Madelung's Disease Research Articles

Benign symmetric lipomatosis of the tongue: Report of a case

Benign symmetric lipomatosis (BSL) is a lesion characterized by symmetrical and diffuse growth of adipose tissue. It is commonly found in the posterior neck or upper trunk, and oral involvement is extremely rare. Although two cases’** of lingual lipomatosis have been described, they were not diagnosed as BSL. The case presented here was diagnosed as BSL based on the gross features and histologic findings.

Madelung's Syndrome: multiple symmetrical lipomatosis, alcoholism, pseudoliposarcoma and progressive neuropathy

Abstract: Three cases of multiple symmetrical lipomatosis (Madelung's disease) are described occurring in three male alcoholics presenting to a Gastroenterology Unit. The patients all had the characteristic development and distribution of multiple lipomata and the later occurrence of a severe progressive peripheral neuropathy. Two patients had alcohol related liver disease.

Ｂｅｎｉｇｎ ｓｙｍｍｅｔｒｉｃａｌ ｌｉｐｏｍａｔｏｓｉｓにより巨舌症を呈した１症例

Benign symmetrical lipomatosis (BSL) that is a subgroup of benign lipomatosis disease is rare in the oral region. We recently experienced a case of macroglossia caused by BSL. The patient, a 71 year old man, came to our department with complaints of swallowing, eating and speech disorders by macroglossia. This case was diagnosied as symmetrical macroglossia. Glossectomy was performed under general anesthesia in order to improve these function disorders.It was diagnosised histologically because of its diffuse invasion of lipoid tissue.

Benign symmetric lipomatosis Launois-Bensaude: Report of ten cases and review of the literature

Benign symmetric lipomatosis Launois-Bensaude is a disease rarely reported in the American literature and not mentioned in the standard English textbooks of dermatology. It seems, however, to be relatively common in Europe. Between 1981 and 1985 we examined twelve patients, and a number of case reports have been published in French and German literature. We describe ten typical cases and review the literature. The disease is characterized by massive symmetric fat deposits predominantly in the neck and shoulder girdle area. Pathogenetically, the increase in fatty tissue is assumed to result from a localized defect in catecholamine-induced lipolysis. The disease is frequently associated with alcoholism, hepatopathy, glucose intolerance, hyperuricemia, and malignant tumors of the upper airways, requiring thorough clinical evaluation of all patients. Dietary treatment and weight loss are of limited value in the management of benign symmetric lipomatosis. Surgical removal of lipomatous tissue is frequently followed by recurrence and should be restricted to decompression in patients with functional impairment.

Lipomatosis of the neck (Madelung's neck).

Benign symmetrical lipomatosis of the neck is a rare disease that has to be differentiated from goiter, sialadenitis, obesity or a lymphatic tumor. Most patients are severe alcoholics, but they may have other endocrine disorders, such as diabetes mellitus, hyperuricemia, or hyperlipidemia. Aside from the cosmetic disfigurement and consequent psychological stress, respiratory distress may be the indication for surgical treatment. Excision of the lipomatosis requires technical skill because the extensive and sometimes infiltrative growth makes dissection of muscle and nerves difficult. The computer tomogram provides good information on the extent of the disease. Three of our 5 patients died 2 1/2 to 6 years after the first operation because of their primary disease.

舌に発症したＢｅｎｉｇｎ ｓｙｍｍｅｔｒｉｃ ｌｉｐｏｍａｔｏｓｉｓの１例

Benign symmetric lipomatosis (BSL) is a rare disease characterized by symmetric, diffuse proliferation of adipose tissue almost in the neck and upper trunk. Four BSL cases had been reported in Japan, all of which had been found in the posterior neck. This report presents an atypical case of BSL manifested in the tongue.A lipoma-like tumor was observed in the bilateral borders of the tongue. The laboratory data were almost normal. He had suffered from apoplexy and alcoholism. A lingual biopsy was performed, and a diagnosis of BSL was made in terms of both the clinical and histological findings.The discussion includes the clinical findings of BSL and differential diagnosis between lipomatosis and lipoma. Especially the differences of macro-and microscopic findings between lipomatosis and lipoma are emphasized.

Malignant degeneration in Madelung's disease (benign lipomatosis of the neck): case report

Although not a common condition, benign lipomatosis of the neck (Madelung's disease) is well documented in the literature and to date we have failed to trace any reference to malignant change developing in this tissue. We wish therefore to report the case of a patient with benign lipomatosis of the neck in which malignant degeneration appeared after an interval of 6 years and in which this transformation from a benign to a malignant lesion was verified by histological examination.

Benign Symmetric Lipomatosis

Madelung's disease, or benign symmetric lipomatosis (BSL), is a disease that is rarely reported in the English language dermatology literature; I was unable to find an American dermatologic journal reference in the past decade. The disease represents the symmetrical deposition of adipose tissue over the head, neck, upper part of the trunk, and proximal part of the upper extremities, while the lower part of the trunk and the legs are typically unaffected. The patients are characteristically not otherwise obese. Since the patient described herein came to a dermatology clinic for assistance, perhaps dermatologists should be reminded of this entity. <h3>Report of a Case</h3> A 62-year-old man came to the Geisinger Medical Center Department of Dermatology with a 13-year history of multiple masses on the head, neck, and upper part of the body. There had been a slow and progressive enlargement of most of the lesions, while a few seemed to

BENIGN SYMMETRICAL LIPOMATOSIS

We present a patient who had benign symmetrical lipomatosis. He was treated surgically (6 operations), and the results were satisfactory. we discuss various factors in this disease and suggest the possibility of a nonsurgical treatment being developed in the future.

Madelung's disease of the wrist and dyschondrosteosis.

Twenty-six index patients with primary Madelung's deformity of the wrist were examined, as were sixty-five of their relatives. Standard radiographs were taken and measured to determine whether mesomelic dwarfism, a feature of dyschondrosteosis, was present. No case of mesomelia was found. No evidence of Madelung's deformity was found in the relatives of the index patients, who were all female. It is concluded that primary Madelung's deformity of the wrist is not an expressions of dyschondrosteosis, that it is not inherited, and that it does not occur in the male. One index patient with dyschondrosteosis was examined. Eight of her relatives were similarly affected, and three of these were male. It is suggested that previously recorded male cases of Madelung's deformity of the wrist were expressions of dyschondrosteosis or of secondary deformity.

The Madelung deformity

Over a period of fifteen years, seventeen patients with Madelung's deformity underwent treatment in a hand surgery department. They were classified into three separate clinical forms: Traumatic Madelung's deformity; Madelung's disease proper; and Dyschondrosteosis of Leri-Weill.

Benign symmetric lipomatosis: Functional sympathetic denervation of adipose tissue and possible hypertrophy of brown fat

Levels of lipolytic hormones (growth hormone, cortisol, and glucagon), insulin, and free fatty acids were measured in a patient with benign symmetric lipomatosis during a 5-hr glucose tolerance test, an insulin tolerance test, a 24-hr fast, and an epinephrine infusion. Although there was no abnormality in insulin dynamics or impairment in lipolytic hormone secretion, there was a failure of free fatty acid concentrations to rise to expected levels during fasting and after glucose. Insulin hypoglycemia and epinephrine infusion resulted in normal and excessive free fatty acid responses, respectively. This pattern of free fatty acid release is consistent with functional sympathetic denervation of adipose tissue. The lipomatous fat in benign symmetric lipomatosis is similar in distribution to the brown fat of human newborns and rodents that may hypertrophy after denervation. It is suggested that the lipomas in this disorder may represent such brown adipose tissue.

Lipid and purine metabolism in benign symmetric lipomatosis.

The benign symmetric lipomatosis (Adenolipomatosis of Launois-Bensaude (1), or Madelung’s disease (2)) is characterized by multiple, symmetric deposites of subcutaneous fat on the neck, the nape, the back and upper part of the trunk. The etiology of this disease is not known: in almost every published case, however, excessive alcohol consumption is common (3). In some cases this disease in accompanied by an additional diabetes (4), by hyperlipoproteinemia of type IV with elevated triglycerides and by hyperuricemia (5).

Laryngeal involvement in benign symmetric lipomatosis.

Infiltration of the false cords with lipomatous tissue in a patient with benign symmetric lipomatosis was treated by laryngofissure and resection of lipomatous tissue with excellent results. The French literature reports a similar case.

Advanced laryngeal compression due to diffuse, symmetric lipomatosis (Madelung's disease).

Massive fat deposition in the neck is an exceedingly rare cause of laryngeal compression but one with a specific constellation of radiographic findings. We have recently seen such a case, which we believe to be of general interest. The differential diagnosis of symmetrical narrowing of the laynx or laryngotracheal airway in adults is limited. When there are no associated intraluminal abnormalities, the process is generally one which weakens the supporting cartilagenous structure, such as traumatic fracture or ischaemia from prolonged use of a cuffed endotracheal tube (Jameset al., 1970), infiltration by primary or metastatic neoplasm (Janower et al., 1970) or inflammation of the cartilage itself (as with relapsing polychondritis) (Horn and O'Loughlin, 1962). In Madelung's disease the larynx and trachea are compressed by prodigious, local adipose proliferation. The patient was first seen at the Massachusetts General Hospital at age 53 in 1967 because of painless, progressive swelling of the neck of six months' duration. There had been some recent exertional fatigue, a sensation of pressure in the ears, and a feeling of “tightness” upon turning the neck. There were no complaints of dysphonia, dysphagia, or dyspnoea. His past history was one of heavy alcohol consumption, but good physical health. He was a heavy smoker throughout his adult life and had had minimal nonpurulent morning sputum production for several years. Some 15 years prior to admission, he had received three “X-ray treatments” to the neck because of “neurodermatitis”; no details of this procedure could be obtained.

Effect of resection of adipose tissue on the diabetes and hyperinsulinism of benign symmetric lipomatosis.

Resection of 1,720 gm. of adipose tissue did not alter a chemical diabetic glucose tolerance and a late excessive plasma insulin rise in a patient with benign symmetric lipomatosis. This suggests that other factors than a feedback from adipose to the beta cell are responsible for the hyper-insulinemia of obesity.

Madelung's deformity: observations in 17 patients.

IN 1878, MADELUNG (8) described a painful abnormality of the wrist and forearm which characteristically began in adolescence. The radiographic alterations of the radius and ulna were described later. The most complete review of the subject is that of Anton et al. (1), who collected and summarized the literature through 1938. Considering this clinical and radiographic abnormality a deformity rather than a syndrome, these workers distinguished it from Madelung's disease, which is characterized by diffuse symmetrical lipomatosis of the upper part of the back, shoulders, and neck. They also suggested that the deformity of the wrist and forearm be considered a dyschondroplasia of the distal radial epiphysis. While this may be a logical name for the entity, it has not found wide usage, Madelung's deformity remaining the preferred term. In 1929, Léri and Weill (7) reported a case of dwarfism and radiographic alterations of the forearm and wrist similar to Madelung's deformity, although no reference was made to Madelung's original description. Believing this represented a previously undescribed generalized osseous dysplasia, they gave it the name “dyschon-drostéose.” There have since been other occasional reports of this condition. Herdman, Langer, and Good (4) and Langer (6) arrived at the following conclusion regarding the relationship of Madelung's deformity to the Léri-Weill syndrome: “Madelung's deformity of the forearm is the most striking feature of dyschondrosteosis, and it is our feeling that all cases with this deformity, except when it is secondary to trauma or infection, should be included under the heading of dyschondrosteosis” (4). Maroteaux and Lamy (10) concurred with this opinion. We have studied the radiographs of 17 patients with abnormalities of the wrist and forearm which were characteristic of Madelung's deformity according to the criteria set forth by Dannenberg et al. (2). In 2 patients the evolution of Madelung's deformity was observed radiographically. In some cases variable shortening of the ulna and humerus was noted. Six patients (CASES 1–6, TABLE II) are felt to represent examples of dyschondrosteosis. Radiographic Observations The radiographic criteria for the diagnosis of Madelung's deformity as proposed by Dannenberg et al. (2) have been utilized in the analysis of our patients. These criteria are presented below, regrouped according to the bones primarily affected: A. Alterations Which Primarily Involve the Radius 1. Double Curvature (Ulnar and Dorsal) (Figs. 1 and 2): Ulnar curvature was present in all but one patient and varied from 8 to 30°. Volar curvature was much less constant and involved only the distal diaphysis and metaphysis. 2. Decreased Length (Fig. 1): The radius was 0.5 to 6.0 ern shorter than normal for the stated age in each instance.3

Benign Symmetric Lipomatosis

To the Editor:— A rapidly growing supraclavicular mass is a disconcerting sign to both the patient and the physician. It is disconcerting to the patient because he is sure it is some form of cancer. It is disconcerting to the physician because he is sure the patient is right. We report here a form of rapidly growing supraclavicular mass, which, though unpleasant in appearance is pleasant in prognosis, for in contrast to the carcinoma and lymphoma usually found in this area, it is benign. Its three most characteristic features are described in the title, benign symmetric lipomatosis. A sufficient number of cases have been reported that it may be considered a distinct entity. 1-5 The cervical area is most frequently affected, showing bilateral accumulation of soft masses which frequently extend in tongue-like projections between the cervical and upper thoracic muscles. As the lobulated masses progressively enlarge they may produce a

Dyschondrosteosis: The most common cause of Madelung's deformity

Dyschondrosteosis is a dominant hereditary bone dysplasia resulting in wrist deformity and mesomelic dwarfism. No previous cases of this condition have been described from North America. This report also includes the first documented case in a male. European authors have distinguished between dyschondrosteosis and Madelung's disease. Clinical and radiologic findings in 5 patients in 2 families presented here and a review of the literature strongly suggest that these two conditons are in fact identical. Dyschondrosteosis would appear to be the preferred designation since, historically, it has included all of the features of the condition.

DYSCHONDROSTEOSIS, A HEREDITABLE BONE DYSPLASIA WITH CHARACTERISTIC ROENTGENOGRAPHIC FEATURES.

Seven cases of dyschondrosteosis are reported. This is a hereditary bone dysplasia with an autosomal dominant mode of transmission characterized by a typical deformity of the distal radius and ulna and proximal carpal bones and mesomelic dwarfism. The wrist deformity has been widely reported in the medical literature under the eponym, Madelung's disease.