Xanthogranulomatous pleuritis - An unusual presentation of tuberculosis.

In pulmonary practice, pleural effusion is a commonly encountered entity and has various etiologies. Pleural effusions in postpartum women can be an incidental self-limiting finding. The presence of systemic or respiratory symptoms, however, calls for prompt etiological workup and targeted therapy. Tuberculous pleuritis and lupus-related pleural disease are well known to flare up in the postpartum period. We describe the case of a young healthcare worker with no previous comorbidities who presented with fever, breathlessness, and chest pain 2 weeks after an uneventful confinement. Chest radiograph revealed moderate left-sided pleural effusion. Pleural fluid analysis was biochemically consistent with tubercular effusion. Pleural biopsy histological examination showed features of xanthomatous pleuritis and Cartridge based nucleic acid amplification test (CB-NAAT) showed evidence of Mycobacterium tuberculosis(MTB). She was initiated on antitubercular medicines to which she responded well with the resolution of clinical symptoms and pleural collection. This is the first case report describing an association of xanthogranulomatous pleuritis with tuberculosis.

Enfermedad de Erdheim-Chester: Serie de diecinueve casos y un diagnóstico diferencial de relevancia / Erdheim-Chester Disease: Series of nineteen cases and a relevant differential diagnosis

La enfermedad de Erdheim-Chester (EEC) es una histiocitosis de células no Langerhans de presentación proteiforme y escaso conocimiento. Se presenta una serie de 19 casos de 4 centros, registrados de junio de 2012 a junio de 2019. Se incluyeron aquellos pacientes con clínica, anatomía patológica e inmunohistoquímica característica de la enfermedad. Se excluyeron pacientes con hallazgos indefinidos. Resultados: al igual que en la literatura, el compromiso más frecuente fue el óseo por imagenología, la mitad de estos sintomático. Nuestra serie presenta mayor incidencia de mujeres (casi 70%). Siete pacientes presentaron nódulo mamario como forma de presentación. La mayoría recibieron dosis media de esteroides asociado a otra droga inmunosupresora. La mortalidad fue del 16%. Conclusión: comunicamos una serie de pacientes con la EEC mencionando las características más destacables. Es llamativo el número de pacientes con afectación mamaria, por lo cual proponemos téngase en cuenta en el diagnóstico diferencial de la patología tumoral mamaria.

Erdheim-Chester disease (ECD) is a non-Langerhans histiocytosis, protein manifestations at start and little known. We included 19 patients from June 2012 to June of 2019. Inclusion criteria: clinical features, histopathology and immunostaining compatible with ECD. We excluded patients with undefined features. Results: Bones were the most frequent affected, half of them were asymptomatic. Seventy per cent of the patients were women, and 7 of them developed a nodule breast as first manifestation of ECD. The patients were treated with corticosteroids associated or not with immunosuppressants. The mortality rate was 16%. Conclusion: We reported a series of patients with ECD, enhancing the most frequent features. It is striking the number of patients with breast involvement; we propose to include the Erdheim-Chester disease in differential diagnosis of breast tumor.

Juvenile Xanthogranuloma: Case Report and Literature Review.

Juvenile Xanthogranuloma (JXG) is a relatively uncommon non-Langerhans cell histiocytosis, which often occurs at an early age and is usually asymptomatic. Herein, we present the case of a 17-year-old man with numerous asymptomatic yellow-brown papulonodular lesions with a symmetric distribution on upper and lower extremities, face, and trunk, developed over the past 4 years. In the histopathologic examination, histiocytes with a Touton-like appearance were observed in favor of xanthogranuloma. The patient was treated with isotretinoin 20 mg daily for 2 months, which surprisingly led to the progression of lesions and thus was discontinued. Although JXG may cause severe morbidities in some circumstances, it is a self-limiting benign disorder and patients should be assured regarding the benign self-regressive nature of the disease.

Levodopa-responsive parkinsonism in a patient with corticobasal degeneration and bilateral choroid plexus xanthogranulomas.

Corticobasal degeneration (CBD) has substantial overlap of clinical features with other neurodegenerative diseases including Parkinson's disease (PD). Its clinical diagnostic accuracy is the lowest among the common neurodegenerative diseases, and its antemortem diagnosis is more challenging when CBD is comorbid with another brain disease. We report an elderly male patient with multiple medical conditions and a family history of essential tremor. He presented with progressive tremor that was initially thought to be essential tremor and later diagnosed as PD despite head computerized tomography showing bilateral intraventricular masses and other minor changes. The clinical diagnosis of PD was supported by his responsiveness to low-dose levodopa. However, postmortem neuropathological examination revealed CBD and bilateral choroid plexus xanthogranulomas with mild ventricular enlargement and multifocal ependymal lining injury presumably due to mild hydrocephalus. CBD is typically levodopa-unresponsive, but hydrocephalus-associated parkinsonism is commonly levodopa-responsive. We raise awareness of the present comorbidity and atypical parkinsonism due to the choroid plexus xanthogranuloma-induced hydrocephalus for the clinical diagnosis and management of parkinsonism.

[Difficult diagnosis of Erdheim Chester disease revealed by central diabetes insipidus]. / Le cas clinique du mois : Diagnostic difficile d'une maladie d'Erdheim Chester révélée par un diabète insipide central.

The Erdheim Chester disease is a rare form of non Langerhans cell histiocytosis. Its rarity and its unspecific clinical presentation, make that its diagnosis is often delayed. We report the case of a 50 years old female who has an Erdheim Chester disease, revealed by a central diabetes insipidus with thickening of the pituitary stalk, with associated gonadotropin deficiency. The Erdheim Chester disease was suspected because of the association with other evocative systemic lesions: eyelid xanthelasmas and bone lesions in metaphyseal-diaphyseal region of the upper and lower ends of both femurs and tibias on bone scan. Confirmation of the diagnosis was initially difficult and delayed, with initially inconclusive cutaneous and bone biopsies. It is the histological re-reading with immunohistochemical study of the bone biopsies which allowed the diagnosis by showing histiocytes positive for the CD68 and negative for the CD1a and the protein S100. The diagnosis was made with a delay of 3 years. In conclusion, although rare, Erdheim Chester disease should be suspected in front of a set of clinical and radiological arguments. Diagnostic confirmation is based on histological and especially immunohistochemical studies.

La maladie d'Erdheim Chester est une forme rare d'histiocytose non langerhansienne. Par sa rareté et son tableau clinique peu spécifique, son diagnostic est souvent retardé. Nous rapportons le cas d'une patiente âgée de 50 ans ayant une maladie d'Erdheim Chester révélée par un diabète insipide central avec épaississement de la tige pituitaire à l'IRM cérébrale et insuffisance gonadotrope associée. Cette maladie a été suspectée devant l'association d'autres atteintes systémiques évocatrices : des lésions cutanées à type de xanthélasmas sus-palpébraux de couleur jaune-orange et une atteinte osseuse au niveau métaphyso-diaphysaire des extrémités supérieures et inférieures des deux fémurs et tibias objectivée à la scintigraphie osseuse. La confirmation du diagnostic était initialement difficile et retardée, avec des biopsies cutanée et osseuse initialement non concluantes. C'est la relecture histologique avec étude immuno-histochimique des biopsies osseuses qui ont permis le diagnostic en montrant des histiocytes positifs pour le CD68 et négatifs pour le CD1a et la protéine S100. Le diagnostic positif a été posé avec un retard de 3 ans par rapport à la première consultation. En conclusion, bien que rare, la maladie d'Erdheim Chester devrait être suspectée devant un faisceau d'arguments cliniques et radiologiques. La confirmation diagnostique repose sur l'étude histologique et, surtout, immuno-histochimique.

Symptomatic Bilateral Xanthogranuloma of the Choroid Plexus.

Xanthogranulomas (XGRs) of the choroid plexus are rare, asymptomatic, and benign lesions usually found incidentally. Here, we present a case of a 47-year-old male with bilateral XGR of the choroid plexus with periventricular edema and discuss our case in relation to a review of existing literature pertaining to the radiology of XGRs. To the best of our knowledge, this is the first reported case of bilateral trigonal XGR causing brain edema without ventricular dilatation. Despite the fact that they can cause hydrocephalus, XGRs are silent and benign lesions. Although the etiopathology of XGRs remains poorly understood, enhanced imaging analyses may provide additional information regarding edema and focal white matter signal changes.

Clinicoradiologic Features and Surgical Outcomes of Sellar Xanthogranulomas: A Single-Center 10-Year Experience.

BACKGROUND: Xanthogranulomas are uncommon granulomatous entities, and xanthogranulomas occurring in the sellar region are extremely rare, with a limited number of cases reported in the literature. Diagnosis, management, and prognosis of sellar xanthogranulomas are poorly understood. METHODS: We retrospectively reviewed 14 patients with histologically proven sellar xanthogranulomas treated at our institution between January 2006 and July 2016. Clinical manifestations, radiologic features, operative records, and follow-up outcomes were analyzed. RESULTS: There were 9 male and 5 female patients with a mean age of 29.2 years. Clinical manifestations included headache (n = 12; 85.7%), visual deficiencies (n = 10; 71.4%), and endocrine disturbances (n = 6; 42.9%). On magnetic resonance imaging, sellar xanthogranulomas revealed isointensity to hyperintensity on T1-weighted imaging and hyperintensity or heterogeneous intensity on T2-weighted imaging with variable enhancement. Gross total resection of the lesion was achieved in 10 cases, and subtotal resection was achieved in 4 cases. Postoperatively, headache was relieved in all cases; transient diabetes insipidus was observed in 2 cases. There were no procedure-related complications. During a mean follow-up period of 52.9 months, visual deficiencies were improved in 7 (70.0%) cases, whereas endocrine disturbances were improved in only 2 (33.3%) cases. No recurrence or regrowth of the residual lesions was observed. CONCLUSIONS: Sellar xanthogranuloma should be considered in the differential diagnosis of lesions in the sellar region. Some radiologic characteristics, including hyperintensity on T1-weighted imaging, heterogeneous hyperintensity on T2-weighted imaging, variable enhancement, and absence of calcification, can assist in making the preoperative diagnosis. Surgical outcomes are favorable.

Adult-Onset Asthma and Periocular Xanthogranulomas Associated with Systemic IgG4-Related Disease.

PURPOSE: The aim of this study was to report a case of Adult-Onset Asthma with Periocular Xanthogranulomas (AAPOX) associated with systemic IgG4-related disease (IgG4-RD). OBSERVATIONS: A 57-year-old man presented with bilateral periorbital swelling for 1 year. Histopathology of a left orbital biopsy showed fibro vascular connective tissue inundated with foamy, lipid-laden histiocytes and touton giant cells with lymphocytic inflammation. Additional stains revealed CD68 positivity, and S100 negativity. The IgG and IgG4 stained slides showed increased IgG4 positive plasma cells but did not meet the criteria for IgG4-related orbital disease. His IgG4 serology was elevated, and IgG4 staining of his tissue previously diagnosed as autoimmune sclerosing pancreatitis was found to meet the criteria for IgG4-RD. CONCLUSIONS AND IMPORTANCE: AAPOX can be associated with systemic IgG4-RD.

Xantogranuloma juvenil múltiple: variante inusual en un lactante / Multiple juvenile xanthogranuloma: unusual variant in an infant

El Xantogranuloma Juvenil (XJ) es un tumor benigno de la infancia, constituido por histiocitos progresivamente lipidizados, en ausencia de anomalías metabólicas. Se reporta un caso de XJ múltiple en un lactante. Paciente masculino de 1 año y 11 meses de edad, sin antecedentes mórbidos. Consultó por lesiones de 15 meses de evolución en cuero cabelludo. Al examen físico destacaban dos pápulas amarillentas y tres máculas anaranjadas en cuero cabelludo. A la dermatoscopía se apreciaba un color amarillonaranja homogéneo. La biopsia incisional demostró infiltrado de histiocitos en dermis y células de Touton. A la inmunohistoquímica, los histiocitos fueron CD68 positivo, S100 negativo y CD1a negativo. Los exámenes de laboratorio y la evaluación por oftalmología no evidenciaron anormalidades. El XJ es un tumor benigno, siendo la forma más común de Histiocitosis no Langerhans. El 90% de las veces ocurre en etapas tempranas de la vida. Se caracteriza por una pápula o nódulo asintomático, amarillo-anaranjado, que compromete cabeza, cuello y/o tronco superior. Hasta el 82% de los casos se manifiesta de manera única. El ojo es el sitio extracutáneo más frecuentemente comprometido. La dermatoscopía describe un patrón de "puesta de sol". Su pronóstico es bueno y tiende a involucionar a los 3-6 años desde su aparición. Se presenta este caso dado que el XJ múltiple es menos frecuente de observar que su forma única y para recordar que su asociación con neurofibromatosis tipo 1 predispone a un mayor riesgo de desarrollar una leucemia mieloide crónica infantil.

The Juvenile Xanthogranuloma (JX) is a benign tumor of childhood, composed of progressively lipidized histiocytes in the absence of metabolic abnormalities. A case of multiple JX is reported in an infant. Male patient of 1 year and 11 months, without morbid history. Checking injuries in scalp of 15 months of evolution. At physical examination highlights two yellow papules and three orange macules in the scalp. Dermatoscopy shown a homogeneous yellow-orange color. The incisional biopsy showed infiltration of histiocytes in the dermis and Touton cells. At immunohistochemistry, histiocytes were CD68 positive, S100 negative and CD1a negative. Laboratory tests and evaluation by Ophthalmology showed no abnormalities. The JX is a benign tumor, been the most common form non- Langerhans histiocytosis. This taking place 90% of the time in early life, and characterized by yellow-orange asymptomatic papule or nodule, which undertake head, neck and / or upper body. Up to 82% of cases appears uniquely. The eye is the most frequently committed extracutaneous site. The dermoscopy describes a pattern of "setting sun". His prognosis is good and tends to return to the 3-6 years since their appearance. We present this case because the multiple JX is less common to observe that this singular form, and to remember that their association with neurofibromatosis type 1 predisposes to increased risk of developing childhood chronic myeloid leukemia.

Giant xanthogranuloma of the pelvis with S1 origin: Complete removal with only posterior approach, technical note.

Xanthogranulomas (XG) are benign proliferative disorder of histiocytes, a non-Langerhans cell histiocytosis. Whose etiology is unknown. The nature of these lesions is controversial and could be either reactive or neoplastic; the presence of monoclonal cells does, however, favor the second hypothesis. Xanthogranuloma is frequently found in young adults and children (under 20 years old), mainly in the skin. In about 5%-10% of all Juvenile XG (JXG) cases xanthogranuloma are extracutaneous. Within this group, the site most frequently involved is the eye. Other involved organs are heart, liver, adrenals, oropharynx, lung, spleen, central nervous system and subcutaneous tissue, although involvement of the spine is uncommon. Isolated lesions involving the sacral region are extremely rare. To date, this is the first reported case of a giant JXG arising from S1 with extension into the pelvic region in an adult spine.

Intracardiac juvenile xanthogranuloma with presentation in adulthood.

Juvenile xanthogranuloma is the most frequent type of non-Langerhans cell histiocytosis. It most commonly presents in infancy and early childhood; manifesting as cutaneous lesions on the head, neck, and trunk that suddenly appear and usually undergo spontaneous regression. Extracutaneous involvement, although rare, may occur along with the cutaneous form or in isolation. It most frequently involves the eye, deep subcutaneous tissues, lung, and liver. Involvement of the heart is exceptionally rare, with only seven reports found in the English literature, all affecting infants. We present the first report of an intracardiac juvenile xanthogranuloma in an adult.