Adult ocular adnexal xanthogranulomatous disease associated with immunoglobulin G4-related disease: an unusual association.

Adult-onset xanthogranuloma (AOX) and immunoglobulin G4-related disease (IgG4-RD) are uncommon fibrosing conditions that may exhibit localized ocular manifestations and occasionally systemic symptoms. These conditions exhibit overlapping clinical and histological features, suggesting a potential correlation between them, although their exact relationship remains unclear. This paper presents the case of a black male patient exhibiting typical histological indications of both AOX and IgG4-RD. The patient responded positively to corticosteroid treatment.

Secondary vs. primary pituitary xanthogranulomas: which yellow is more mellow?

Pituitary xanthogranulomatomas (XG) are a rare pathological entity caused by accumulation of lipid laden macrophages and reactive granuloma formation usually triggered by cystic fluid leakage or hemorrhage. Our aim was to compare clinical characteristics and presenting features of patients with secondary etiology of XG and those with no identifiable founding lesion (primary -"pure" XG) in order to gain new insights into this rare pituitary pathology. In a retrospective review of 714 patients operated for sellar masses, at tertiary center, we identified 16 (2.24%) with histologically confirmed diagnosis of pituitary XG over the period of 7 years (2015-2021). Patients were further analyzed according to XG etiology: "pure"- XG (n = 8) with no identifiable founding lesion were compared to those with histological elements of pituitary tumor or cyst - secondary XG (n = 8). We identified 16 patients (11 male), mean age 44.8 ± 22.3 years, diagnosed with pituitary XG. Secondary forms were associated with Ratke's cleft cyst (RCC, n = 2) and pituitary adenoma (PA, n = 6). The most common presenting features in both groups were hypopituitarism (75%), headache (68.5%) and visual disturbances (37.5%). Predominance of male sex was noted (males 68.75%, females 31.25%), especially in patients with primary forms. Patients with primary pituitary XG were all males (p = 0.0256) and more frequently affected by panhypopituitarism (87.5% vs. 25%, p = 0.0406) compared to patients with secondary causes. Hyperprolactinemia was noted in pituitary tumor group with secondary etiology only (p = 0.0769). Majority of lesions were solid on magnetic resonance imaging - MRI (81.25%). Distinct clinical phenotype was observed dependent on the etiology of XG.

Efficacy of denosumab in the treatment of hypercalcemic renal dysfunction in sarcoidosis: a case report.

A 70-year-old female patient was admitted for close examination and treatment of hypercalcemia (corrected serum calcium levels: 3.04 mmol/L) and renal dysfunction (serum creatinine levels: 254.59 µmol/L). The patient had a history of sarcoidosis, diagnosed based on epithelioid cell granulomas in subcutaneous nodule biopsies, uveitis, and bilateral hilar lymphadenopathy, which had spontaneously remitted 10 years before admission. Because the patient was diagnosed with hypercalcemia associated with recurrent sarcoidosis, prednisone (20 mg/day) was initiated, and its dose was tapered following the decrease in serum calcium and creatinine levels. However, the levels of these parameters increased again when the prednisone dose was reduced to ≤ 4 mg/day. We were concerned about glucocorticoid-induced osteoporosis in the patient but hesitated to use first-line bisphosphonates because of renal dysfunction. Therefore, denosumab was initiated to reduce the risk of hypercalcemia, renal dysfunction, and glucocorticoid-induced osteoporosis. Serum creatinine and corrected serum calcium levels subsequently decreased. The prednisone dose could be reduced following repeated denosumab administration.Thus, denosumab can be a multifaceted, beneficial option for sarcoidosis-induced hypercalcemia, as it alleviates renal dysfunction indirectly by normalizing serum calcium levels, facilitates reduction of the glucocorticoid dose, and ameliorates glucocorticoid-induced osteoporosis.

A Rare Case of Thyroid Sarcoidosis.

ABSTRACT: Thyroid sarcoidosis is a rare manifestation of sarcoidosis, an inflammatory disease characterized by the formation of noncaseating granulomas in various organs. The diagnosis of thyroid sarcoidosis is challenging because of its nonspecific symptoms and the absence of specific biomarkers. Here, we report the case of a 43-year-old woman who presented with a 2-year history of neck swelling, dysphonia, and dysphagia, and suspected nodule in her left thyroid.

Intralesional corticosteroid treatment of periocular xanthogranuloma associated with adult-onset asthma.

INTRODUCTION: Adult-onset asthma and periocular xanthogranuloma (AAPOX) is a rare non-Langerhans cell histiocytic disorder. AIM: To describe the periocular clinical findings in a patient diagnosed with AAPOX, treated successfully by intralesional corticosteroids. CASE REPORT: A 40-year-old woman presented with bilateral eyelid swelling and adult-onset asthma. Initial examination revealed bilateral yellow-orange, elevated, indurated, and nonulcerated masses at the upper eyelids. The laboratory data showed high level of IgG. Periocular biopsy samples showed xanthoma cells positive for CD68 and Touton giant cells. The patient received 2 intralesional 40 mg of triamcinolone acetonide with a local control. No complications were noted. COMMENTARIES: AAPOX is characterized by a histiocytic proliferation associated to an adult-onset asthma, systemic lymphadenopathy, salivary gland enlargement and elevated serum levels of IgG. Treatment options vary with no current consensus. Intralesional corticosteroids have been rarely reported in controlling the signs and symptoms of adult-onset xanthogranulomatous disease. Surgery was successful but demonstrated recurrence. Methotrexate has been proposed to treat refractory cases or as a potential corticosteroid-sparing therapy. In this case, intralesional corticosteroid was an effective and safe treatment for eyelid adult xanthogranuloma.

Caracterización de enfermos con cáncer de tiroides operados en el Hospital Clínico Quirúrgico General "Freyre de Andrade" / Characterization of Patients with Thyroid Cancer Operated on in "Freyre de Andrade" General Clinical Surgical Hospital

Introducción: El cáncer de tiroides es el tumor más común de los malignos originados en órganos endocrinos (más del 92 por ciento) y comprende un grupo de tumores que son diferentes clínicamente, epidemiológicamente y en cuanto a pronóstico. Objetivo: Caracterizar a los enfermos con cáncer de tiroides operados en el Hospital Clínico Quirúrgico General "Freyre de Andrade". Métodos: Se realizó un estudio descriptivo, retrospectivo de corte longitudinal en el Hospital Clínico Quirúrgico General "Freyre de Andrade" en el período 2014-2019. El universo estuvo constituido por 32 enfermos operados de cáncer de tiroides. Fueron analizadas variables sociodemográficas, clasificación de Bethesda, diagnóstico histológico, técnica quirúrgica empleada y complicaciones. Resultados: El cáncer de tiroides predominó en mujeres (87,5 por ciento) entre 51 - 60 años (25,0 por ciento). Según la clasificación de Bethesda prevaleció la categoría VI (15,62 por ciento). Predominó el carcinoma papilar (65,62 por ciento). A todos los enfermos se le realizó una tiroidectomía total y las complicaciones fueron el hematoma posoperatorio (6,25 por ciento) y el granuloma (6,25 por ciento). Conclusiones: El cáncer tiroideo predomina en mujeres entre la quinta y sexta década de la vida. La técnica quirúrgica que se utiliza es la tiroidectomía total con baja morbilidad. Predomina la variante histológica del carcinoma papilar(AU)

Introduction: Thyroid cancer is the most common malignant tumor originating in endocrine organs (more than 92 percent) and comprises a group of clinically, epidemiologically and prognostically different tumors. Objective: To characterize patients with thyroid cancer operated on at Freyre de Andrade General Clinical Surgical Hospital. Methods: A descriptive, retrospective and longitudinal study was carried out at Freyre de Andrade General Clinical Surgical Hospital in the period 2014-2019. The universe consisted of 32 patients operated on for thyroid cancer. Sociodemographic variables, Bethesda classification, histological diagnosis, used surgical technique and complications were analyzed. Results: Thyroid cancer predominated in women (87.5 percent) between 51 and 60 years of age (25.0 percent). According to the Bethesda classification, category VI prevailed (15.62 percent). Papillary carcinoma predominated (65.62 percent). All patients underwent total thyroidectomy, while the complications were postoperative hematoma (6.25 percent) and granuloma (6.25 percent. Conclusions: Thyroid cancer predominates in women between the fifth and sixth decades of life. The used surgical technique is total thyroidectomy, reporting low morbidity. The histological variant of papillary carcinoma predominates(AU)

Sarcoid-like granulomatous myositis-associated hypercalcemia. An infrequent case to consider / Hipercalcemia asociada a miositis granulomatosa sarcoidea simil- sarcoidea. Un caso infrecuente a considerar

Abstract PTH-independent hypercalcemia due to granulomatous disease is well-documented and sarcoidosis is the most characteristic disease, although there are others. We describe a case of sarcoid-like granulomatous myositis. An 87-year-old man was referred with tetraparesis and hypercalcemia (albumin-corrected calcium of 13.4 mg/dl) following a trip to the Caribbean. The evaluation showed a suppressed PTH, 25-hydroxy vitamin D of 7.5 ng/ml, 18F-FDG PET/CT showed markedly increased uptake in intercostal, back, shoulder, but tock and thigh muscles and a deltoid biopsy confirmed extensive granulomatous myositis. He was prescribed glucocorticoids which resulted in normalized plasma calcium levels and complete recovery from tetraparesis. Sarcoid-like granulomatous myositis should be incorporated into the differential diagnosis of PTH-independent hypercalcemia, especially in the absence of clinical features of sarcoidosis and with special emphasis on the use of 18F-FDG PET/CT to ensure a correct approach.

Resumen La hipercalcemia PTH-independiente asociada a enfermedades granulomatosas está bien documentada y la sarcoidosis es la enfermedad más característica, a pesar de que existen otras. Des cribimos un caso de miositis granulomatosa simil-sarcoidea. Un hombre de 87 años consultó por tetraparesia e hipercalcemia (calcio corregido por albúmina 13.4 mg/dl) luego de un viaje al Caribe. La evaluación mostró una PTH suprimida, 25-hidroxivitamina D 7.5 ng/ml, 18F-FDG PET/CT mostró marcado aumento de captación a nivel de musculatura intercostal, dorsal, deltoidea, glúteos y muslos. Una biopsia deltoidea confirmó una miositis granulomatosa extensa. Se prescribieron glucocorticoides, resultando en normalización del calcio plasmático y completa recuperación de la tetraparesia. La miositis granulomatosa simil-sarcoidea debe ser incorporada den tro del diagnóstico diferencial de la hipercalcemia PTH-independiente, especialmente en ausencia de hallazgos clínicos de sarcoidosis y con especial énfasis en el uso de 18F-FDG PET/CT para su correcta aproximación.

Comunicación de un caso de granulomatosis con poliangeítis con detección de granulomas en médula ósea / A case report of granulomatosis with polyangiitis with granulomas in bone marrow

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La Primera Guerra Mundial: el amanecer de las transfusiones sanguíneas / First World War: the dawn of blood transfusions

En 1914 el infierno se afloró en Europa y se extendió a una buena parte del mundo. La capacidad destructiva del hombre para con el hombre llegó a niveles insospechados. Centenares de miles de bajas precisaban atención sanitaria urgente en cada campaña. Esta enorme demanda hizo que en apenas cinco años la Medicina avanzara lo que en otra situación hubiera necesitado muchas décadas. El tratamiento quirúrgico de las heridas, el tratamiento protésico, la cirugía plástica, la radiología y la anestesia fueron los campos donde se avanzó más. Sin embargo, probablemente uno de los mayores hitos alcanzados en este periodo, y seguramente uno de los de más trascendencia, fue el desarrollo de la terapia transfusional. En pocos años pasó de ser una técnica de «científicos extraños» a ser una herramienta terapéutica indispensable. Aquí se describe el camino hacia la consecución y sus protagonistas

In 1914, hell broke out in Europe and spread to much of the world. The destructive capacity of man against man reached unsuspected levels. Hundreds of thousands of casualties needed urgent health care in each campaign. This enormous demand meant that in just five years, medicine would advance what in another situation would have needed many decades. Surgical treatment of wounds, orthopedic treatment, plastic surgery, radiology and anesthesia were the fields where more progress was made. However, probably one of the major milestones reached in this period of time, and certainly one of the most transcendent, was the development of blood transfusion. In a few years it went from being a technique of "strange scientists" to become essential. Here we describe the path to achievement and its main characters

Dermatite perioral granulomatosa com acometimento extrafacial na infância: boa resposta terapêutica com uso de azitromicina oral / Granulomatous perioral dermatitis with extra-facial involvement in childhood: good therapeutic response with oral azithromycin

RESUMO Objetivo: Apresentar um caso de dermatite perioral granulomatosa (DPG) com acometimento extrafacial e resposta terapêutica satisfatória ao uso de macrolídeo oral por curto período. Descrição do caso: Escolar de nove anos, sexo feminino, com quadro exuberante de DPG com acometimento extrafacial. Durante o período de evolução, submeteu-se a múltiplas terapêuticas ineficazes, apresentando melhora significativa das lesões após o uso de azitromicina por cinco dias. Comentários: A DPG é uma afecção dermatológica inflamatória representada por erupções papuloeritematosas em região perioral, nasal e periorbitária, mais comum em crianças e adolescentes. Raramente estende-se à região genital, ao tronco e às extremidades, caracterizando o comprometimento extrafacial. De etiologia ainda desconhecida, parece apresentar correlação com uso de corticosteroides tópicos e outros agentes.

ABSTRACT Objective: To present a case of granulomatous perioral dermatitis (GPD) with extra-facial involvement and good response to short-term treatment with oral macrolide. Case description: A 9-year-old girl presented with exuberant GPD with extra-facial involvement. During follow-up, she received multiple ineffective therapies, but showed significant improvement of the lesions after the use of azithromycin for five days. Comments: GPD is an inflammatory dermatological condition represented by papulo-erythematous eruptions on perioral, nasal and periorbital regions, more prevalent in children and adolescents. It rarely extends to the genital region, trunk, and extremities, which characterizes its extra-facial manifestation. Its etiology is unknown, but it seems to have a correlation with the use of topical corticosteroids and other agents.

Methotrexate Pneumonitis in Crohn's Disease: A Rare Case Report and Review of Literature / Neumonitis por metotrexato en la enfermedad de Crohn: un caso clínico inusual y revisión de la literatura

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Granuloma aséptico facial idiopático: hallazgos clínicos y ecográficos de 3 casos / Idiopathic Facial Aseptic Granuloma: Clinical and Ultrasound Findings in 3 Cases

El granuloma aséptico facial idiopático es una entidad propia de la infancia que se caracteriza por la presencia de uno o varios nódulos asintomáticos en la mejilla. Aunque su patogenia no está clara, se ha sugerido que pueda pertenecer al espectro de la rosácea infantil. Resuelve espontáneamente pero puede plantear dudas diagnósticas con otras lesiones que sí requieren tratamiento. Presentamos los hallazgos clínicos y ecográficos y la evolución, tanto clínica y ecográfica, de 3 nuevos casos. En 2 pacientes, la lesión se presentó en el contexto de una rosácea infantil. En ecografía, el granuloma aséptico facial idiopático mostró un patrón característico, con variaciones en función del momento evolutivo. La ecografía de alta frecuencia puede facilitar el diagnóstico diferencial, evitando biopsias o extirpaciones innecesarias

Idiopathic facial aseptic granuloma is a typical childhood disease characterized by the presence of one or more asymptomatic nodules on the cheek. Although pathogenesis remains unclear, the disease is thought to be a type of childhood rosacea. It resolves spontaneously, yet it could be confused with other lesions that require treatment. We present clinical and ultrasound findings and outcome from 3 new cases. In 2 cases, the lesion presented as childhood rosacea. Ultrasound revealed a characteristic pattern, with variations depending on the stage of development. High-frequency ultrasound can facilitate the differential diagnosis and thus obviate unnecessary biopsy or excision

Hyponatremia as presentation in a patient with neurosarcoidosis / Hiponatremia como presentación en un paciente con neurosarcoidosis

Sarcoidosis is an inflammatory disease with unknown cause characterized by non-caseating granuloma formations. It may present with bilateral hilar lymphadenopathy, skin lesions, eye and musculoskeletal system involvement. Hypercalcemia and hypercalciuria are important electrolyte imbalances resulting from sarcoidosis and sometimes they may cause nephrolitiasis and kidney failure. Hyponatremia, although being quite rare, has been found in some patients with sarcoidosis. Herein, we have reported a patient with neurosarcoidosis who presented with hyponatremia and responded well to corticosteroid treatment (AU)

La sarcoidosis es una enfermedad inflamatoria, de causa desconocida, que se caracteriza por la formación de granulomas no caseificantes. Se puede presentar con linfadenopatía hiliar bilateral, lesiones en la piel, en los ojos, y la implicación del sistema musculoesquelético. La hipercalcemia y la hipercalciuria son importantes desequilibrios electrolíticos resultantes de la sarcoidosis, y algunas veces pueden causar nefrolitiasis e insuficiencia renal. La hiponatremia, a pesar de ser bastante rara, se ha encontrado en algunos pacientes con sarcoidosis. Aquí, hemos informado de un paciente con neurosarcoidosis que se presentó con hiponatremia y respondió bien al tratamiento con glucocorticoides (AU)

Masa intracardiaca de origen inusual / Unusual origin of an intracardiac mass

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Sarcoidosis: an unusual presentation / Sarcoidosis: un cuadro clínico inicial poco frecuente

A 35-year-old man presented with a 3-year history of arthralgia and purple coloration of the skin of his fingers and feet. Hand and foot radiography showed cystic bone lesions on phalanges suggestive of sarcoidosis. Lab tests revealed increased liver enzymes. Liver MRI evidenced an enlarged liver and retroperitoneal lymphadenopathy. Histological analysis of the finger skin, lymph nodes and liver demonstrated the presence of granulomas, confirming the diagnosis of sarcoidosis. The patient started prednisolone with rapid improvement of the symptoms. Skin lesions are divided into two groups: specific for sarcoidosis (with granulomas, lupus pernio-like) and nonspecific (without granulomas, erythema nodosum-like). Specific cutaneous lesions usually cause no other symptoms beyond cosmetic changes. Lupus pernio stands out for having distinctive features but, to the best of our knowledge, the simultaneous involvement of both hands and feet has never been reported (AU)

Se presenta el caso de un hombre de 35 años con una historia de artralgia y con la piel de los dedos y los pies de color violáceo, de 3 años de duración. La radiografía de pies y manos mostró lesiones quísticas óseas en las falanges, indicativas de sarcoidosis. Las pruebas de laboratorio revelaron una elevación de las enzimas hepáticas. La resonancia magnética hepática puso de manifiesto hepatomegalia y linfadenopatía retroperitoneal. El análisis histológico de la piel de los dedos, los ganglios linfáticos y el hígado mostró la existencia de granulomas, lo que confirmó el diagnóstico de sarcoidosis. El paciente comenzó el tratamiento con prednisolona con una rápida mejoría de los síntomas. Las lesiones de la piel se dividen en 2 grupos: específicas de la sarcoidosis (con granulomas y lupus característico del eritema pernio) e inespecíficas (sin granulomas y de tipo eritema nudoso). Las lesiones cutáneas específicas generalmente no causan más síntomas que los cambios estéticos. El lupus pernio destaca por presentar características distintivas, pero no nos consta que se haya descrito nunca la afectación simultánea de ambas manos y pies (AU)

Quemadura del tubo orotraqueal flexometálico en cirugía con láser / Flexometalic endotraqueal tube burn in laser surgery

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Linfadenitis por Mycobacterium bovis, una entidad a tener en cuenta / Mycobacterium bovis lymphadenitis, an entity to consider

Las medidas de control del ganado y la pasteurización de los productos lácteos han hecho que la enfermedad tuberculosa por Mycobacterium bovis sea infrecuente en nuestro medio. El diagnóstico de esta entidad requiere un alto índice de sospecha, fundado en una historia clínica detallada y pruebas complementarias oportunas. La presentación más frecuente es extrapulmonar, principalmente como linfadenitis, por lo que ante una adenopatía de mala evolución se recomienda remitir al paciente para estudio y valorar la realización de punción aspiración con aguja fina para examen citológico y cultivo. Presentamos un caso de linfadenitis por M. bovis en una niña de siete años que respondió adecuadamente al tratamiento médico (AU9

Cattle control measures and milk products pasteurization have made Mycobacterium bovis disease uncommon in our environment. The diagnosis of this disease requires a high index of suspicion based on a detailed medical history and appropriate laboratory tests. Extrapulmonary cases are the main presentation, especially lymphadenitis, for this reason, is recommended to refer to study all the adenopathies with poor evolution, for evaluating de need of a fine-needle aspiration for citologý examination and culture. We report the case of M. bovis lymphadenitis in a 7-year-old girl with adequate response to medical treatment (AU)

Granuloma aséptico facial idiopático. Utilidad de la ecografía cutánea / Idiopathic facial aseptic granuloma: usefulness of cutaneous ultrasound

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