RESUMEN
BACKGROUND: Oral verruciform xanthoma (OVX) is a rare lesion. The purpose of the present study is to describe the clinical features of 13 OVXs and review all cases reported in the English literature. MATERIAL AND METHODS: Thirteen cases of OVX diagnosed during a 47-year period were retrospectively collected. The patients gender and age, as well as the main clinical features of the lesions were retrieved from the biopsy request forms. Pubmed, Scopus and Google ScholarTM electronic databases were searched with the key word verruciform xanthoma. Only cases of histologically confirmed OVX were included in the study. RESULTS: The 13 OVXs represented approximately 0.04% of 35,617 biopsies accessioned during the study period. They affected 13 patients, 8 males and 5 females with a mean age of 48.8±14 years. They mainly appeared as asymptomatic granular nodules or plaques, with elastic or normal consistency and white color, in the gingiva or hard palate. Literature review yielded 416 cases of OVX. With the addition of cases of the present study, 429 cases of OVX have been presented in the English literature. OVX has a slight male predominance with a male to female ratio of 1.4:1 and the majority of patients are in the 5th to 7th decade of life. Clinically, OVX mainly presents as an asymptomatic, single, papillary or granular plaque or nodule, with elastic or soft consistency and white, red or pink color. It measures approximately 1cm and is most commonly located on the gingiva, tongue, hard palate or buccal mucosa. The treatment of choice is surgical excision with little rates of recurrence. CONCLUSIONS: Verruciform xanthoma is a rare lesion most often encountered on the ginigival mucosa. As its clinical presentation is not pathognomonic, it should be included in the differential diagnosis of verrucous or papillary lesions.
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Enfermedades de la Boca , Xantomatosis , Adulto , Anciano , Femenino , Humanos , Masculino , Persona de Mediana Edad , Enfermedades de la Boca/diagnóstico , Enfermedades de la Boca/terapia , Estudios Retrospectivos , Xantomatosis/diagnóstico , Xantomatosis/terapiaRESUMEN
Xanthogranulomatous osteomyelitis (XO) is an exceedingly rare disease characterized by infiltration of histiocytes and foamy macrophages. Both on gross examination and on radiographs, XO can mimic malignancy. We describe the case of a 5-year-old female with Alagille syndrome who presented with a pathologic fracture of the right humerus. Initial radiographs revealed multiple osteolytic lesions in the distal humerus while MRI showed a large soft tissue mass. Biopsy confirmed the diagnosis of XO, which has hitherto not been described in a patient with Alagille syndrome. The patient was admitted for IV antibiotics and taken to the operating room for an incision and debridement. Tissue cultures were obtained and grew Salmonella. Antibiotic therapy was tapered, and the patient responded to treatment. She was doing well at her 6-month follow-up visit. In the discussion section, we explore how osteopenia and immune dysregulation caused by Alagille syndrome can affect the development of XO. We summarize all previously reported cases of XO and conclude that XO presents as an osteolytic lesion that expands rapidly over the course of a few weeks. We highlight that XO can mimic sarcoma because of its mass effect but can be distinguished radiographically by the presence of surrounding sclerosis. Given the rapid onset of XO, we classify it as an acute osteomyelitis. We discuss how leukemia and Ewing sarcoma can present similarly to acute osteomyelitis. We then emphasize key features that can be used to distinguish these malignancies from acute osteomyelitis.
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Síndrome de Alagille/complicaciones , Granuloma/diagnóstico por imagen , Fracturas del Húmero/diagnóstico por imagen , Osteomielitis/diagnóstico por imagen , Xantomatosis/diagnóstico por imagen , Biopsia , Preescolar , Terapia Combinada , Diagnóstico Diferencial , Femenino , Granuloma/microbiología , Granuloma/terapia , Humanos , Fracturas del Húmero/terapia , Imagen por Resonancia Magnética , Osteomielitis/microbiología , Osteomielitis/terapia , Xantomatosis/microbiología , Xantomatosis/terapiaAsunto(s)
Artritis Reumatoide , Artritis , Colesterol , Metotrexato/administración & dosificación , Líquido Sinovial/metabolismo , Xantomatosis , Anciano , Artralgia/diagnóstico , Artralgia/tratamiento farmacológico , Artralgia/etiología , Artritis/diagnóstico , Artritis/etiología , Artritis Reumatoide/sangre , Artritis Reumatoide/complicaciones , Artritis Reumatoide/fisiopatología , Artritis Reumatoide/terapia , Colesterol/análisis , Colesterol/aislamiento & purificación , Diagnóstico Diferencial , Articulaciones de los Dedos/diagnóstico por imagen , Gota/diagnóstico , Humanos , Masculino , Articulación Metacarpofalángica/diagnóstico por imagen , Articulación Metacarpofalángica/metabolismo , Microscopía de Polarización/métodos , Radiografía/métodos , Resultado del Tratamiento , Xantomatosis/complicaciones , Xantomatosis/diagnóstico , Xantomatosis/fisiopatología , Xantomatosis/terapiaRESUMEN
PURPOSE: Adult-onset xanthogranuloma (AOX) of the corneoscleral limbus is a rare inflammatory condition of unknown aetiology. Similar to limbal juvenile xanthogranuloma (JXG), it presents as a growing mass at the corneoscleral junction. Limbal AOX and JXG can lead to sight-threatening complications if not managed in a timely manner. This systematic review summarises the main clinical and histopathological features of limbal AOX/JXG and discusses the management of this uncommon disease. METHODS: We performed a literature search in the MEDLINE database for all historical entries, using the search terms "limbus", "limbal" and "xanthogranuloma", and retrieved all articles reporting on limbal xanthogranuloma. After refining the search to articles relevant to limbal AOX, we were able to identify ten adult cases of limbal AOX and compare those with all reported cases of limbal JXG. RESULTS: Clinically, AOX usually presents as an isolated smooth, yellowish, dome-shaped nodule at the corneoscleral junction, similar to an ocular presentation of JXG, with which it also shares similar histopathological features. CONCLUSION: Limbal JXG and AOX may represent the same disease entity. Diagnosis relies on the clinical presentation, pathology and immunohistochemical profile. Spontaneous regression is unlikely, and thus prompt surgical intervention should be considered to prevent sight-threatening complications. Xanthogranuloma should be included in the differential diagnosis of corneoscleral limbal masses in patients of all age groups.
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Enfermedades de la Córnea , Granuloma , Limbo de la Córnea , Xantogranuloma Juvenil , Xantomatosis , Adolescente , Adulto , Anciano , Preescolar , Enfermedades de la Córnea/diagnóstico , Enfermedades de la Córnea/epidemiología , Enfermedades de la Córnea/terapia , Femenino , Granuloma/diagnóstico , Granuloma/epidemiología , Granuloma/terapia , Humanos , Masculino , Persona de Mediana Edad , Xantogranuloma Juvenil/diagnóstico , Xantogranuloma Juvenil/epidemiología , Xantogranuloma Juvenil/terapia , Xantomatosis/diagnóstico , Xantomatosis/epidemiología , Xantomatosis/terapia , Adulto JovenRESUMEN
Homozygous familial hypercholesterolemia (HoFH) is a very rare condition (1 case per 1 million people) with a dismal outcome due to inevitable coronary artery disease that occurs when left untreated. Lipoprotein apheresis (LA), previously known as low-density lipoprotein (LDL) apheresis, is very effective in reducing LDL-cholesterol (LDL-C) if HoFH is refractory to aggressive drug therapy and diet control. In this study, we report a case with HoFH, who presented with xanthomata over the 4 limbs when she was 3 years old. When she was 11 years old, she began treatment with semi-selective LA with double filtration plasmapheresis (DFPP) once per week because HoFH was refractory to high-dose statin and diet control. LDL-C was reduced from 8.2 ± 0.9 to 2.69 ± 0.75 mmol/l (reduction rate = 67.3 ± 6.1%). The xanthomata over the 4 limbs were nearly completely resolved after 2 years of DFPP. Two years later, after the initiation of DFPP, we performed coronary angiography and echocardiography for regular checkup in the absence of chest pain, and the result was negative. To date (11 years after initiation of DFPP), she has not complained of any chest pain, shown intolerance to exercise, or exhibited ST-T change on electrocardiography. At the age of 20, multidetector computed tomography showed no significant stenosis over the coronary arteries. At the most recent follow-up visit, she was found to have good heart function and no xanthomata. LA is effective in the treatment of HoFH when drug therapy and diet control fail. With this treatment, pre-existing xanthomata can regress and coronary artery disease can be prevented.
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Hiperlipoproteinemia Tipo II/terapia , Plasmaféresis/métodos , Xantomatosis/terapia , LDL-Colesterol/sangre , Angiografía Coronaria , Enfermedad de la Arteria Coronaria/prevención & control , Femenino , Homocigoto , Humanos , Inhibidores de Hidroximetilglutaril-CoA Reductasas/uso terapéutico , Hiperlipoproteinemia Tipo II/sangre , Hiperlipoproteinemia Tipo II/complicaciones , Hiperlipoproteinemia Tipo II/diagnóstico por imagen , Rosuvastatina Cálcica/uso terapéutico , Factores de Tiempo , Resultado del Tratamiento , Xantomatosis/sangre , Xantomatosis/complicaciones , Xantomatosis/diagnóstico por imagen , Adulto JovenAsunto(s)
Blefaroplastia , Cáusticos/administración & dosificación , Enfermedades de los Párpados/terapia , Ácido Tricloroacético/administración & dosificación , Xantomatosis/terapia , Administración Tópica , Adulto , Anciano , Terapia Combinada/métodos , Párpados/efectos de los fármacos , Párpados/cirugía , Femenino , Humanos , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Resultado del TratamientoRESUMEN
INTRODUCTION: Xanthogranulomatous cholecystitis (XGC) is often mistaken for, and may predispose to, gallbladder carcinoma (GB Ca). This study reviews the worldwide variation of the incidence, investigations, management and outcome of patients with XGC. METHODS: Data from 29 studies, cumulatively containing 1599 patients, were reviewed and results summarized by geographical region (Europe, India, Far East and Americas) with 95% confidence intervals (CIs) to present variability within regions. The main study outcomes were incidence, association with GB Ca and treatment of patients with XGC. RESULTS: Overall, the incidence of XGC was 1.3-1.9%, with the exception of India where it was 8.8%. The incidence of GB Ca associated with XGC was lowest in European studies (3.3%) varying from 5.1-5.9% in the remaining regions. Confusion with or undiagnosed GB Ca led to 10.2% of patients receiving over or under treatment. CONCLUSIONS: XGC is a global disease and is associated with GB Ca. Characteristic pathological, radiological and clinical features are shared with GB Ca and contribute to considerable treatment inaccuracy. Tissue sampling by pre-operative endoscopic ultrasound or intra-operative frozen section is required to accurately diagnose gallbladder pathology and should be performed before any extensive resection is performed.
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Colecistitis/epidemiología , Neoplasias de la Vesícula Biliar/epidemiología , Granuloma/epidemiología , Xantomatosis/epidemiología , Américas/epidemiología , Asia/epidemiología , Colecistitis/diagnóstico , Colecistitis/terapia , Errores Diagnósticos , Endosonografía , Europa (Continente)/epidemiología , Femenino , Neoplasias de la Vesícula Biliar/diagnóstico , Neoplasias de la Vesícula Biliar/terapia , Granuloma/diagnóstico , Granuloma/terapia , Humanos , Incidencia , Masculino , Persona de Mediana Edad , Valor Predictivo de las Pruebas , Pronóstico , Estudios Retrospectivos , Tomografía Computarizada por Rayos X , Xantomatosis/diagnóstico , Xantomatosis/terapiaRESUMEN
Xanthelasma palpebrarum (XP) is the predominant form of cutaneous xanthoma, as it accounts for greater than 95% of cases. It is characterized by the presence of foam cell clusters containing a large amount of low-density lipoprotein (LDL), which are located in the connective tissue of skin, tendons, and fascia. XP lesions commonly present as distinctive yellow-orange macules, papules, or nodules, and are primarily on the upper eyelids as well as the inner canthus. Women are affected twice as often as men, with lesions typically emerging between the ages of 35 and 55. The pathophysiology of XP involves abnormal lipid metabolism and is often associated with hyperlipidemic states like Type II and IV hyperlipidemia, hypothyroidism, weight gain, and fatty diet. Despite the availability of various treatment methods, current XP management lacks standardization, particularly due to limited comparative research. To address this gap, we conducted an extensive literature review of 45 studies published between 2012 to 2023, which provides an updated overview of current XP treatment modalities. This comprehensive analysis will inform researchers and clinicians on the evolving landscape of XP management.
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Enfermedades de los Párpados , Xantomatosis , Humanos , Xantomatosis/terapia , Xantomatosis/diagnóstico , Enfermedades de los Párpados/terapia , Enfermedades de los Párpados/diagnóstico , Enfermedades de los Párpados/metabolismo , Femenino , Párpados/patología , Masculino , AdultoRESUMEN
Benign tumors of the eye and eyelid are common in children and adults, and they rarely undergo malignant transformation. Their workup and management have evolved over the years with increasing advancements in surgical and laser therapies. This contribution focuses on describing the following benign eye and eyelid tumors and their diagnostic and treatment approaches: congenital and acquired melanocytic nevi; nevus of Ota (Hori nevus); conjunctival papilloma; seborrheic keratosis; epidermoid cyst; dermoid cyst; milium; xanthelasma; hemangioma (cherry angioma and pyogenic granuloma); neurofibroma; neurilemmoma (schwannoma); and fibroepithelial polyp. Surgical removal is the primary treatment approach for many of these benign tumors. With advancements in laser technologies, there are now several laser types that can be used in the treatment of these benign eye and eyelid tumors. Other treatment modalities include cryosurgery, electrosurgery, and topical or intralesional medications. We hope this review will provide a reference to dermatologists and ophthalmologists in their approach to evaluation and management of benign eye and eyelid tumors.
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Neoplasias de los Párpados , Humanos , Neoplasias de los Párpados/terapia , Neoplasias del Ojo/terapia , Neoplasias del Ojo/diagnóstico , Quiste Dermoide/terapia , Neurofibroma/terapia , Neurofibroma/cirugía , Hemangioma/terapia , Quiste Epidérmico/terapia , Quiste Epidérmico/diagnóstico , Quiste Epidérmico/cirugía , Nevo Pigmentado/terapia , Queratosis Seborreica/terapia , Queratosis Seborreica/diagnóstico , Neurilemoma/terapia , Terapia por Láser , Xantomatosis/terapiaRESUMEN
Xanthomas are a common manifestation of lipid metabolism disorders. They include hyperlipemic xanthoma, normolipemic xanthoma, and a related condition, necrobiotic xanthogranuloma (NXG). All 3 forms can be associated with monoclonal immunoglobulin (MIg). In an attempt to improve diagnosis, understanding, and treatment of this association, we retrospectively analyzed a personal series of 24 patients (2 hyperlipemic xanthoma, 11 normolipemic xanthoma, and 11 NXG) and 230 well-documented reports from the literature. With the exception of the nodules and plaques featured in NXG, the clinical presentation of xanthomatous lesions usually resembled that seen in common hyperlipidemic forms and could not be used to suspect MIg-associated xanthomas. Extracutaneous sites were not rare. The MIg was an IgG in 80% of cases. Myeloma was diagnosed in 35%. Hypocomplementemia with low C4 fraction was present in 80% of studied patients. Low C1 inhibitor serum levels were found in 53%. Cryoglobulinemia was detected in 27%. These abnormalities suggest immune complex formation because of interactions between the MIg and lipoproteins and argue in favor of a causal link between MIg and xanthomas. Monoclonal gammopathy therapy could thus be an option. Indeed, among the patients who received chemotherapy, hematologic remission was accompanied by improvement in xanthoma lesions in several cases.
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Paraproteinemias/complicaciones , Paraproteinemias/terapia , Xantomatosis/complicaciones , Xantomatosis/terapia , Humanos , Paraproteinemias/diagnóstico , Paraproteinemias/patología , Estudios Retrospectivos , Piel/patología , Xantomatosis/diagnóstico , Xantomatosis/patologíaRESUMEN
OBJECTIVE: To explore the diagnosis and treatment of xanthogranulomatous prostatitis. METHODS: A 75-year-old man presented with a 3-month history of difficult urination and frequent micturition, which was exacerbated for 2 days. Digital rectal examination indicated an enlarged prostate size of II degrees with hard texture but no tenderness. Serum total PSA was 172.5 microg/L. TRUS revealed 200 ml of post-micturition residual urine, thickened bladder wall, prostate size of 4.3 cm x 3.8 cm x 5.0 cm and no isochrones. MRI showed an enlarged prostate gland, with marked enlargement of the central zones and low-signal intensity of the peripheral gland, part of the prostate gland protruding to the bladder with no clear dividing line. It was diagnosed as prostate cancer initially, and confirmed by needle biopsy. RESULTS: Histopathological examination revealed large numbers of "foamy macrophages" in the lesion, with a few multinucleated giant cells, leukocytes, mononuclear, plasmocytes and fibroplasia. Immunohistochemistry showed CD68 (+) and PSA (-). The patient was treated with oral Tamsulosin and glucocorticoid and by temporary catheterization, and followed up for 20 months. Urination symptoms began to alleviate and serum PSA to decrease at 4 months. The PSA level was 9.2 microg/L at 13 months and 3.6 microg/L at 17 months. CONCLUSION: Xanthogranulomatous prostatitis is a rare clinically, which can be confirmed by histopathological examination. It is treated mainly by supportive therapy and, for the cases with severe lower urinary tract obstruction, TURP can be employed. Follow-up must be performed by possible examination of PSA and necessary needle biopsy of the prostate.
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Prostatitis , Xantomatosis , Anciano , Humanos , Masculino , Prostatitis/diagnóstico , Prostatitis/patología , Prostatitis/terapia , Xantomatosis/diagnóstico , Xantomatosis/patología , Xantomatosis/terapiaRESUMEN
Atypical fibroxanthoma (AFX) is a rare neoplastic disease of the skin. Since the term was coined in the early 1960s, the disease has been viewed in many ways. For a long time AFX was regarded as a superficial variant of malignant fibrous histiocytoma (MFH). When the concept of MFH was re-evaluated and the term "undifferentiated pleomorphic sarcoma" (UPS) introduced, the controversy about the nature of AFX increased. The following review aims at providing an understanding of the present status of diagnosis and therapy of AFX based on the historical context and current data.
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Histiocitoma Fibroso Benigno/diagnóstico , Histiocitoma Fibroso Benigno/terapia , Neoplasias Cutáneas/diagnóstico , Neoplasias Cutáneas/terapia , Xantomatosis/diagnóstico , Xantomatosis/terapia , Diagnóstico Diferencial , HumanosRESUMEN
Familial hypercholesterolemia (FH) is the most common monogenic disorder. Due to the marked elevation of cardiovascular risk, the early detection, diagnosis, and proper management of this disorder are critical. Herein, the 2022 Korean guidance on this disease is presented. Clinical features include severely elevated low-density lipoprotein cholesterol (LDL-C) levels, tendon xanthomas, and premature coronary artery disease. Clinical diagnostic criteria include clinical findings, family history, or pathogenic mutations in the LDLR, APOB, or PCSK9. Proper suspicion of individuals with typical characteristics is essential for screening. Cascade screening is known to be the most efficient diagnostic approach. Early initiation of lipid-lowering therapy and the control of other risk factors are important. The first-line pharmacological treatment is statins, followed by ezetimibe, and PCSK9 inhibitors as required. The ideal treatment targets are 50% reduction and < 70 or < 55 mg/dL (in the presence of vascular disease) of LDL-C, although less strict targets are frequently used. Homozygous FH is characterized by untreated LDL-C > 500 mg/dL, xanthoma since childhood, and family history. In children, the diagnosis is made with criteria, including items largely similar to those of adults. In women, lipid-lowering agents need to be discontinued before conception.
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Hiperlipoproteinemia Tipo II , Xantomatosis , Adulto , Niño , LDL-Colesterol , Ezetimiba/uso terapéutico , Femenino , Humanos , Hiperlipoproteinemia Tipo II/diagnóstico , Hiperlipoproteinemia Tipo II/tratamiento farmacológico , Hiperlipoproteinemia Tipo II/genética , Proproteína Convertasa 9/genética , Xantomatosis/diagnóstico , Xantomatosis/etiología , Xantomatosis/terapiaRESUMEN
Familial hypercholesterolemia is an autosomal codominant disorder associated with markedly elevated plasma concentration of LDL-cholesterol and increased cardiovascular risk. Homozygous patients have rapid development of atherosclerosis with death from cardiovascular disease even in childhood. Life-long recurrent apheresis to reduce plasma LDL-cholesterol is considered the gold standard for treatment. Liver transplantation can be curative for this condition, but is usually only considered after the development of cardiovascular disease. We report a 5.5-yr-old child initially misdiagnosed with heterozygous familial hypercholesterolemia and treated by low-fat diet only. In view of persistent hypercholesterolemia and development of xanthomatosis, new molecular studies indicated the presence of two different mutations in the LDL receptor gene, with one being a deletion of two exons not identifiable with standard sequencing analysis. Recurrent plasma apheresis in combination with statins lowered, but did not normalize plasma LDL-cholesterol levels. It caused progressive reduction of the size of xanthomas and prevented the development of vascular complications. After two yr, liver transplantation normalized LDL-cholesterol levels and completely resolved the skin lesions. Preemptive liver transplantation is a definitive cure of familial homozygous hypercholesterolemia and might be more effective if performed before development of vascular complications.
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Hiperlipoproteinemia Tipo II/genética , Hiperlipoproteinemia Tipo II/cirugía , Trasplante de Hígado/métodos , Xantomatosis/genética , Biopsia con Aguja , Eliminación de Componentes Sanguíneos/métodos , Preescolar , LDL-Colesterol/análisis , Diagnóstico Diferencial , Femenino , Estudios de Seguimiento , Homocigoto , Humanos , Hiperlipoproteinemia Tipo II/patología , Inmunohistoquímica , Medición de Riesgo , Índice de Severidad de la Enfermedad , Factores de Tiempo , Resultado del Tratamiento , Xantomatosis/diagnóstico , Xantomatosis/terapiaRESUMEN
BACKGROUND: Atypical fibroxanthoma (AFX) is an uncommon superficial fibrohistiocytic tumor. It was originally described as a low-grade dermal tumor of atypical spindle cells, but this view has changed, and it is generally considered to be a tumor of intermediate malignant potential. OBJECTIVE: To review the current literature on AFX pertaining to epidemiology, pathogenesis and etiology, clinical presentation, histology, immunohistochemistry, prognosis and follow-up, and treatment. MATERIALS AND METHODS: Extensive literature review was conducted using OVID Medline and Pubmed searching for articles relating to AFX. RESULTS: AFX typically presents as a red or pink papule or nodule on the head or neck of an elderly man. The pathogenesis is most commonly related to ultraviolet radiation. It can clinically mimic other cutaneous malignancies and histologically can mimic squamous cell carcinoma, desmoplastic melanoma, and undifferentiated pleomorphic sarcoma. Immunohistochemistry is important in making the distinction. The prognosis is generally excellent, although there are rare cases of metastatic disease. There is a higher cure rate with Mohs' micrographic surgery than with wide local excision. CONCLUSIONS People with AFX generally have had significant ultraviolet radiation exposure. They should be examined at least every 6 months for recurrence, metastasis, and the development of additional skin cancers.
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Granuloma/diagnóstico , Granuloma/terapia , Enfermedades de la Piel/diagnóstico , Enfermedades de la Piel/terapia , Xantomatosis/diagnóstico , Xantomatosis/terapia , Granuloma/etiología , Humanos , Enfermedades de la Piel/etiología , Xantomatosis/etiologíaRESUMEN
BACKGROUND: Atypical fibroxanthoma (AFX) is a rare cutaneous malignancy of older adults. Little is known about the behavior of AFX in the setting of chronic lymphocytic leukemia (CLL) and non-Hodgkin lymphoma (NHL). OBJECTIVE: To further understand the development, characteristics, and behavior of AFX in the setting of concomitant CLL and other types of NHL. METHODS AND MATERIALS: Study approval was obtained from the Mayo Clinic Institutional Review Board. The master diagnosis index was queried from January 1, 1980, through December 31, 2008, to identify patients with AFX and CLL or other types of NHL. A retrospective chart review was conducted. RESULTS: Ten patients were identified with AFX and NHL. These patients did not show a greater risk of recurrence, metastasis, or death than that found in previous case reports. Of these 10 patients, four had AFX and CLL. The outcomes of these patients were no different from those of patients with AFX and other types of lymphoma. CONCLUSIONS: AFX did not demonstrate aggressive features such as recurrence or metastasis in patients with concomitant CLL or other NHL. More studies are needed to definitively characterize the behavior of AFX in this patient population.
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Histiocitoma Fibroso Maligno/patología , Leucemia Linfocítica Crónica de Células B/patología , Linfoma no Hodgkin/patología , Neoplasias Cutáneas/patología , Xantomatosis/patología , Anciano , Anciano de 80 o más Años , Causas de Muerte , Femenino , Histiocitoma Fibroso Maligno/terapia , Humanos , Leucemia Linfocítica Crónica de Células B/terapia , Linfoma no Hodgkin/terapia , Masculino , Estudios Retrospectivos , Factores de Riesgo , Neoplasias Cutáneas/terapia , Xantomatosis/terapiaRESUMEN
UNLABELLED: Monoclonal gammopathy may manifest itself through a range of skin disorders, including plane normolipemic xanthoma and necrobiotic xanthogranuloma. The present paper describes two patients with these cutaneous symptoms. The first has extensive areas of skin affected by flat xanthomas, monoclonal gammopathy with > 10% infiltration of bone marrow with clonal plasmocytes and, according to PET-CT, unclear lymphadenopathy in the retroperitoneal area. The size of this lymphadenopathy (histologically no malignant infiltration and no confirmed infectious aetiology) has not changed significantly over a 4-year follow-up. Repeated PET-CT scans showed decrease in SUV value in this infiltration from 7.5 to 3.8. Four cycles of treatment with a combination of bortezomib, cyclophosphamide and dexamethasone brought neither reduction in monoclonal immunoglobulin nor change to skin morphology. We believe that the abdominal lymphadenopathy is associated with xanthomatosis but have been unable to confirm this unequivocally. The second patient is being followed up for more than 10 years, originally for MGUS, later for asymptomatic multiple myeloma. Last year, painful subcutaneous and cutaneous infiltrates, isolated on an upper limb and more frequent on lower limb, started to occur. These infiltrates are palpable. PET-CT imaging provided an excellent depiction of these infiltrates, showing no pathology on the head, chest and abdomen and no osteolytic foci on the skeleton. CT imaging showed clearly numerous infiltrates in the skin and subcutaneous tissue of lower limbs, particularly both shanks, reaching up to 2 cm in depth. The largest infiltrate, measuring 3.5 by 2 by 10 cm, was identified in the distal dorsal part of the right shank. PET imaging of lower limbs showed distinctly pathological accumulation in all infiltrates described above; the accumulation of glucose in the lower part of the right shank reached 10.0 SUV. CT images of lower limbs showed increased density saturated hypodermis even in the areas where there is no increased accumulation of 18 fluoroglucose. Following 40 Gy irradiation, the size of infiltrate in the radiated area decreased and their soreness ceased. CONCLUSION: PET-CT imaging offered information on extra-cutaneous signs of plane normolipemic xanthomas and provided excellent depiction of the areas of the skin and hypodermis affected by necrobiotic xanthogranuloma. Chemotherapy with cyclophosphamide, bortezomib and dexamethasone brought no reduction in monoclonal immunoglobulin concentration, and no reduction in plane normolipemic xanthomas. Radiotherapy targeted at large foci of xanthogranulomas led to partial regression and ceased infiltrate soreness.
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Gammopatía Monoclonal de Relevancia Indeterminada/complicaciones , Xantogranuloma Necrobiótico/complicaciones , Xantomatosis/complicaciones , Humanos , Masculino , Persona de Mediana Edad , Gammopatía Monoclonal de Relevancia Indeterminada/diagnóstico , Gammopatía Monoclonal de Relevancia Indeterminada/terapia , Xantogranuloma Necrobiótico/diagnóstico , Xantogranuloma Necrobiótico/inmunología , Xantogranuloma Necrobiótico/terapia , Tomografía de Emisión de Positrones , Tomografía Computarizada por Rayos X , Xantomatosis/diagnóstico , Xantomatosis/inmunología , Xantomatosis/patología , Xantomatosis/terapiaRESUMEN
INTRODUCTION: Xanthelasma palpebrarum (XP) is a common xanthomatous lesion of the eyelid and periorbital skin. Several methods of treatment have been reported in the literature, each having its own indications and risks. We present a new treatment method for removing XP using a plasma exeresis device (Plexr®, GMV, Italy). METHODS: Fifteen patients with a total of 27 treated XPs were assessed and clinically identified by the treating dermatologist. Patients were photographed and assessed by a dermatologist prior to and immediately after treatment. A patient survey was conducted 12 months after the procedure, which assessed the outcome of the procedure (redness, pigmentation disorders, and scars). RESULTS: After just a single treatment session using a plasma sublimation, all 27 XPs showed complete clearance. There were no reports of scars, pigmentary alteration, or recurrence of lesions up to 12 months. CONCLUSIONS: We present plasma sublimation as a new method for the treatment of XP. During the procedure, the method allows to control the depth of tissue destruction and the presence of xanthoma tissue, and to minimize pain and trauma, making it particularly ideal for treating areas around the eye.