swelling and pain, nodular skin erythema, and bilateral hilar lymphadenectasis. The patient had received treatment involving non - steroidal anti - inflammatory drugs and glucocorticoids in other hospitals, but the effects were poor, and the conditions reemerged. The LS duration has lasted for more than 3 months. Following traditional Chinese medicine (TCM) treatment, syndrome differentiation as well as giving patients oral Chinese medicine decoction, the symptoms of the patient were rapidly relieved within one week and did not recur during a six - month follow - up period. This case is the first clinical report of acute sarcoidosis LS treated using T CM and reflects the significant advantages of this form of therapy in emergency treatment

El síndrome de Löfgren (LS) es una manifest ación única y aguda de sarcoidosis, caracterizada por eritrema nodoso, linfadenectasis hilar bilateral, y/o a r tritis de tobillo bilateral o periartritis. Una paciente de 37 años de sexo femenino con LS se presentó con fiebre, acompañada de inflamación y do lor múltiple de articulaciones, eritrema nodular cutáneo, y linfadenectasis hilar bilateral. La paciente recibió un tratamiento que consistió en antiinflamatorios no esteroidales y glucocorticoides en otros hospitales, pero los efectos fueron leves y las c ondiciones reemergieron. El LS ha durado más de tres meses. Siguiendo el tratamiento de medicina tradicional china (MTC), la diferenciación de síndrome, así como darles a los pacientes una decocción de medicina china por vía oral, los síntomas de la pacien te rápidamente fueron aliviados en el curso de una semana y no recidivaron durante los seis meses de un seguimiento. El caso es el primer reporte clínico de tratamiento de sarcoidosis aguda asociada a LS usando TCM y refleja las significativas ventajas de esta forma de terapia en el tratamiento de emergencia.

Sarcoidosis cutánea: preámbulo de la enfermedad sistémica / Cutaneous sarcoidosis: Preamble of the systemic disease

RESUMEN La sarcoidosis es una enfermedad granulomatosa, multisistémica, de causa desconocida y con múltiples manifestaciones clínicas. La característica histopatológica esencial son los granulomas uniformes no caseificantes con escasos linfocitos periféricos (granulomas desnudos). Se presenta el caso de un paciente masculino de 62 años que acudió a Cuerpo de Guardia por presentar tos seca, falta de aire, inflamación de ganglios axilares y lesiones en piel, en forma de placas, máculas y otras de aspecto psoriasiforme. Las manifestaciones clínicas, los resultados de los exámenes complementarios y al hacer el diagnóstico diferencial con otras afecciones que cursan con hallazgos similares, se llegó al diagnóstico de sarcoidosis sistémica. El paciente evolucionó favorablemente con el tratamiento indicado. El compromiso cutáneo de la sarcoidosis representa un desafío dada la variada presentación de esta entidad y los diagnósticos diferenciales a considerar.

ABSTRACT Sarcoidosis is a multisystemic granulomatous disease of unknown etiology with multiple clinical manifestations. The essential histopathologic feature is uniform non-caseating granulomas with few peripheral lymphocytes (naked granulomas). We present the case of a 62-year-old male patient who came to the emergency room due to a dry cough, shortness of breath, swollen axillary glands and skin lesions, in the form of plaques, macules, and others with a psoriasiform appearance. The clinical manifestations, the results of the complementary tests and when making the differential diagnosis with other conditions that present similar findings, the diagnosis of systemic sarcoidosis was reached. The patient evolved favorably with the indicated treatment. The cutaneous involvement of sarcoidosis represents a challenge given the varied presentation of this entity and the differential diagnoses to be considered.

Sarcoidose como diagnóstico diferencial de mieloma múltiplo / Sarcoidosis as a differential diagnosis of multiple myeloma

A sarcoidose caracteriza-se como doença granulomatosa que acomete diferentes órgãos humanos, especialmente os pulmões, sendo sua patogênese pouco conhecida. No caso em questão, a paciente iniciou com sintomas inespecíficos, como fraqueza, perda ponderal e tosse seca esporádica, sendo internada para extensão da propedêutica. Sugeriu-se como hipótese diagnóstica inicial possível quadro de mieloma múltiplo, tendo em vista a anemia, a disfunção renal, a hipercalcemia e, sobretudo, as lesões osteolíticas apresentadas pela paciente. Todavia, o diagnóstico de sarcoidose foi selado a partir das biópsias de medula óssea e de linfonodo inguinal, que evidenciaram mielite e linfadenite granulomatosas, respectivamente. A terapêutica instituída baseou-se na administração de corticosteroides e em medidas de redução da calcemia. A paciente recebeu alta, com melhora do quadro clínico, para acompanhamento ambulatorial da doença. Conclui-se que a sarcoidose não possui tratamento curativo, mas a terapêutica imunossupressora é eficaz no controle da progressão da enfermidade, fazendo com que o paciente tenha um prognóstico favorável.

Sarcoidosis is characterized as a granulomatous disease that affects different human organs, especially the lungs, and its pathogenesis is little known. In this case, the patient started with nonspecific symptoms, such as weakness, weight loss, and sporadic dry cough, being hospitalized for extension of the propaedeutics. The initial diagnostic hypothesis suggested was a possible case of multiple myeloma, based on the anemia, renal dysfunction, hypercalcemia and, above all, the osteolytic lesions presented by the patient. However, the diagnosis of sarcoidosis was made after bone marrow and inguinal lymph node biopsies that showed granulomatous myelitis and lymphadenitis, respectively. The therapy instituted was based on the administration of corticosteroids and on measures to reduce the level of calcium. The patient was discharged, with clinical improvement, for outpatient follow-up of the disease. It is concluded that sarcoidosis has no curative treatment, but immunosuppressive therapy is effective in controlling the progression of the disease, giving the patient a favorable prognosis.

A case of facial atrophic sarcoidosis in an adolescent, successfully treated with the combination of prednisone and hydroxychloroquine

Abstract Sarcoidosis is a multisystem granulomatous disorder of unknown aetiology. Cutaneous involvement occurs in up to 30% of patients and skin findings are often the initial presenting symptom. The facial atrophic form of sarcoidosis without associated ulceration in adolescents has rarely been described in the literature. We report a case of 13-year-old male patient with a facial atrophic sarcoidosis who was successfully treated with the combination of prednisone and hydroxychloroquine.

Cutaneous sarcoidosis: clinico-epidemiological profile of 72 patients at a tertiary hospital in São Paulo, Brazil,

Abstract Background: Sarcoidosis is a multisystem disease of unknown cause that is characterized by the presence of granulomas in various organs. Cutaneous involvement is common and the reported incidence has varied from 9% to 37%. Studies on cutaneous sarcoidosis in Brazil are lacking. Objectives: To describe the clinical and epidemiological aspects of patients with cutaneous sarcoidosis diagnosed at the Department of Dermatology of the University of São Paulo, from May 1994 to March 2018. Methods: Clinical data of patients with confirmed cutaneous sarcoidosis were retrospectively reviewed and classified according to gender, ethnicity, age at diagnosis, cutaneous presentation, systemic involvement and treatment. Results: Cutaneous sarcoidosis was diagnosed in 72 patients with a female predominance (74%). The mean age at diagnosis was 49.6 years and most of the patients were white (61%). Papules and plaques were the most common lesions. Systemic sarcoidosis was detected in 81% of patients, affecting mainly the lungs and thoracic lymph nodes (97%). Typically, cutaneous lesions were the first manifestation (74%). Systemic therapy was necessary for 72% of patients; the dermatologist managed many of these cases. Oral glucocorticoids were the most commonly used systemic medication (92%). The mean number of systemic drugs used was 1.98 per patient. Limitations: Insufficient data in medical records. Conclusions: This series highlights the dermatologist role in recognizing and diagnosing cutaneous sarcoidosis, evaluating patients for systemic disease involvement and treating the skin manifestations. Cutaneous sarcoidosis was once considered exceedingly infrequent in Brazil in comparison to infectious granulomatous diseases; however, the present series seems to suggest that the disease is not so rare in this region.

¿Cuándo solicitar PET/CT en la evaluación de sarcoidosis? / When should a PET/CT be requested in sarcoidosis?

Sarcoidosis is a multisystemic disease of variable course that mainly affects thoracic lymph nodes and lung parenchyma. However, any organ can be involved. There is no single test for the definitive diagnosis or a precise method to evaluate the course of the disease. PET/CT is not included in the standard workup for sarcoidosis, but it may assess the inflammatory activity, detect extrapulmonary disease such as cardiac involvement and evaluate the response to treatment. It is important to be aware of the optimal timing to request a PET/CT in sarcoidosis and which patients would benefit with this procedure.

Childhood Sarcoidosis mimicking tuberculosis: a case report

Abstract Sarcoidosis is a rare multisystem chronic inflammatory disease in children. We present a case of a five-year-old child with clinical features mimicking several diseases, including tuberculosis. After failure of treatment based on the suspected diagnosis, an axillary lymph node biopsy showed noncaseating granulomas compatible with sarcoidosis and appropriate treatment was then started.

Sarcoidosis and chronic hepatitis C: treatment with prednisone and colchicine

Abstract Sarcoidosis is a disease which still has uncertain etiology. Possible environmental causes are cited in the literature, like organic and inorganic particles and infectious agents. Recent studies have demonstrated the occurrence of sarcoidosis in patients with chronic C hepatitis; however, this association remains without statistical or causal evidence. In this report a case of sarcoidosis associated with chronic hepatitis C will be described, with subcutaneous lesions, considered rare, and good response to treatment with colchicine and prednisone. The hepatitis C virus was isolated in sarcoid tissue and the association between the two diseases will be discussed.

Tuberculosis - sarcoidosis, un desafío diagnóstico: reporte de un caso / Tuberculosis - sarcoidosis, a challenge diagnosis: case report

Introducción: La tuberculosis (TBC) y la sarcoidosis presentan similitudes en cuanto a su manifestación clínica, radiología e histología. El término tuberculosis-sarcoidosis, hace referencia a una entidad con diferentes manifestaciones, en donde la TBC puede preceder, seguir o coexistir con el curso de la sarcoidosis. Presentación del caso: Mujer de 47 años, con antecedentes de miomectomía y colecistectomía, con historia de cuatro meses de dolor abdominal tipo cólico, de localización difusa asociado a hiporexia y baja de peso de 15 kilogramos. Paciente consultó por empeoramiento de cuadro inicial, acompañado de cefalea frontal intensa, quedando hospitalizada para estudio por especialistas. Evolucionó el primer día con paresia facial periférica derecha, compromiso del trigémino izquierdo V1-V2, hipoestesia abdominal izquierda T10 a L1, síntomas deglutorios del nervio gloso-cutáneo lateral izquierdo. Se decidió continuar estudio con punción lumbar, informándose: proteínas: 0,81 mg/dl, glucosa: 62 mg/dl, 100% mononucleares, tinción Ziehl Nielsen y Gram negativa. Tomografía de tórax identificó adenopatías mediastínicas. Se realizó biopsia ganglionar mediastínica que informó focos de necrosis caseosa con tinción Ziehl Nielsen (+). Se decidió inicio de tratamiento antituberculoso y corticoidal, evolucionando al mes en malas condiciones: con dolor neuropático costal y uveítis bilateral, por ello se decidió agregar metrotrexato (MTX). Por nula respuesta al tratamiento y por tratarse de una enfermedad resistente a corticoides y MTX, se decidió manejo con infliximab, con buena respuesta clínica. Discusión: Estas entidades pueden ser confundidas con una serie de condiciones neurológicas, músculo-esqueléticas o vasculares, requiere de un manejo multidisciplinario precoz, aunque por lo general, la respuesta a tratamiento clásico es escasa.

Introduction: Tuberculosis (TBC) and sarcoidosis have many similarities in their clinical manifestation, radiology and histology. The concept tuberculosis-sarcoidosis, refers to a clinical entity with different kinds of presentations, where TBC may precede, follow or coexist with the course of Sarcoidosis. Case report: 47 year old woman with a history of myomectomy and cholecystectomy, with a history of 4 months of difuse crampy abdominal pain, with hyporexia and 15 kilogram weight loss. Worsening of the pain made her go to the emergency unit, also presented frontal intense headache. In that moment she is admitted in the hospital to be studied for specialists. The symptoms evolved the next day with peripheral right facial paresis, left trigeminal commitment V1-V2, left abdominal hypoesthesia T10 to L1, swallowing symptoms of left lateral cutaneous nerve gloso. It was decided to continue study with lumbar puncture informing: protein: 0.81 mg / dl, glucose 62 mg / dl, 100% mono-nuclear Ziehl Nielsen and Gram negative. Chest CT identified mediastinal lymph nodes. In their biopsy, cheesy foci of necrosis, Ziehl Nielsen (+) was reported. She started corticoidaland antituberculosis treatment but she evolved in poor conditions; adding neuropathic costal pain and bilateral uveitis, so methotrexate (MTX) was added. Because of the none responding symptoms and dealing with a corticoid- and resistent disease it was decided to start with Infliximab, with great clinical response. Discussion: These entities can be confused with several neurological, muscle, or vascular conditions, it requires a multidisciplinary approach early, although generally, the classic response to treatment is low.

Subacute Papular Cutaneous Sarcoidosis Resistant to Conventional Therapy: A Case Report.

Sarcoidosis is a multisystem disease of obscure etiology that may involve almost any organ system; therefore, it results in various clinical manifestations. We are presenting a case of 60-year-old female complaining of multiple raised skin colored, to reddish lesions over face and few lesions over neck and extremities since 4 months cutaneous examination revealed multiple well defined skin colored to erythematous papulo-nodular lesions involving whole face with few discrete papular lesions over neck and extremities. Nasal skin was globular, studded with multiple nodules suggestive of lupus pernio. Histopathology showed many non-caseating naked epithelioid cell granulomas of variable sizes in the dermis. He was given oral methylprednisolone 16 mg twice daily after meal along with oral methotrexate 15 mg once in a week and tablet hydroxychloroquine 200 mg twice daily with keeping an eye over related adverse effects of all prescribed drugs. Still patient did not get remission after 6 months of a complete therapy. Due to the rarity of this disorder and its non-responding nature and course with conventional modalities of treatment made us to report this article for further studies and research.

Exacerbation of recalcitrant cutaneous sarcoidosis with adalimumab - a paradoxical effect? A case report / Agravamento de lesoes refratarias de sarcoidose cutanea com adalimumab- um efeito paradoxal? Relato de um caso

The paradoxical adverse effects of tumor necrosis factor-alpha (TNF-alpha) antagonists have been described frequently as a result of the widespread use of these drugs. Among the TNF-alpha blocking agents, few reports exist relating the use of adalimumab in cutaneous sarcoidosis, although all of them show good results. More recently, sarcoidosis onsets have been reported with various TNF-alpha inhibitors. The current case is, to our knowledge, the first to describe the exacerbation of cutaneous lesions of sarcoidosis treated with adalimumab.

Os efeitos paradoxais dos anti-TNF-alpha têm sido cada vez mais descritos com a utilização mais ampla dessas drogas. Entre os TNF-alpha, registam-se poucos casos com a utilização de adalimumab no tratamento da sarcoidose cutânea, sendo que todos eles apresentam bons resultados. Têm sido descritos, mais recentemente, casos de sarcoidose induzidos por vários anti-TNF-alpha. O presente caso é, até à data, o primeiro a descrever a exacerbação de lesões cutâneas de sarcoidose tratadas com adalimumab.

Angiotensin-converting enzyme inhibitors in the treatment of sarcoidosis and association with ACE gene polymorphism: Case series.

Angiotensin-converting enzyme (ACE) is used as a marker for sarcoid disease activity.1 We present an observational study of four African-American patients all of whom demonstrated improvement in their sarcoidosis after treatment with ACE inhibitors for hypertension.

Neurosarcoidosis: guidance for the general neurologist / Neurosarcoidose: orientações para o neurologista geral

Neurosarcoidosis (NS) more commonly occurs in the setting of systemic disease. The diagnosis is based on a clinical history suggestive of NS, presence of noncaseating granulomas, and supportive evidence of sarcoid pathology, laboratory, and imaging studies. NS could involve any part of the nervous system and often demands high doses of steroids for symptom control. It presents low response to isolated steroids administration and frequently requires immunosuppressive agents. In NS, lymphocytes are polarized toward an excessive Th1 response, leading to overproduction of TNF-alpha and INF-gama, as well as lL-2 and IL-15. Infliximab, a chimeric monoclonal antibody that neutralizes the biological activity of TNF-alpha, is a new option in the NS treatment. We revised pathophysiology, clinical manifestations, diagnostic work up, and treatment of NS as guidance for the general neurologist.

A neurosarcoidose (NS) ocorre frequentemente no contexto de doença sistêmica. O diagnóstico é baseado na história clínica sugestiva de NS, presença de granulomas não-caseosos e achados anatomopatológicos, laboratoriais e radiológicos de sarcoidose. A NS causa manifestações neurológicas variadas, que apresentam, em geral, baixa resposta ao corticoide isoladamente e, portanto, necessitam uso de imunossupressores. Na NS, os linfócitos estão polarizados para resposta Th1 excessiva, levando à produção aumentada de TNF-alfa e IFN-gama, assim como IL-2 e IL-15. Infliximabe, um anticorpo monoclonal quimérico que neutraliza a atividade biológica do TNF-alfa, é uma nova opção no tratamento da NS. Revisou-se a fisiopatologia, as manifestações clínicas, o diagnóstico e o tratamento da NS para orientar neurologistas gerais.

Gastrointestinal sarcoidosis: a case report

Gastrointestinal sarcoidosis commonly occurs subclinically, with clinical manifestations present in only 0.1 to 0.9% of patients with the disease. Gastric sarcoidosis particularly involves the antrum. Small bowel, liver and spleen involvement is seen in approximately 10% of patients with systemic sarcoidosis. We report a rare case of gastrointestinal sarcoidosis of the stomach and terminal ileum, affecting the liver and spleen without pulmonary involvement, who responded favorably to corticosteroid treatment

Isolated Spinal Cord Neurosarcoidosis Diagnosed by Cord Biopsy and Thalidomide Trial

We report a case of 54-yr-old woman who presented with 4-extremities weakness and sensory changes, followed by cervical spinal cord lesion in magnetic resonance imaging. Based on the suspicion of spinal tumor, spinal cord biopsy was performed, and the histology revealed multinucleated giant cells, lymphocytes and aggregated histiocytes within granulomatous inflammation, consistent with non-caseating granuloma seen in sarcoidosis. The patient was treated with corticosteroid, immunosuppressant and thalidomide for years. Our case indicates that diagnosis of spinal cord sarcoidosis is challenging and may require histological examination, and high-dose corticosteroid and immunosuppressant will be a good choice in the treatment of spinal cord sarcoidosis, and the thalidomide has to be debated in the spinal cord sarcoidosis.

Sarcoidosis cutánea / Cutaneous sarcoidosis

La sarcoidosis es una enfermedad granulomatosa multisistémica de etiología desconocida. Entre sus manifestaciones clínicas destaca la afectación cutánea, existiendo lesiones específicas e inespecíficas, pudiendo ser la primera manifestación de la enfermedad. Presentamos tres pacientes con sarcoidosis; dos de los casos expresaron la enfermedad como granuloma anular generalizado y el otro caso se manifestó con nódulos subcutáneos.

Sarcoidosis is a multisystemic granulomatous disease of unknown etiology. Cutaneous lesions, both specific and non specific, stand out among other clinical manifestations and can be the first manifestation of the disease. We present three patients with sarcoidosis, two cases present as generalized granuloma annulare, and the third as subcutaneous nodules.

49-year-old woman with refractory chronic sinusitis. Sarcoidosis with granulomatous sinusitis Photo Quiz

A 49-year-old married, non- smoker housewife had purulent rhinorrhea, nasal congestion, post nasal drip [PND], and feeling of sinus pressure following an episode of common cold. She had no complaints of fever, cough, dyspnea, or arthralgia. Physical examination revealed PND and a posterior auricular lymphadenopathy [1cm X 1cm]. The patient received amoxicillin for 2 weeks with mild improvement. But again, she experienced the exacerbation of signs and symptoms a week later. CT-scan of the paranasal sinuses was performed [Figure 1]. She also had erythematous patches on her face around the right eye and her back, along with splenomegaly. The patient was treated with amoxicillin/clavulanate [for two weeks], loratadine, and beclomethasone nasal spray as well as normal saline nasal wash. There was a slight improvement in patient's signs but, repeatedly after two weeks signs and symptoms exacerbated. Due to the lack of response to antibiotics, a biopsy of the skin lesion was taken which revealed the following histopathological findings

Tratamiento con hidroxicloroquina de la sarcoidosis cutánea. Revisión de la terapéutica actual. A propósito de una caso / Hydroxychloroquine treatment of cutaneous sarcoidosis. Review of current therapy. With regard to a case

Se describe una paciente con sarcoidosis cutánea crónica localizada en región frontotemporal derecha que se extiende al pabellón auricular, de diez años de evolución. La sarcoidosis cutánea es llamada la “gran imitadora” por sus similitudes clínicas con otras patologías cutáneas. La paciente refiere haber realizado múltiples tratamientos con corticoides tópicos, antibióticos tópicos, metronidazol tópico e isotretinoina vía oral, sin respuesta a los mismos. Se establece tratamiento local con tacrolimus 0.1% (dos veces al día) con escasa respuesta, observándose buena respuesta clínica luego de la administración sistémica de hidroxicloroquina 200 mg/d vía oral. El objetivo de esta publicación radica en describir una paciente con una forma cutánea pura de sarcoidosis facial al momento del diagnóstico, con buena respuesta a la hidroxicloroquina oral y efectuar una revisión bibliográfica de la terapéutica actual.