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At least 19 recordsLinked to original sources
PubMed1999

[Symmetrical benign lipomatosis of the tongue and Launois-Bensaude lipomatosis].

BACKGROUND: There are many causes of macroglossia, including fatty infiltration. Unlike unique or multiple lipomas of the tongue, non-encapsulated adipocyte masses develop in the tongue in symmetrical benign lipomatosis. Only six cases have been reported in the literature. CASE REPORT: A 69-year-old man with a history of chronic alcoholism developed a soft tumefied formation on the lateral margins of the tongue. A yellow-colored content was perceived through the thin mucosa. The formation was bilateral and had developed for more than 10 years. The patient also presented Launois-Bensaude lipomatosis localized on the neck, the nuchal region and the shoulders. DISCUSSION: This is, to our knowledge, the first reported case of symmetrical benign lipomatosis associated with Launois-Bensaude lipomatosis. An analogy between these two conditions has been suggested, but the six earlier cases of lipomatosis of the tongue reported in the literature developed in patients without cutaneous lipomatosis.

Aged↗
PubMed1980

[Diffuse polypoid lipomatosis coli with hypertrophy of the appendices epiploicae in congenital lipomatosis (author's transl)].

In a 62-year-old patient diffuse polypoid lipomatosis of the colon and hypertrophy of the appendices epiploicae was found together with subcutaneous lipomata and bone changes. Out of the five cases of diffuse lipomatosis coli published so far certain parallels can be drawn in only one case. The clinical picture and course indicate that it is either a pathogenetically uniform form of mesenchymal dysplasia or a syndrome.

Colon↗
PubMed2000

[Epidural lipomatosis].

UNLABELLED: ONSET: Epidural lipomatosis is a rare disorder defined as a pathological overgrowth of normal epidural fat. It is more often associated with administration of exogenous steroid with variable duration and doses. Furthermore, it may occur in some patients in the absence of exposure to steroids but generally associated with obesity. Whatever the predisposing factor, the majority of these patients are men. The causal effect of epidural lipomatosis in the development of spinal cord or radicular compression is generally well accepted. DIAGNOSIS: The diagnosis of epidural lipomatosis can be established by melography, computed tomography (CT) and magnetic resonance imaging (MRI). MRI is considered the imaging procedure of choice, allowing an assessment of the extent of lipomatosis and, as well as CT, an identification of the lipomatous tissue. Most cases of epidural lipomatosis with corticosteroid use occur in the thoracic region, while most idiopathic cases occur in the lumbar region. TREATMENT: Management of treatment depends on the severity of the neurological signs and the patient's background. The most common treatment for epidural lipomatosis with corticosteroid use consists in surgical decompression but with a high risk of postoperative mortality. In some cases however, medical treatment includes corticosteroid withdrawal or reduction and calorie restriction, leading to clinical improvement. Treatment for idiopathic epidural lipomatosis is more often medical, based on weight loss and physical therapy with generally successful outcome. The pathogenesis of epidural lipomatosis remains unknown but different suggested hypotheses may lead to a metabolic disorder as the underlying cause.

Adolescent↗
PubMedDate not supplied

[Lumbar epidural lipomatosis].

Spinal epidural lipomatosis is a pathological accommodation of fat tissue in the spinal canal. It seems to be a disease entity, which, though rare, has recently been diagnosed more frequently and can be accompanied by neurological deficits. The thoracic spinal canal is the preferred localization. Eighteen cases of symptomatic lumbar epidural lipomatosis have been described in the literature. We are reporting on our experience with another 8 patients. Three of these patients presented with the typical signs of spinal nerve irritation. In these cases epidural lipomatosis was associated with a small disk herniation without direct contact to the spinal nerve. Another 5 patients showed the clinical picture of a spinal claudication. In all 5 patients, there was a concentric compression of the thecal sac by epidural fat. In one patient, the cause of the lipomatosis was assumed to be long-term steroid therapy following kidney transplantation. Four patients suffered from extreme obesity. No cause for lipomatosis could be found in 3 patients. A microdiskektomy was performed in the 3 patients with the associated disk herniation; the remaining patients were treated conservatively. In 6/8 patients (3x surgery/3x diet), an "excellent" or "good" clinical result could be achieved after 1 year. Two patients had a "satisfactory" result. Lumbar epidural lipomatosis can be treated conservatively in cases with only mild neurological dysfunctions and known cause (e.g. obesity, steroid therapy). The surgical removal of associated disk herniation proved to be sufficient in cases described in this paper.

Adult↗
PubMed2006

Facial infiltrative lipomatosis.

BACKGROUND: Although there are multiple case reports and small series concerning facial infiltrative lipomatosis, there is no composite radiological description of the condition. OBJECTIVE: Radiological evaluation of facial infiltrative lipomatosis using plain film, sonography, CT and MRI. MATERIALS AND METHODS: We radiologically evaluated four patients with facial infiltrative lipomatosis. Initial plain radiographs of the face were acquired in all patients. Three children had an initial sonographic examination to evaluate the condition, followed by MRI. One child had a CT and then MRI. RESULTS: One child had abnormalities on plain radiographs. Sonographically, the lesions were seen as ill-defined heterogeneously hypoechoic areas with indistinct margins. On CT images, the lesions did not have a homogeneous fat density but showed some relatively more dense areas in deeper parts of the lesions. MRI provided better delineation of the exact extent of the process and characterization of facial infiltrative lipomatosis. CONCLUSION: Facial infiltrative lipomatosis should be considered as a differential diagnosis of vascular or lymphatic malformation when a child presents with unilateral facial swelling. MRI is the most useful single imaging modality to evaluate the condition, as it provides the best delineation of the exact extent of the process.

Adipose Tissue↗
PubMed2004

Benign symmetric lipomatosis (Madelung's disease): case reports and current management.

Benign symmetrical lipomatosis (BSL) is a rare disorder characterized by the presence of multiple, symmetric, nonencapsulated fat masses in the face, neck, and other areas. Typically, this entity has been related to the presence of three anterior bulges in the neck. The disorder was first described by Brodie in 1846. After that, Madelung in 1888 and Launois and Bensaude in 1898 characterized the disease. There are multiple synonyms for this disorder, such as Madelung's disease, Launois-Bensaude syndrome, and multiple symmetrical lipomatosis. Benign symmetric lipomatosis is usually described in adults from 30 to 60 years old, with an incidence of about 1 in 25,000 and a male-to-female ratio of 15:1 to 30:1. Most cases have no hereditary pattern. More than 90% of the patients have associated alcoholism. The etiology of benign symmetric lipomatosis remains unknown, but an abnormal lipogenesis induced by catecholamines has been observed. The transformation of BSL to a malignant tumor is extremely rare. In the current report, the authors describe two cases of benign symmetric lipomatosis treated in their department and a review of the literature.

Alcoholism↗
PubMed2003

Idiopathic symptomatic epidural lipomatosis of the lumbar spine.

BACKGROUND: Symptomatic spinal epidural lipomatosis (SEL) of the lumbar spine is a rare disease, often associated with steroid overload. Idiopathic lipomatosis is even much less frequent. Signs and symptoms depend upon the level and degree of nerve root compression. Diagnosis is best based on MRI. Weight reduction can be curative, however after failure of medical treatment or in severe cases surgical decompression should be performed. METHOD: Four patients with severe symptoms of lumbar spinal epidural lipomatosis were treated by surgical decompression. Patient history and neurological examination are described, diagnostic imaging is demonstrated, surgical treatment and outcome are documented. Different surgical techniques including laminectomy, interlaminar fenestration and lateral recess decompression were applied and are discussed. FINDINGS: All four patients improved after surgery. No surgical complications were observed. Even though limited to four cases this is the second largest series of operated idiopathic spinal epidural lipomatosis. INTERPRETATION: Surgical decompression was effective in improving symptoms in severe lumbar idiopathic spinal epidural lipomatosis.

Aged↗
PubMed2006

[Precocious mediastinal lipomatosis: a rare complication of systemic corticosteroid therapy].

BACKGROUND: Corticosteroid-induced lipomatosis results from hypertrophy within adipose tissue; the condition is frequently asymptomatic and its incidence is underestimated. We report a case of mediastinal lipomatosis that is rare in terms of both site and presenting symptoms. CASE REPORT: A 46-year-old woman with no disease history other than obesity with a weight of 90 kg had been treated since 2002 for mixed connective tissue disease (profound lupus and dermatomyositis). She had been treated with oral corti costeroids (1 mg/kg/d). Two months after the start of treatment, she presented chest pains, resting dyspnea particularly aggravated in dorsal decubitus, chest edema in the subclavicular space and jugular turgescence. Chest x-ray revealed widening of all levels of the mediastinum. The chest CT scan showed lipomatosis throughout the entire mediastinum with no associated chest abnormalities or pericardial effusion. Rapid downward dosage adjustment ofcorticosteroids to 10 mg/d coupled with synthetic antimalarials resulted in gradual reduction of symptoms. The chest scan performed two months later short stabilization of the patient's mediastinal lipomatosis. DISCUSSION: The effects of long-term of glucocorticosteroid therapy are well-known, in particular Cushing's syndrome. Lipomatosis has been described more recently and affects different axial regions. Mediastinal localization is seen in 15% of patients treated. This presentation is less common than orbital and epidural localizations. Although often asymptomatic, as in our own report, it may present with worrying symptoms that pose real diagnostic problems. The diagnostic examinations of choice are CT scan or MRI. Regression following discontinuation or reduction of corticosteroids is inconsistent and often gradual.

Adrenal Cortex Hormones↗
PubMed2005

Epidural spinal lipomatosis with acute onset of paraplegia in an HIV-positive patient treated with corticosteroids and protease inhibitor: case report.

STUDY DESIGN: Case report. OBJECTIVE: To report a case of HIV-related lipodystrophy with a rapid onset of symptoms from epidural lipomatosis in the wake of protease inhibitor and steroid treatment. SUMMARY OF BACKGROUND DATA: Symptomatic spinal epidural lipomatosis is considered to be a rare condition usually presenting with slowly progressive cord or nerve root compression. Only 2 cases of spinal lipomatosis in HIV-related lipodystrophy have been reported. METHODS: We describe the case of a 41-year-old male with HIV who received protease inhibitor medication and had neurologic deficits rapidly develop. RESULTS: The patient had complete paraplegia develop within 12 hours from admission following a 1-day history of unsteady gait and a 3-day history of leg numbness. After diagnosis of epidural lipomatosis on magnetic resonance imaging, the patient underwent decompressive thoraco-laminectomy. He recovered well and was able to walk by postoperative day 4. CONCLUSION: It is important to maintain an awareness for the possible association between HIV lipodystrophy and symptomatic epidural lipomatosis.

Acute Disease↗
PubMed2005

[Multiple symmetric lipomatosis in the otolaryngology as diagnostic and therapeutic problem].

Multiple symmetric lipomatosis (MSL) is a systemic disease connected with a degeneration of the adipose tissue. Association of reduced glucose tolerance, hyperinsulinemia, hyperlipoproteinemia, hyperuricemia, macrocytic anemia and renal tubular acidosis, polyneuropathy have been described. Lipomatosis was initially described in 1846 by Brodie but the exact aetiopathogenesis is still unknown. Depending on the anatomical location of the lipomatosis we can divided MSL into two types. MATERIAL, METHODS AND THERAPY: We describe the findings in three patients with symmetric lipomatosis: two (n=2) with the first and one (n=1) with the second type of the disease. The patients were diagnosed and treated in the Department of the Otolaryngology during last of two years (2002 to 2004). Patients had executing following research: ultrasonography of the neck and abdominal cavity (n=2), radiological examination of the chest (n=3), computer tomography of the neck (n=1), thin-needle biopsy (n=3), histopathological examination (n=2) and laboratory investigations (n=3). Two patients received the pharmacological treatment (magnesium and the vitamin B6) and we observed marked regress of the disease. In the patient who at first did not agree for the treatment, came up to the heavy increase mass of tumors, especially on the neck. Liposuction and the pharmacological treatment were executed in this patient. He is still in the observation. RESULTS: In case of tumors of face and neck, we have to take into account degenerative processes of the adipose tissue. Our observations indicate the efficiency of the magnesium and the vitamin B6 therapy in patients with multiple symmetric lipomatosis. The obtainment in this range of reliable conclusions require of research at the greater number of patients. We want to underline that there are any research of the influence of the magnesium and the vitamin B6 on the course of this disease in the literature.

Adipose Tissue↗
PubMed1996

Epidural lipomatosis not induced by corticosteroid therapy. Three cases including one in a patient with primary Cushing's disease (review of the literature).

We report three cases of epidural lipomatosis including one in a patient with primary Cushing's disease. Our literature review found 16 additional cases of symptomatic epidural lipomatosis in patients who were not receiving corticosteroids. The presenting symptoms were nonspecific. The main clinical symptoms were nerve root pain, weakness of the lower limbs upon exertion, paraparesis or isolated back pain. Degenerative lesions were common and were sometimes the cause of the symptoms. Cases were evenly distributed between the thoracic and lumbar spine. Of the 18 patients, 14 were men and eight were older than 54 years. Three-fourths of patients were obese. Spinal cord or nerve root compression occurred in some instances. Modern imaging techniques (computed tomography and magnetic resonance imaging) can establish the diagnosis rapidly. In patients without neurologic compromise, surgery should be considered only if symptoms fail to respond to weight reduction. The rate of occurrence of epidural lipomatosis in patients with Cushing's disease is probably underestimated. Routine investigation by magnetic resonance imaging of Cushing's disease patients who have manifestations known to occur in epidural lipomatosis would allow to evaluate the role of increased production of endogenous corticosteroids in the occurrence of epidural lipomatosis.

Adrenal Cortex Hormones↗
PubMed1996

[Replacement lipomatosis of renal tissue: a peculiar reaction to inflammation].

Replacement lipomatosis is an abnormal fatty proliferation of the renal sinus, hilus, perirenal and, sometimes, periureteral spaces. Our experience with 18 cases of replacement lipomatosis is reported: 6 patients had the massive form, one of them bilateral, 5 patients had associated xanthogranulomatous pyelonephritis and 7 had an initial focal form. After a thorough review of the literature of the last 30 years, we analyzed the etiopathogenetic bases of this abnormal chronic reaction. The cause of this abnormal fatty proliferation is thought to be related to peculiar individual reactivity; the association of predisposing factors (chronic obstruction and stones) with multiple infections (especially by Coli) does not seem to cause the abnormality. This hypothesis is confirmed by the frequent association of replacement lipomatosis and xanthogranulomatous pyelonephritis, where abnormal individual reactivity has been postulated. Replacement lipomatosis may develop even when the classic predisposing factors are not present or, in contrast may not develop even when they are present. The peculiarity of this pseudotumoral form, where the expansile trend of fatty tissue is apparent, is to mimic neoplastic mesenchymal disease. So far, few cases have been reported in the literature-i.e., less than 20 massive cases in the last 30 years. In this study, the clinical presentation and pathologic findings are correlated with imaging data, especially of CT; the diagnostic yield of this method is stressed, in one with the differential diagnosis with the other, neoplastic or not, forms with negative densitometry. The similarities and differences between replacement lipomatosis and xanthogranulomatous pyelonephritis, which are distinct conditions, are emphasized, so that a careful study of imaging findings may help make the correct diagnosis.

Humans↗