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Forty years old patient with benign symmetric lipomatosis Launois-Bensaude was treated by liposuction of shoulders, arm, thighs and buttock made in stages. About 1200 ml of tissue were removed on average with no need of blood loss compensation. Good functional and cosmetic effect was achieved. The average duration of full remission was 10 months, then the relapse have been slowly recoming.
Activity of the antioxidation enzymatic system, involving evaluation of glutathione-reductase, -peroxidase and -S-transferase as well as glucose-6-phosphate dehydrogenase activities, was studied in blood plasma, leukocytes, lymphocytes and erythrocytes of patients with benign symmetric lipomatosis. Elevation in the rate of lipid peroxidation induced the antioxidation enzymatic system and caused a imbalance of its individual units. Intensive antioxidation therapy using "Aevit" allowed correction of the imbalance observed in the enzymatic activity.
We present a case of multiple symmetric lipomatosis Type I (Madelung's disease) with severe organic affection, hepatic cirrhosis, sever sensitive polyneuropathy and neuropathic ulceration at the left lower limb. A nephrotic syndrome developed in a larval form due to proliferative glomerulonephritis as the result of a metainfectious complications of the infection at the lower limb. We discuss the etiopathogenicity of the organic affection and we highlight the pathogenic links between the disease and its complications.
A 50 year old female was found to have a lump on the right side of abdomen with discharging wound on the same side and was ultrasonographically diagnosed as renal cell carcinoma. On histopathology, it showed replacement of the kidney by mature adipose tissue renal replacement lipomatosis, along with pyelitis glandularis which is a rare condition.
On the occasion of two separate observations of multiple lipomatosis on a 48 years old female patient and a neurofibromatosis on a 49 years old male patient, the differential diagnosis of these diseases was compiled with regard to the cutaneous and subcutaneous symptoms and to the relations to the nervous system and the inner organs. The differential diagnostical plan requires a series of clinical examinations for the clarification of these pathological conditions, which often comprise more than one disease or one syndrome.
Multiple Symmetric Lipomatosis (MSL) is a syndrome characterized by the occurrence of symmetric lipomas over various regions of the body. No clear etiology has been recognized while a frequent association with systemic metabolic abnormalities has been described. The metabolic situation of a subject affected by MSL was assessed before and after surgical excision of lipomas. A condition of impaired glucose tolerance (IGT) was verified both before and after surgery by the performance of oral glucose tolerance test, glucagon test, and daily glucose profile. No significant differences were observed after the ablation of lipomatous masses with regard to glucose, IRI, IRCP and NEFA behaviour. We concluded that the resection of lipomas can not modify glucose tolerance in MSL and that lipomas can be considered as tissues metabolically independent from the rest of body fat.
Multiple Symmetric Lipomatosis (MSL or Madelung's Disease) is a rare entity characterized by the presence of non-capsulated lipomas mainly located at the neck and upper part of the thorax and frequently associated to chronic hepatopathy, neuropathy and alcoholic habit. We review and update the clinical, etiopathogenic, diagnostic and therapeutical aspects of this rare entity. We stress the presence of neuropathy, a typical feature of the disease, which has also a predictive value.
Multiple symmetric lipomatosis (MSL) (or Madelung's disease or Launois-Bensaude syndrome) is a rare inherited disease clinically characterized by a massive development of large symmetric unencapsulated lipomas on the subcutaneous tissue of face, neck, trunk and arms, resulting in a grotesque aspect of the patient. Less frequently the accumulation of excessive fatty tissue can spread deeply to the superficial fascia. Peripheral neuropathy, macrocytic anemia and chronic hepatopathy have been reported to cohesist. Macrocytic anaemia and chronic hepatopathy are probably secondary to high alcohol consumption, that is frequently associated. MSL, that was first described by Sir Benjamin Brodie in 1846, affects mainly the men (ratio man/woman 30:1), with an incidence in Mediterranean area of 1:25.000 men; the ages at onset range from 20 to 50 years. It is not known yet the pathway of inheritance and the molecular basis of the genetic defect responsible for the development of fat accumulation. It has been postulated a defective lipolytic response to catecholamines; this altered response could be due to an abnormal amount or a defective function of Gs-protein, the coupler between beta-adrenergic receptors on the surface of adipocytes and adenylate cyclase, or, alternatively, the defect could be in the catalytic unit of adenylate cyclase. The number and function of alpha- and beta-adrenergic receptors and the lipolytic response to cAMP (the second messenger) are normal. Recently it has been hypothesized that the defective lipolysis is due to a disorder in the mitochondria of brown fat, whose distribution is similar to the peculiar position of the lipomas in this pathology; the brown fat, unlike white adipose tissue, has abundant mitochondria. The alcohol abuse, frequently present in these subjects, might facilitate the clinical expression of the molecular defect. The therapy of lipomas is essentially surgical, but this approach is not easy, because the lipomas are not capsulated and extremely vascularized. Moreover the surgical excision is not always a successful treatment for the lipomas as they frequently recurrent after a short period from the exeresis. In this report we describe a 59-years old white man, alcohol abuser, with a typical clinical picture of MSL, developed when he was 37 years old. The patient presented multiple lipomas around the shoulders, face, neck and arms, that had been surgically excised eight times. Magnetic resonance imaging showed the presence of fat deposits also in the mediastinum, that caused a tracheal compression. Hepatic cirrhosis and serious side effects from peripheral neuropathy, represented by Charcot's joint and neuropathic ulcer on the sole foot were observed.(ABSTRACT TRUNCATED AT 400 WORDS)
Encephalocraniocutaneous lipomatosis is a distinct neurocutaneous syndrome characterized by an extensive fatty tissue nevus of the scalp, protuberances of the cranial bones, lipodermoid of the conjunctiva, multiple intercranial lipomas, and porencephaly. A further case of this multisystem birth defect is reported. The patchy arrangement of lesions that is usually unilateral suggests a mosaic phenotype. The clinical criteria to distinguish this disorder from other mosaic neurocutaneous phenotypes, such as Schimmelpenning syndrome, Proteus syndrome, or Delleman syndrome, are outlined. To explain the origin of this nonhereditary genodermatosis, the concept of a lethal autosomal mutation that survives in a mosaic state is proposed.
We present a case of multiple symmetric lipomatosis. We comment aspects diagnostics and therapeutic.
Lipomatosis of the ileocecal valve is a diffuse infiltration of the submucous coat with fat cells. This syndrome is discussed on the basis of a personal observation and a review of the literature. The characteristic endoscopic picture is the overgrown, yellowish lips of Bauhin's valve prolapsing into the cecum, reminiscent of a uterine cervix. Coloscopy with biopsy is therefore the method of choice in assuring proper diagnosis. On the other hand, radiology cannot recognize the disease with certainty. Generally treatment of this benign syndrome is only necessary in the event of disturbances. As a surgical procedure ileocecal resection is suggested.
A case of diffuse mediastinal lipomatosis (ML) is described, associated to exogenous obesity, infrequent entity, which is comprehended within the causes which can cause mediastinal broadening. The clinical characteristics of our observation and the general aspects of interest are reviewed for this type of mediastinal affectation.
Two cases of pseudotumoral lipomatosis in the renal sinus and responsible for clinical symptoms and radiological renal alterations are reported. Emphasis is put on the misleading features of this disease which may suggest a malignancy. Outcome is consistently favorable. Computed tomography ensures the preoperative diagnosis, avoiding an unnecessary nephrectomy.
Epidural lipomatosis (EL) is characterized by abnormal accumulation of unencapsulated fat in the epidural space, and usually occurs as a complication of longterm steroid therapy. This condition, which may result in devastating neurologic complications, has also been reported without exogenous steroid intake. We describe a case of nonsteroid induced symptomatic EL associated with obesity, and emphasize the possibility of effective medical management of this entity with weight reduction instead of decompressive laminectomy whenever neurologic symptoms are mild and stable.
Successful surgical management of an unusual case of diffuse lipomatosis is reported. The clinical and radiological pictures are described and the essential points to be taken into consideration in differential diagnosis are illustrated.
We report a patient who presented with intestinal obstruction; his small intestine showed intense segmentary lipomatosis associated with unusually situated multiple intussusceptions. Preoperatively, we diagnosed intussusceptions in the ileocecal region by ultrasonography and computed tomographic scan. During surgery, the ileum was dilated and contained numerous movable polypoid masses. One reducible intussusception was encountered in the ileocecal region. In addition, an ileoileal intussusception that could not be reduced was resected with an end-to-end anastomosis. At histological examination, more than 150 submucosal lipomas were found.
We present the case of a 42-year-old man who complained of low back pain radiating into the posterior aspect of both thighs. There were no neurological deficits, while general medical examination revealed a Cushing's syndrome due to corticosteroid therapy for rheumatic polyarthritis. Myelography showed irregular and circumferential narrowing of the dural sac from L-3 to S-1 caused by abundant epidural fat as demonstrated by MRI. Surgical decompression produced immediate relief of symptoms. The literature of epidural spinal lipomatosis is reviewed and therapeutic considerations are discussed.
We describe the case of a 38-year-old male with a benign symmetrical lipomatosis Launois-Bensaude. He has suffered from severe obesity since the age of seven. At the age of 30, hypogonadism was diagnosed and treated. He developed a Pickwick-syndrome, with hypokapnia and secondary polycytemia. Ulcerations developed on the lower parts of his legs, which were treated conservatively for many years at our dermatological clinic. Because of intertriginous dermatitis in the right groin, he treated himself with external corticosteroids containing dequalonium. Ulceration and fatty necrosis followed, necessitating extensive plastic surgical intervention, with removal of approximately 30 kg of skin and adipose tissue.