Verruciform xanthoma associated with lichen planus

Verruciform xanthoma (VX) is a rare benign lesion of unknown etiology, with a rough or papillary aspect, painless, sessile, well-defined, most lesions do not exceed 2 cm in their largest diameter, the degree of keratinization of the surface influences color, varying white to red, affecting mainly the gingiva and alveolar mucosa, and can also be seen in skin and genital. Herein, we present a report a clinical case of oral verruciform xanthoma in the buccal mucosa associated with the lichen planus lesion, as well as the morphological and immunohistochemical characteristics of the lesion. The clinical diagnostic hypothesis of oral lichen planus of the white reticular lesions on the buccal mucosa and on the tongue was confirmed by histopathology before a subepithelial connective tissue exhibiting intense inflammatory infiltrate in a predominantly lymphocytic band. In contrast, the hypothesis of the verrucous lesion in the left buccal mucosa was leukoplakia, with histopathological evidence showing exophytic and digitiform proliferations with parakeratin plugs between the papillary projections. Subepithelial connective tissue was characterized by macrophages with foamy cytoplasm (xanthoma cells). An immunohistochemical examination was performed, showing positivity for CD68, a macrophage marker, in addition to testing by Schiff's periodic acid (PAS) with diastasis, which was detected the presence of lipids inside these macrophages. The patient is free of recurrences of verruciform xanthoma and is being monitored due to the presence of lesions of oral lichen planus.

Critérios diagnósticos e estratificação de risco na hipercolesterolemia familiar / Criteria diagnosis and risk stratification of familial hypercholesterolemia

A hipercolesterolemia familiar (HF) é uma doença genética relativamente comum caracterizada por níveis elevados de LDL-colesterol (LDL-C) e, por conseguinte, associada a risco de desenvolvimento prematuro de doença cardiovascular aterosclerótica. O tratamento hipolipemiante reduz significativamente o risco cardiovascular desses pacientes, tornando fundamental a identificação precoce desses indivíduos, seguida de tratamento adequado assim que possível. Para tanto, existem escores diagnósticos de HF, como o escore holandês Dutch Lipid Clinic Network, que avalia níveis de LDL-C, antecedente familiar e/ou pessoal de evento cardiovascular isquêmico e a presença de sinais físicos, como xantomas. Uma vez feito o diagnóstico de HF, torna-se muito importante a estratificação de risco desses pacientes. A identificação de fatores de risco associados (como tabagismo,diabetes mellitus, hipertensão arterial, aumento de Lp(a), entre outros) aliada ao uso de métodos para detecção de doença aterosclerótica subclínica em indivíduos com HF pode auxiliar na identificação daqueles que têm maior risco cardiovascular e são candidatos a estratégias mais agressivas de redução de LDL-C. Nesse artigo, revisamos os principais critérios diagnósticos de HF e a estratificação de risco desses pacientes

Familial hypercholesterolemia (FH) is a relatively common genetic disease that is characterized by elevated LDL-cholesterol (LDL-C) levels. As a consequence, it is associated with the risk of premature development of atherosclerotic cardiovascular disease.Lipid-lowering therapies significantly reduces the cardiovascular risk in these patients, making early identification of these individuals essential, followed by adequate treatment as soon as possible. There are diagnostic scores of FH for this purpose, such as the Dutch Lipid Clinic Network score, which evaluates LDL-C levels, family history and/or personal history of ischemic cardiovascular event and the presence of physical signs, such as xanthomas. Once FH has been diagnosed, it is very important to stratify the risk in these patients. The identification of associated risk factors (such as smoking, diabetes mellitus, high blood pressure, elevated Lp(a), among others), together with the use of methods to detect subclinical atherosclerotic disease in individuals with FH, can assist in the identification of those with a higher cardiovascular risk, and who are therefore candidates for more aggressive strategies to reduce LDL-C. This article gives a review of the main diagnostic criteria of FH, and the risk stratification in these patients

Simultaneous Xanthogranulomatous Cholecystitis and Gallbladder Cancer in a Patient with a Large Abdominal Aortic Aneurysm

There have been reports of the coexistence of abdominal aortic aneurysm (AAA) with intra-abdominal malignancy including gastric, colonic, pancreatic, and renal. We herein report a case of a previously undiagnosed AAA and a presenting complaint consistent with acute cholecystitis. Following cholecystectomy, this was noted to be a rare form of chronic cholecystitis: xanthogranulomatous cholecystitis. There is a known possible association of this uncommon condition with gallbladder cancer. The management of concomitant pathologies can present a real challenge to the multidisciplinary team, especially with large aneurysms.

Diffuse plane xanthomatosis associated with monoclonal gammopathy / Xantomatose plana difusa associada a gamopatia monoclonal

Diffuse plane normolipemic xanthomatosis (DPNX) is a rare, non-inherited disease that is often associated with systemic diseases, mainly malignant hematological (especially multiple myeloma) or lymph proliferative disorders. The DPNX can precede the appearance of such conditions by several years, so careful follow-up and periodic laboratory examinations are recommended even for patients that seemed to have no underlying disease. We describe a case associated with monoclonal gammopathy. This case shows that dermatological lesions can be the first manifestation of important hematological diseases and so physicians should be familiarized with this entity.

A xantomatose plana difusa normolipêmica (XPDN) é uma dermatose adquirida rara, muitas vezes associada a doenças sistêmicas, nomeadamente neoplasias hematológicas(sobretudo o mieloma múltiplo) ou a processos linfoproliferativos. A XPDN pode preceder o aparecimento dessas doenças em vários anos, sendo por isso recomendada uma vigilância clínica e laboratorial periódica, mesmo para os doentes que aparentemente não apresentam uma doença associada. Descrevemos um caso associado à gamopatia monoclonal. Este caso demonstra a importância das manifestações cutâneas como primeira manifestação de doenças hematológicas importantes e por isso os clínicos devem estar familiarizados com esta entidade.

Xanthogranulomatous Pancreatitis Presents as a Solid Tumor Mass: A Case Report

Xanthogranulomatous inflammation (XGI) is a rare, idiopathic process in which lipid-laden histiocytes are deposited at various locations in the body. Although XGI has been reported to occur in various organs such as the gallbladder, kidney, bone, stomach, colon, appendix, lymph nodes, urachus, and urinary bladder and in soft tissues, xanthogranulomatous pancreatitis (XGP) is extremely rare. Herein, we report a case of XGP occurring in a 70-yr-old woman, who presented with abdominal pain for several months. On physical examination, mild epigastric tenderness was noted. Abdomen CT scan revealed a low attenuated mass in uncinate process of pancreas, suggesting malignant lesion. Whipple's operation was performed and the final pathologic diagnosis was XGP. The patient's post-operative course was uneventful, and no recurrence was found within 7 months of the operation. When a pancreatic mass does not show clinico-radiological features typical of common pancreatic neoplasms, XGP should be considered for a differential diagnosis.

Xantoma plano difuso normolipêmico idiopático com hiperesplenismo / Diffuse plane idiopathic normolipemic xanthoma with hiperesplenism

O xantoma plano difuso normolipêmico é doença rara, do grupo das histiocitoses, caracterizada pelo aparecimento de placas amareladas ou amarelo-alaranjadas, distribuídas simetricamente na pele e geralmente acompanhadas por xantelasma. Acomete principalmente adultos, podendo ou não apresentar alterações discretas dos lípides séricos. Relata-se o caso de uma paciente do sexo feminino, de 85 anos, há um ano com extensas placas amarelo-alaranjadas no tronco e abdome, assintomáticas. Os exames laboratoriais não demonstraram aumento dos lípides séricos, nem ocorrência de desordens retículo-endoteliais.

Diffuse plane normolipemic xanthoma is a rare disease, of a group of clinical syndromes called histiocytoses, characterized by the presence of yellowish or yellow-orange plaques, distributed symmetrically on the cutaneous surface and usually accompanied by xanthelasma. It affects mainly adults and it may cause discrete changes in serum lipids. The case of an 85-year-old female patient who has been showing extensive asymptomatic yellow-orange plaques in the trunk and abdomen for a year is reported. Laboratory tests did not show an increase in serum lipids or the occurrence of reticuloendothelial disorders.

Xanthogranulomatous Pancreatitis Combined with Intraductal Papillary Mucinous Carcinoma In Situ

Xanthogranulomatous lesion is a rare condition in which lipid-laden histiocytes are deposited at various locations in the body. Xanthogranulomatous pancreatitis (XGP) associated with an intraductal papillary mucinous tumor (IPMT) is extremely rare. In this study, we described a case of XGP associated with IPMT and include a review of the literature. A pancreatic cystic mass was detected in a 72-yr-old woman by abdominal computed tomography. Pylorus-preserving pancreaticoduodenectomy was performed and diagnosis of XGP combined with intraductal papillary mucinous carcinoma in situ was made. After 13 months of follow-up, the patient is in good health without any evidence of tumor recurrence. Although XGP associated with IPMT is rare, we suggest that such cases should be brought to the attention of clinical investigators, as it may produce clinical features that mimic pancreatic cancer.

Xanthogranulomatous cholecystitis: differentiation from associated gall bladder carcinoma.

Xanthogranulomatous cholecystitis (XGC) is a destructive form of chronic cholecystitis. In some patients it coexists with gall bladder carcinoma (GBC) and is often difficult to differentiate between the two. Present study was performed with an aim to identify differentiating features of XGC and those of XGC with associated Gall bladder carcinoma (XGC ass. GBC). A retrospective analysis of prospectively maintained data of 4800 cholecystectomies performed from January 1988 to December 2003 was carried out. On histopathology 453 cholecystectomy specimens revealed XGC. These patients were divided into two groups, those with associated GBC (n=26) and those without GBC (n=427). Clinical, radiological and operative findings were compared in these two groups. P value of < 0.05 was considered statistically significant. The incidence of associated GBC in present series was 6%. XGC patients with associated GBC, at presentation were older than those with XGC alone and there was male preponderance. XGC patients with associated GBC were more likely to present with anorexia, weight loss, palpable lump and jaundice. Gall stones were present in majority of patients in both the groups. GB wall thickening, GB mass, enlarged abdominal lymph nodes may be found on imaging in both the groups but more so in patients with associated GBC. Both preoperative FNAC and peroperative FNAC/imprint cytology failed to reveal the associated GBC with XGC in some patients.

Xanthogranulomatous cholecystitis.

Twelve cases of xanthogranulomatous cholecystitis are reported. The clinical presentation was similar to chronic cholecystitis. All patients had associated gallstones. The diagnosis was achieved at histopathological examination of the resected gall bladders and none had any focus of malignancy. Cholecystectomy was curative.

A Case of Simultaneous Xanthogranulomatous Cholecystitis and Carcinoma of the Gallbladder

Xanthogranulomatous cholecystitis (XGC) is a rare inflammatory disease of the gallbladder. Not only does XGC occasionally present as a mass formation with adjacent organ invasion like a malignant neoplasm, it can also infrequently be associated with gallbladder cancer. In the situation, it is difficult to make a differential diagnosis between the diseases. Here, we describe a case of a simultaneous XGC and a carcinoma of the gallbladder in a 61-year-old woman. To the best of our knowledge, there are only a small number of reports on this combination of diseases.

Xantoma y ateroma, hipercolesterolemia familiar homo y heterocigota: riesgo de infarto precoz / Xanthoma and atheroma, homo and heterozygous familiar hypercholesterolemia: risk of early myocardiac infarct

Se presentan dos pacientes con hipercolesterolemia familiar y xantomas en cada una de sus variantes genéticas: homocigota y heterocigota. Se hace revisión y actualización de ambos cuadros: clínica, dermatológica, histopatológica, de laboratorio, por ultrasonografía, genética y terapéutica, destacando la importancia del dignóstico precoz a través de xantoma; para la prevención y el tratamiento oportuno de enfermedades cardiovasculares graves

Xanthelasma of esophagus and stomach.

Gastric xanthelasma, a benign condition, has been reported before; there has been no documentation of xanthelasma of the esophagus. We report a patient with xanthelasma of the stomach and esophagus.

Xantomatosis plana difusa asociada a gammapatía monoclonal / Diffuse plane xantomatosis associated with monoclonal gammapathy

La xantomatosis plana difusa es una enfermedad rara, dentro del grupo de las xantomatosis. Se presenta con infiltración y cambio de coloración de la piel; puede asociarse a enfermedades sistémicas, las que marcan su pronóstico. Se presenta un caso de xantomatosis plana difusa asociada a gammapatía monoclonal