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

doi:10.1016/S0190-9622(87)70253-9

Journal of the American Academy of Dermatology

Volume 17, Issue 4, October 1987, Pages 663-674

https://doi.org/10.1016/S0190-9622(87)70253-9 Get rights and content

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.

References (51)

ML Greene et al.
Benign symmetric lipomatosis (Launois-Bensaude adenolipomatosis) with gout and hyperlipoproteinemia
Am J Med
(1970)
ME Kodish et al.
Benign symmetric lipomatosis: functional sympathetic denervation of adipose tissue and possible hypertrophy of brown fat
Metabolism
(1974)
FT Kurzweg et al.
Lipomatose multiple familiale
Am J Surg
(1951)
PE Launois et al.
De l'adéno-lipomatose symétrique
Bull Soc Med Hôp Paris
(1898)
OW Madelung
Über den Fetthals (diffuses Lipom des Halses)
Langenbecks Arch Klin Chir
(1888)
WP Herrmann et al.
Lipomatosis symmetrica benigna mit Hyperlipoproteinämie Typ IV und Epipharynx-Karzinom
Z Hautkr
(1978)
V Sudár
Zur benignen symmetrischen Lipomatosis (Adenolipomatosis Launois-Bensaude; Madelung'scher Fetthals)
Schweiz Med Wochenschr
(1976)
LM Taylor et al.
Benign symmetric lipomatosis
Mayo Clin Proc
(1961)
DE Comings et al.
Benign symmetric lipomatosis
JAMA
(1968)
DA Strange et al.
Benign symmetric lipomatosis
JAMA
(1968)

M Grivaux et al.

Adénolipomatose symétrique de Launois et Bensaude et troubles du métabolisme lipidique

Sem hôp Paris

(1966)

W Häuptli et al.

Die benigne symmetrische Lipomatose: ein Symptom der äthylischen Hepatopathie?

Schweiz Med Wochenschr

(1979)

K Chakrokhi

La lipomatose symétrique diffuse à prédominance cervicale

Rev laryng (Bordeaux)

(1960)

G Clement-Coujard et al.

A propos de trois cas de maladie de Launois-Bensaude

Sem hôp Paris

(1953)

M Deparis et al.

Cirrhose alcoolique et maladie de Launois-Bensaude. Etude historique, clinique et pathogénique

Rev Int Hépat

(1968)

M Kahn

Aspect endocrinien de la lipomatose symétrique à prédominance cervicale de Launois-Bensaude

HA Springer et al.

Launois-Bensaude adenolipomatosis

Plast Reconstr Surg

(1972)

FW Storck

Über die “metastasierende und rezidivie-rende” Lipomatosis indolens

Zentralbl Chir

(1953)

C Garnier et al.

Lipomatosis Launois-Bensaude bei Morbus Recklinghausen

Akt Dermatol

(1982)

G Enzi et al.

Mulitple symmetric lipomatosis. A defect in adrenergic-stimulated lipolysis

J Clin Invest

(1977)

A Gate et al.

Le syndrome de Launois-Bensaude. A propos de 12 cas cliniques

Ann Chir Plast Esthet

(1966)

P Mounier-Kuhn et al.

Localisation laryngée d'une maladie de Launois-Bensaude

J Franc Otorhinolaryng

(1967)

T Barale et al.

Maladie de Launois-Bensaude féminine

Bull Soc Fr Dermatol Syph

(1972)

PE Launois et al.

L'adenolipomatose symétrique à prédominance cervicale chez la femme

Clin méd

(1901)

JP Lyon

Adiposis and lipomatosis

Arch Intern Med

(1910)

Cited by (147)

Diagnostic Pathology: Pediatric Neoplasms
2018, Diagnostic Pathology: Pediatric Neoplasms
Parapharyngeal space lipomatosis with secondary dyspnea, disphagia and disphonia
2015, International Journal of Surgery Case Reports
Citation Excerpt :
In this case, the differential diagnosis in this case included two entities: Madelung–Launois disease (benign symmetric lipomatosis) [6], and lipoma. Benign symmetric lipomatosis is an entity of unknown etiology, Kodish [7] stated that it was the result of hypertrophy of brown adipose tissue; it was first described in 1846 by Benjamin Brodie [8], and subsequently by Otto Madelung in 1888 and a decade later Launois and Besaunde [8] presented two case studies of 35 and 30 patients respectively; it predominantly affected males with reports emerging that refer to a ratio of 15:1, with a range of age of onset between 20 and 60 years; a pattern for family history has not yet been discovered and it is considered to be a sporadically occurring entity; there are some reports of family history, with dominant autosomal patterns of inheritance [6]. Lipomas constitute a very rare tumor in the parapharengeal space, as previously only 11 cases had been reported in the literature, presenting features which may include symptoms of pressure, with signs such as obstructive dysphagia and airway obstruction, sleep apnea manifested as dyspnea,similar to the present case [3–5,9,10].
Parapharyngeal space tumors are a small percentage of all head and neck neoplasms. Due to their anatomic location, they represent a therapeutic challenge. To our knowledge, 11 cases of parapharyngeal lipomatous tumors have been reported in the literature.
A 48 year old male with chief complaints of dyspnea, dysphagia and dysphonia was found to have a parapharyngeal space tumor. He was scheduled to undergo lumpectomy and neck exploration.
Benign tumors represented 70% of all cases. Open neck surgery is considered the gold-standard of treatment.
It is important to bear in mind the lipomas of the parapharyngeal space to establish an accurate diagnosis and implement timely, appropriate treatment in order to avoid future complications and reduce morbidity and mortality.
Distal acquired benign symmetric lipomatosis: A rare subtype of an uncommon entity
2013, Journal of the American Academy of Dermatology
Madelung's disease associated with polyneuropathy and symptomatic hypokalemia
2013, Journal of the Formosan Medical Association
Citation Excerpt :
Besides, image of fatty accumulations shows that the fatty bulk in MSL is noncapsulated and distributed along vascular and muscular planes, in contrast with lipoma, which is capsulated in subcutaneous space. Most patients are associated with alcoholism, but the casual effect between alcohol and MSL is unknown.1,2,4,5 Alcoholism-related macrocytic anemia and elevated liver enzyme are noted.
Madelung's disease (multiple symmetric lipomatosis) is a rare disease characterized by abnormal diffuse lipomatosis in proximal upper limbs and neck. Previous reports have shown that this disease is associated with alcoholism, polyneuropathy, mitochondrial disease, and glucose intolerance. Here, we describe a 46-year-old man having Madelung's disease associated with polyneuropathy and symptomatic hypokalemia. He presented with insidious-onset weakness and numbness in lower limbs for 7 years and recent deterioration of symptoms. Proximal weakness improved with potassium supplement. Our observation may extend the phenotype of Madelung's disease to hypokalemic periodic paralysis.
Madelung's disease: A case report
2012, Italian Journal of Medicine
Madelung's disease, also known as Multiple Symmetric Lipomatosis (MSL) or the Launois-Bensaude syndrome, is characterized by subcutaneous lipid deposits, which, unlike lipomas, are nonencapsulated. The disease is diagnosed mainly in Mediterranean countries, and 90% of all cases involve male alcoholics. These masses are usually found on the neck, chest, abdomen, and arms. The symptoms are the result of acute sensorimotor axonal neuropathy caused by compression. Treatment (surgical and/or medical) does not produce significant or lasting results.
We describe the case of a 57-year old male alcoholic (daily ethanol intake: approximately 2 L of wine), who has been treated and followed by our staff for 6 years.
As this case illustrates, management of MSL is often associated with serious difficulties related to the patient's alcoholism. The effects of adipose tissue masses on the sensitive and motor nervous system are complicated by the effects of alcoholic neuropathy, requiring complex long-term monitoring. Treatment is often compromised by poor compliance related to patient's drinking problems. Madelung's disease is currently regarded as a rare disease, although we suspect that the incidence in Italy (and in the rest of the world) has probably been underestimated for various reasons.
Multiple symmetrical lipomatosis: Case report and literature review
2012, Journal of Laryngology and Otology

View all citing articles on Scopus

View full text

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

Am J Med

Metabolism

Am J Surg

De l'adéno-lipomatose symétrique

Bull Soc Med Hôp Paris

Über den Fetthals (diffuses Lipom des Halses)

Langenbecks Arch Klin Chir

Lipomatosis symmetrica benigna mit Hyperlipoproteinämie Typ IV und Epipharynx-Karzinom

Z Hautkr

Zur benignen symmetrischen Lipomatosis (Adenolipomatosis Launois-Bensaude; Madelung'scher Fetthals)

Schweiz Med Wochenschr