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.

Fibroxantoma atípico: caso clínico / Scalp atypical fibroxanthoma: report of one case

Atypical Fibroxanthoma is an unusual dermal mesenchymal tumor. It especially affects older adults and occurs in areas of sun exposure. We report a 75 years old male with a history of sun exposure without using a hat presenting with a scalp nodule. An incisional biopsy showed an atypical fibroxantoma. In a new surgical procedure, the tumor was completely excised. The tumor relapsed in two occasions after subsequent excisions and the patient was treated with adjuvant radiotherapy avoiding new relapses.

Fox-Fordyce disease: report of two cases with perifollicular xanthomatosis on histological image

Abstract: Fox-Fordyce disease is a relatively infrequent pathology of the apocrine glands that affects almost exclusively young women. The disease is characterized by the presence of pruritic follicular papules mainly in the armpits that respond poorly to treatment and severely affect the patient's quality of life. We report two cases with clinical diagnosis and histopathological confirmation, presenting perifollicular xanthomatosis on histological examination, recently described as a distinctive, consistent, and specific feature of this disease.

Serum lipid profile and clinical characteristics of patients with xanthelasma palpebrarum

Abstract: Background: Although many factors are involved in the etiology of xanthelasma palpebrum, lipid disorder is strongly associated with its induction. Xanthelasma palpebrum, the most common type of xanthoma, usually presents in middle-aged females and results in aesthetic problems. Objective: To evaluate thelipid profile and important clinical aspects of xanthelasma palpebrum patients. Methods: In this descriptive study, we enrolled 42xanthelasma palpebrumpatients, and 42 cases of non-inflammatory skin disorders as thecontrol group, matched for age and gender.The clinical characteristics of the patients and fasting serum lipid profile were recorded for both groups. The data obtained were analyzed using SPSS-16. Results: Xanthelasma palpebrum was found more commonly in middle-aged females with disease onset of less than 1 year, and without significant familial history of xanthoma. Furthermore,xanthelasma lesionswere most often seen in the upper lid with mild extension and was rarely associated with systemic disease. There was no statistically significant difference between two groups regarding hypertriglyceridemia (p= 0.231) and hypercholesterolemia (p= 0.302). The mean serum levels of cholesterol (221.51±60.4 mg/dl), triglyceride (185.98±71.1 mg/dl) and VLDL (37.7±17.6 mg/dl) were significantly higher and themedian HDL (36.2 (31, 41) mg/dl) level was lower in thepatient group. Conclusion: In our study, hypercholesterolemia and hypertriglyceridemia did not reveal a significant difference between thepatient and control groups; however, mean serum values for cholesterol, triglyceride, VLDL and HDL showed a significant difference between the two groups. Therefore, in addition to lipid abnormality, other factors could be involved in the pathogenesis of xanthelasma palpebrum.

Two friends with eroded nodules on the ears: atypical fibroxanthoma case report

AbstractAtypical fibroxanthoma is an uncommon mesenchymal tumor that manifests clinically as a reddish papule or nodule in sun-exposed areas of the body. The clinical presentation is not specific and histology and immunohistochemistry are both necessary for a correct diagnosis. Surgery is the gold standard of therapy. Recurrence and metastasis should be excluded with a follow-up at 6 months, since this tumor should nowadays be considered a medium-grade neoplasm, rather than low-grade as previously believed. We report the case of two friends who came to our hospital during the same period, complaining of very similar lesions. After biopsy and immunohistochemical examination, a diagnosis of atypical fibroxanthoma in both cases was formulated.

Tumor fibromixoma lipoesclerosante óseo: Presentación de un caso y revisión de la literatura / Bone liposclerosing myxofibrous tumor: Case presentation and literature review

El tumor fibromixoma lipoesclerosante óseo (TFMLE) fue descrito inicialmente por Ragsdale en 1986, como una lesión fibroósea polimórfica de hueso con una mezcla de elementos histológicos incluyendo lipoma, fibroxantoma, mixoma, mixofibroma, necrosis grasa, osificación isquémica, áreas de displasia fibrosa y rara vez cartílago o cambios quísticos. La localización más frecuentemente descrita es en la región intertrocantérica del fémur. Es un hallazgo en pacientes asintomáticos o bien estar asociado a fractura. Radiológicamente es una lesión lítica, geográfica, de margen bien definido y habitualmente esclerótico, en algunos casos puede observarse mineralización en el interior de la lesión o un cierto grado de expansión en el contorno. Se ha descrito la estrecha relación del TFMLE con la displasia fibrosa por las características histológicas y la presencia de una mutación Gsα, otra hipótesis de la etiología de la lesión incluye la reacción de la displasia fibrosa a la fatiga por estrés.

The bone liposclerosing myxofibrous tumor (LSMFT) was initially described by Ragsdale in 1986 as a polymorphic fibroosseous bone lesion with a mix of histologic elements that include lipoma, fibroxanthoma, myxoma, myxofibroma, fat necrosis, ischemic ossification, areas of fibrous dysplasia, and infrequent presence of cartilage or cystic changes. The most frequently reported location is the intertrochanteric area of the femur. Radiologically it is a lytic, geographic lesion, with well-defined margins and usually sclerotic. In some cases findings include mineralization inside the lesion or a certain degree of expansion to the contour. The close relationship between LSMFT and fibrous dysplasia has been described based on the histologic characteristics and the presence of the Gsα mutation. Another hypothesis of the etiology of the lesion is the reaction of fibrous dysplasia to stress.

Kikuchi´s disease of the xanthomathous type with cutaneous manifestations

Kikuchi's disease is a benign, self-limiting disease, whose pathogenesis remains unknown. Patients most often present with cervical lymphadenopathy, sometimes associated with fever and leukopenia. It has been reported that up to 40% of patients with Kikuchi's disease have also cutaneous eruptions, but no specific skin changes have been described. Kikuchi's disease can be subclassified into three histologic subtypes: a proliferative type, a necrotizing type and a xantomathous type. Most patients with Kikuchi's disease require no specific treatment, because the disease regresses spontaneously, within a few weeks to months. We report a case of a 31-year-old woman with xanthomatous type of Kikuchi's disease, whose first manifestation was the onset of erythematous papules with central suppuration on her face and on her left hand.

Xantomas eruptivos: manifestación cutánea de desórdenes de las lipoproteínas / Eruptive xanthomas: skin manifestation of the lipoprotein disorders

Los xantomas constituyen tumores cutáneos, y se presentan por depósitos de lipoproteínas en los macrófagos tisulares. Clínicamente se manifiestan como lesiones papulosas o nodulares de color amarillento, estando relacionada su distribución con las diferentes formas clínicas de presentación. Aunque no se observan con frecuencia, su presencia puede alertar sobre la existencia de alteraciones en los niveles lipídicos en sangre, y es por lo que se presentó este caso, donde la presencia de los xantomas fue indicador de un incremento de los niveles de colesterol y triglicéridos, lo que pudo constituir un importante factor de riesgo para que la paciente presentara alteraciones en otros sistemas...

Xanthomas are skin tumors, and they present as a cause of lipoprotein deposits in tissue macrophages. Clinically they take the form of yellow papular or nodular lesions, being related its distribution with their different forms of presentation. Though they are not very frequent, their presence can alert about the existence of alterations in the lipid levels in blood. That is why we presented this case, where the presence of xanthomas was an indicator of the cholesterol and triglycerides level increase, what probably was an important risk fact for the patient to present alterations in other systems...

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.

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.

Xantoma plano difuso normolipémico asociado a mieloma múltiple: comunicación de un caso y revisión de la literatura / Normolypemic diffuse plane xanthoma associated with multiple myeloma: case report and literature review

El xantoma plano difuso es una entidad poco frecuente que se incluye dentro del grupo de las xantomatosis normolipémicas. Suele asociarse con alteraciones del sistema retículo endotelial, sobre todo con gammapatía monoclonal y mieloma múltiple. A continuación se comunica el caso de una paciente de sexo femenino, de 65 años de edad, que consultó por placas amarillents sobreelevadas localizadas en región periorbitaria, submamaria, axilas y miembros superiores. El estudio histológico de las lexiones cutáneas confirmó el diagnóstico de xantoma y los estudios efectuados con posterioridad como la punción de médula ósea y la proteinuria de Bence-Jones revelaron datos consistentes con mieloma múltiple.

A Case of Cholangiocarcinoma Suspected by Continuous Elevation of CA 19-9 after Surgery of Xanthogranulomatous Cholecystitis / 대한소화기학회지

Xanthogranulomatous cholecystitis (XGC) is an unusual and destructive inflammatory process that is characterized by thickening of the gallbladder (GB) wall with a tendency to adhere to neighboring organs. XGC is often mistaken for GB carcinoma, and the frequency of the coexistence of these two lesions is approximately 10%. Therefore, in case of severe XGC, there is chance of either overlooking the carcinoma or other significant lesions. CA 19-9 is commonly measured in the serum of patients with hepatobiliary malignancies. Although CA 19-9 can be elevated in benign conditions such as cholestasis, pancreatitis, tuberculosis, thyroid disease etc., malignancy should be considered at first in setting of its significant and persistent elevation. We report a case of a 62-year-old man who showed continuously rising level of CA19-9 over 2000 U/mL after cholecystectomy for xanthogranulomatous cholecystitis and finally was diagnosed as cholangiocarcinoma by short-term follow up.

Xantoma eruptivo com aspecto histopatológico inusitado simulando granuloma anular: relato de caso / Eruptive xanthoma with unexpected granuloma annulare-like microscopic appearance: case report

Xantoma eruptivo e granuloma anular são doenças dermatológicas com quadros clínicos distintos que, algumas vezes, apresentam semelhanças histopatológicas que podem conduzir a diagnóstico errôneo. Relata-se o caso de paciente do sexo masculino com 34 anos, portador de dislipidemia, com lesões clinicamente características de xantoma eruptivo cujo exame histopatológico foi sugestivo de granuloma anular. No entanto, a revisão da lâmina mostrou tratar-se de xantoma eruptivo. A remissão completa e rápida das lesões após o tratamento da dislipidemia confirmou o diagnóstico de xantoma eruptivo e motivou a pesquisa sobre as semelhanças e diferenças histopatológicas entre essas doenças.

Eruptive xanthoma with unexpected granuloma annulare-like microscopic appearance - Case report Abstract: Eruptive xanthoma and granuloma annulare are dermatological diseases with different clinical findings that, sometimes, exhibit histopathological similarities with potential for misinterpretation. We report a case of an eruption of yellow-orange papules with erythematous borders in a 34-year-old male with high levels of serum triglycerides and cholesterol. The skin biopsy specimen has diagnosed granuloma annulare. Review of the histologic material revealed eruptive xanthoma. Remission of the eruption after treatment of dyslipidemia confirmed the diagnosis of the eruptive xanthoma and motivated research about the histological similarities and differences between these diseases.

Xantoastrocitoma pleomórfico na infância: relato de dois casos / Pleomorphic xanthoastrocytoma: report of two cases

O xantoastrocitoma pleomórfico é uma neoplasia rara,classificada como grau II ou III pela Organização Mundial da Saúde,a depender do aspecto histopatológico.Descrevemos dois casos de portadores de xantoastrocitoma pleomórfico,cujo tratamento escolhido foi o cirúrgico.A associação com quimioterapia foi realizada em um dos pacientes.Haja vista a baixa freqüência desse tipo de neoplasia,torna-se fundamental a realização de novos estudos para o desenvolvimento de protocolos de tratamento que vislumbrem a diminuição da taxa de recidivas.