Xantomas tuberosos en un paciente pediátrico: la clave en el diagnóstico precoz de una patología grave y poco frecuente / Tuberous xanthomas in a pediatric patient: a clue to the early diagnosis of a severe and infrequent disease

Los xantomas cutáneos reflejan el depósito de lípidos en la piel y pueden ser la única manifestación temprana de dislipidemias de inicio en la infancia. Las características y distribución de los xantomas orientan a la patología de base; los xantomas tuberosos tienen una fuerte asociación con la hipercolesterolemia homocigota familiar, una patología muy infrecuente. Su detección temprana otorga una ventana terapéutica para prevenir la ateroesclerosis acelerada y la mortalidad. Se presenta el caso de una paciente que comenzó a los dos años con xantomas tuberosos, que fueron la clave diagnóstica para identificar la hipercolesterolemia homocigota familiar subyacente.

Cutaneous xanthomas reflect lipid deposition on the skin and may be the only early manifestation of a childhoodonset dyslipidemia. Characteristics and distribution of the xanthomas signalize the underlying pathology, tuberousxanthomas being strongly associated with homozygous familial hypercholesterolaemia, an extremely rare condition. Its early detection provides a therapeutic window to prevent accelerated atherosclerosis and mortality. We present the case of a patient who started at two years with tuberous xanthomas, which were the diagnostic clue to identify the underlying homozygous familial hypercholesterolaemia.

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.

Xantoma del tendón de aquiles bilateral: debut de xantomatosis cerebrotendinosa / Bilateral achilles tendon xanthoma: cerebrotendinous xanthomatosis debut

OBJETIVO Presentación de un inusual caso de xantomatosis cerebrotendinosa en un paciente de edad cuya primera manifestación fueron xantomas bilaterales del tendón de Aquiles. MATERIAL Y MÉTODOS Mujer de 62 años, que presenta tumoraciones, que presenta tumoraciones sólidas y polilobuladas, en la cara posterior de ambos tendones de Aquiles de 8 años de evolución. El diagnóstico se realizó mediante el hallazgo de hiperlipidemia y estudio genético. Se realió la exóresis quirúrgica parcial de las tumoraciones. RESULTADOS A los 5 años de la cirugía del pie izquierdo y 4 años del pie derecho la paciente estaba asintomática. Presentaba una fuerza para la flexión plantar bilateral de 5/5, pudiendo caminar y subir escaleras sin molestias. Presentaba una escala AOFAS de 85 y 90 puntos en el pie izquierdo y derecho, respectivamente. No hubo recidivas. DISCUSIÓN Los xantomas son depósitos de colesterol en el tejido conectivo de la piel, tendones o fascia, como resultado de una hiperlipoproteinemia. La importancia del caso radica en su sospecha diagnóstica, ya que la xantomatosis cerebrotendinosa suele manifestarse en pacientes de menos de 30 años de edad y en los que se ha recomendado la resección radical de las tumoraciones, e incluso del tendón, debido a las frecuentes recidivas. CONCLUSIÓN En pacientes de mayor edad con lesiones que infiltran el tendón, se puede optar por un tratamiento menos agresivo con un buen resultado clínico.

OBJETIVE Presentation of an unusual case of cerebrotendinous xanthomatosis in an elderly patient whose first manifestation was bilateral Achilles tendon xanthomas. MATERIAL AND METHODS 62-year-old woman presenting solid and polylobed tumors on the posterior aspect of both Achilles tendons for eight years. The diagnosis was made by means of hyperlipidemia and a genetic study. Surgical partial excision of the tumors was performed. RESULTS Five years after surgery on the left foot and four years after the right foot, the patient was asymptomatic. Bilateral plantar flexion force was 5/5, The patient was able to walk and climb stairs without discomfort. AOFAS score was 85 and 90 on the left and right feet, respectively. There were no recurrences. DISCUSSION Xanthomatosis is a genetic alteration with deposits of cholesterol in connective tissue of the skin, tendons or fascia, because of hyperlipoproteinemia. The importance of the present case lies in its diagnostic suspicion, since cerebrotendinous xanthomatosis manifests usually in patients under 30 years of age and in whom radical resection of tumors, and even of the tendon, has been recommended due to frequent recurrences . CONCLUSION In older patients with tumors that infiltrate the tendon, a less aggressive treatment can be chosen with a good clinical result.

Análise do tratamento cirúrgico dos xantelasmas palpebrais: experiência de um serviço de Cirurgia Plástica / Analysis of surgical treatment of eyelid xanthelasmas: experience of a Plastic Surgery service

Introdução: Xantelasmas são placas amareladas benignas que acometem pele de pálpebras e cursam com limitação funcional e queixa estética, impactando vida social e emocional. Com prevalência de 1,4% na população geral, predominam no sexo feminino e associam-se a hiperlipidemias, formados por deposições dérmicas de colesterol. De diagnóstico clínico, está indicada histopatologia em casos duvidosos. O tratamento de escolha é cirúrgico, através de exérese simples ou associada a blefaroplastia, epicantoplastia medial, retalhos locais e enxertos de pele total. Outras opções são terapia a laser, cauterização química com ácido tricloroacético, radiofrequência e crioterapia. Apesar da variedade terapêutica disponível, ainda são descritas na literatura taxas de recidiva local de até 40%. Objetivo: Analisar taxas de recidiva local associadas à ressecção cirúrgica enquanto tratamento de escolha para os xantelasmas. Métodos: Trata-se de estudo observacional retrospectivo, em que foram analisados prontuários de 18 pacientes submetidos à ressecção cirúrgica de xantelasmas pelo serviço de Cirurgia Plástica do Hospital Universitário da UFJF nos anos de 2016 a 2018. Foram avaliadas localização das lesões e taxas de recidiva local. Resultados: A localização das lesões mostrou-se predominante em pálpebras superiores, acometendo 10 pacientes (55%), sem predileção por lateralidade. Evoluíram com recidiva local 4 pacientes (22%). Conclusão: Os resultados do presente trabalho corroboram a hipótese de que taxas de recidiva local são menores em casos submetidos a tratamento cirúrgico, quando comparadas às descritas na literatura, bem como maior satisfação dos pacientes com os resultados estéticos apresentados. Conclui-se que a ressecção cirúrgica é segura e satisfatória em relação às demais terapêuticas. (AU)

Introduction: Xanthelasmas are benign yellowish plaques that affect the eyelid skin and cause functional limitation and aesthetic complaint, impacting social and emotional life. With a prevalence of 1.4% in the general population, they predominate in females and are associated with hyperlipidemias, formed by dermal cholesterol depositions. Clinically diagnosed, histopathology is indicated in doubtful cases. The treatment of choice is surgical, through simple or associated excision of blepharoplasty, medial epicantoplasty, local flaps and total skin grafts. Other options are laser therapy, trichloroacetic acid chemical cauterization, radiofrequency and cryotherapy. Despite the available therapeutic variety, local recurrence rates of up to 40% are still described in the literature. Objective: To analyze local recurrence rates associated with surgical resection as the treatment of choice for xanthelasmas. Methods: This was a retrospective observational study, which analyzed the medical records of 18 patients who underwent surgical resection of xanthelasmas by the Plastic Surgery Service of the University Hospital of UFJF from 2016 to 2018. We evaluated lesion location and recurrence rates. Results: The location of the lesions was predominant in the upper eyelids, affecting 10 patients (55%), with no preference for laterality. Four patients (22%) evolved with local recurrence. Conclusion: The results of the present study corroborate the hypothesis that local recurrence rates are lower in cases submitted to surgical treatment when compared to those described in the literature, as well as greater patient satisfaction with the aesthetic results presented. It is concluded that surgical resection is safe and satisfactory in relation to other therapies. (AU)

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.

Xantoastrocitoma pleomórfico / Pleomorphic Xanthoastrocytoma

El xantoastrocitoma pleomórfico, por su extrema rareza, conlleva alta complejidad en el diagnóstico histopatológico. Se presenta el caso clínico en un sujeto de sexo masculino, de 40 años, con antecedentes de crisis convulsivas de presentación tardía, secundarias a meningioma atípico grado II localizado en región occipital izquierda, resecado por dos ocasiones en el transcurso de 6 años. Recibió radioterapia a dosis completa luego de la segunda resección. El diagnóstico histopatológico inicial fue meningioma atípico gra-do II. El paciente acude al HCAM por cefalea holocraneal intensa y hemiparesia braquiocrural derecha; en los estudios de resonancia magnética nuclear con gadolinio se observó el crecimiento de una lesión occipi-tal izquierda con edema perilesional que ameritó resección total de la lesión a través de la craniectomía previa. Como hallazgo macroscópico, se describe una masa violácea que infiltra duramadre carente de un plano de clivaje; el estudio histopatológico detalla una neoplasia glial hipercelular con infiltración difusa con reacción inmunohistoquímica intensa para PGAF (proteína glial acida fibrilar), S100 y CD56 en células tumorales, CD34 positivo. KI67 positivo en 3% y P53 débilmente positivo, compatible con xantoastroci-toma pleomórfico WHO II.(AU)

The pleomorphic xantoastrocytoma, due to its extreme rarity, carries high complexity in the histo-pathological diagnosis. The clinical case is presented in a male subject, 40 years old, with a history of sei-zures with late presentation, secondary to atypical meningioma grade II located in the left occipital region, resected twice in the course of 6 years. He received full-dose radiation therapy after the second resection. The initial histopathological diagnosis was atypical meningioma grade II. The patient comes to HCAM due to intense holocranial headache and right brachiocrural hemiparesis; In the gadolinium nuclear magnetic resonance studies the growth of a left occipital lesion with perilesional edema that warranted total resec-tion of the lesion through previous craniectomy was observed. As a macroscopic finding, a violaceous mass is described which infiltrates dura mater lacking a plane of cleavage; The histopathological study details a hypercellular glial neoplasia with diffuse infiltration with intense immunohistochemical reaction for PGAF (glial acidic glial protein), S100 and CD56 in tumor cells, CD34 positive. It was KI67 positive in 3% and P53 weakly positive, compatible with pleomorphic xantoastrocytoma WHO II. (AU)

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.

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

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.

Los xantomas gástricos están asociados con lesiones malignas y premalignas / Gastric Xanthoma is Associated with Malignant ad Premalignant Lesions

Los xantomas gástricos son lesiones encontradas incidentalmente en la endoscopia digestiva alta. Consisten en la acumulación de lípidos en la mucosa gástrica. Estas lesiones se han reportado en serie de casos asociadas con metaplasia o atrofia y en reportes de casos vinculados con cáncer gástrico; sin embargo, no se han realizado estudios comparativos que busquen comprobar esta asociación. El objetivo de este trabajo es evaluar si los pacientes con xantomas tienen más lesiones malignas (displasia o cáncer) o condiciones premalignas (atrofia o metaplasia). Materiales y métodos: se trata de un estudio retrospectivo de casos y controles realizado en el Hospital El Tunal, en pacientes que fueron sometidos a endoscopia de vías digestivas altas (EVDA) para evaluación de dispepsia. A cada caso de xantoma se le asignó un control de endoscopia realizado el mismo día por el síntoma de dispepsia y escogido de manera aleatoria. Todos los pacientes fueron sometidos a biopsias para evaluación histológica y por infección de Helicobacter pylori. Resultados: en total se reclutaron 186 pacientes que cumplieron los criterios de inclusión, ya que tenían indicación de EVDA porque cursaban con dispepsia no investigada. Fueron comparados dos grupos de pacientes: el primero, con hallazgo de xantomas gástricos en la endoscopia (n = 90) y el segundo, sin estos hallazgos (n = 96). El promedio de edad de los pacientes con xantomas fue de 57, 4 versus 38,3 del grupo control, 35% sexo masculino versus 35,6%, respectivamente. Los xantomas fueron únicos en el 72%. La mayoría se ubicó en el antro (53%), cuerpo (32%), antro o cuerpo (10%) y fondo (5%). En los pacientes sin xantomas no se presentaron casos de displasia (0%), mientras que en el grupo con xantomas, 5,5% de los pacientes lo presentó (5 casos), de los cuales 2 fueron de bajo grado y 3, de alto grado. El 66% del grupo xantoma versus el 85% del control tenía infección por H. pylori. Tres pacientes presentaron ...

Gastric xanthoma lesions, which involve lipid accumulation in the gastric mucosa, are found incidentally during upper endoscopy. These lesions have been reported in series of cases associated with metaplasia or atrophy and in reports that link them to gastric cancer, but no analysis has been done to date that examines the possible connections among these associations. The aim of this study was to evaluate whether patients with xanthoma have more malignant lesions (dysplasia or cancer) or premalignant conditions (atrophy or metaplasia). Materials and Methods: This is a retrospective case-control study that was conducted in the Hospital El Tunal among patients who underwent upper endoscopies for evaluation of dyspepsia. Participants were chosen at random from among patients who had symptoms of dyspepsia. Each participant underwent endoscopy to check for xanthoma on the same day. All patients had biopsies taken and were assessed histologically for H. pylori infection. Results: The study enrolled a total of 186 patients who met the inclusion criteria of requiring upper endoscopy due to symptoms of dyspepsia. as was indicated field-work one EVDA that with uninvestigated dyspepsia were recruited. Two groups of patients were compared: one included 90 patients who were diagnosed with xanthoma through gastric endoscopy (n = 90), and another group which consisted of 96 patients in whom no symptoms of xanthoma were found. The average age of the patients with xanthoma was 57.4 years while the average age of the control group was 38.3 years. 35% of the xanthoma group were male while 35.6% of the control group were male. 72% of the patients in the xanthoma group had only one lesion: 53% were located in the antrum, 32% were located in the corpus, 10% in the antrum or corpus, and 5% in the fundus. Among patients without xanthomas, there were no cases of dysplasia (0%) while 5.5% of the patients with xanthomas (5 cases) developed dysplasia. Two were low ...

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.

Aspectos de imagem do xantoma do tendão calcâneo na ultrassonografia e ressonância magnética / Achilles tendon xanthoma imaging on ultrasound and magnetic resonance imaging

O xantoma no tendão calcâneo é uma doença rara e tem uma alta associação com hiperlipidemia primária. O diagnóstico precoce é fundamental para o início do tratamento e para alterar o curso da doença. Os exames de imagem podem auxiliar nesse diagnóstico. Este estudo relata o caso de um homem de 60 anos apresentando nódulos indolores nos cotovelos e tendões calcâneos, sem crises típicas de gota, acompanhado no ambulatório de doenças microcristalinas da Unifesp para esclarecimento diagnóstico. Os testes laboratoriais solicitados apresentavam dislipidemia. Ultrassom (US) mostrou espessamento difuso dos tendões calcâneos com áreas hipoecoicas. Ressonância magnética (RM) mostrou espessamento difuso dos tendões, com áreas de sinal intermediário e padrão reticulado no interior. Os exames de imagem mostraram aspectos relevantes no diagnóstico de xantoma, auxiliando no diagnóstico diferencial.

The Achilles tendon xanthoma is a rare disease and has a high association with primary hyperlipidemia. An early diagnosis is essential to start treatment and change the disease course. Imaging exams can enhance diagnosis. This study reports the case of a 60-year-old man having painless nodules on his elbows and Achilles tendons without typical gout crisis, followed in the microcrystalline disease clinic of Unifesp for diagnostic workup. Laboratory tests obtained showed dyslipidemia. The ultrasound (US) showed a diffuse Achilles tendon thickening with hypoechoic areas. Magnetic resonance imaging (MRI) showed a diffuse tendon thickening with intermediate signal areas, and a reticulate pattern within. Imaging studies showed relevant aspects to diagnose a xanthoma, thus helping in the differential diagnosis.

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.

Xantomatosis eruptiva asociada a hiperlipoproteinemia tipo IV de Fredrickson: a propósito de un caso / Eruptive xanthomatosis associated with Fredrickson type IV hyperlipoproteinemia: case report

Los xantomas cutáneos son una expresión de depósito de lípidos en la piel; pueden ser normolipémicos o dislipémicos, producidos por un defecto genético primario o una alteración metabólica. Las manifestaciones cutáneas de las xantomatosis se presentan con diversas características. Presentamos una paciente joven, sin antecedentes patológicos previos, que consultó por la aparición repentina de lesiones xantomatosas cutáneas, en la cual se arribó al diagnóstico de xantomatosis eruptiva asociadaa trastorno lipídico subyacente. El interés de nuestra presentación radica en que esta afección cutánea puede constituir la única manifestación de las alteraciones de las lipoproteínas, clasificadas dentro del grupo de las dislipidemias de Fredrickson y ser el punto de partida para su diagnóstico y tratamiento oportuno ya que generan un compromiso sistémico y riesgo de vida.

Cutaneous xanthomas are an expression of lipid deposition on the skin. They can be normolipidemic or dyslipidemic and are caused by a primary genetic defect or a metabolic disorder. Cutaneous manifestations of xanthomatosis show various features. We report a young woman with no previous medical history who presented sudden onset of cutaneous xanthomatous lesions, and whose diagnosis showed eruptive xanthomatosis associated with an underlying lipid disorder. The interest of our presentation is that this cutaneous affectation may be the only manifestation of lipoproteins disorders, classified within the group of Fredrickson's dyslipidemias and also the starting point for an appropiate diagnosis and treatment because they generate a systemic commitment and life threatening.