Launois-bensaude syndrome: A benign symmetric lipomatosis without alcohol association.

Sir,

Launois-Bensaude syndrome (LBS) was first revealed by French physicians Launois (1856–1914) and Bensaude (1866–1939).[1] They described the disorder in 1898, after studying 65 cases. The syndrome is also known as multiple symmetrical lipomatosis, cephalothoracic lipodystrophy, and Madelung disease. The classical tetrad of symptoms includes lipoma, symmetric nature, diffused localization, and cervicofacial predominance. This metabolic disorder occurs mostly in men age between 35 and 50 years with known alcohol use. Familial cases have been reported with autosomal dominant transmission and penetrance variable.

The course of the disease is fairly stereotyped, especially after a relatively rapid progression over several years; the disease progression slows or stabilizes.[2] We report a single case of LBS with absence of alcohol use.

Case Report

The patient was 72-year-old male from North Africa/Mediterranean region belonging to Arabic ethnic group. The patient reported a history of smoking but without any alcohol use or alcoholism.

Twenty-four years before his admission, the patient underwent a lipoma resection from the back without local recurrence. Lipomatous lesions occurred in the breasts, abdomen, and roots of thighs, the volume size increased gradually and was up to 20 cm for the largest lesion localized in the abdomen, and the lesion was highly symmetrical with impact change on walk due to inguinal lesion [Figure 1a and b]. The patient's sister reported similar profile despite she was 40 years old; thus, she underwent liposuction.

Figure 1

(a) Diffuse lipomatosis in breasts. (b) Diffuse lipomatosis in abdomen and roots of thighs (a) (b)

Our patient was overweight with body mass index of 29.38 kg/m2, waist circumference of 106 cm, and high blood pressure at 160/100 mmHg; evidence of heart failure or neurological deficits was not shown. The blood assessment showed low-density lipoprotein cholesterol level at 1.24 g/L and high-density lipoprotein at 0.39 g/L while normal range is between 0.4 and 1 g/L; the liver functions as well as glycemia were assessed. Hence, the diagnosis of metabolic syndrome was retained according to International Diabetes Federation 2005 criteria.

Ultrasound and abdominal computed tomography revealed symmetrical lipoma disseminated and confluent in given localizations.

The patient was treated for hypertension and healthier lifestyle, and diet methods were suggested to improve the metabolic syndrome. The patient was subsequently referred to a plastic surgeon for liposuction of inguinal lipoma.

Discussion

LBS, a benign symmetric lipomatosis (BSL), is a rare origin of obesity, related to symmetric lipomas evolving in limb's roots, upper chest wall while sparing face, neck, and extremities.[3]

The classification of Enzi et al.[4] defines two types according to the location of the disease. Type 1 is characterized by deep lipomatous masses, atrophy of the subcutaneous fat, and lipomas located in the cervical area of the body with supraclavicular and deltoid spaces allowing pseudo-athlete appearance for the patient. Type 2 or Enzi et al. type is characterized by disseminated and subcutaneous lipomatosis affecting the trunk, abdomen, and the limbs root’s, with very close to obesity standards.

Several physiopathological explanations have been suggested as a deficit of the adrenergic stimulation of lipolysis.[5] The alcohol may be a cofactor which induces a change in the number and function of beta-adrenergic receptors. Genetic alterations with a likely autosomal dominant with variable penetrance on mitochondrial DNA were also found.[6]

The presence of a similar case in the sister can suggest the second hypothesis (but in our context, the genetic study has not been able to be performed).

Our patient demonstrated BSL of Type 2 lipomatosis according to the Enzi classification. In general, the complications of the disorder are related to underlying conditions and lipomatous proliferation. Thus, in Type 1, intramediastinal localization might originate a vena cava syndrome and tracheal compression.[7] It might present also a slight decrease in cervical amplitude, intertrigo folds, and clothing discomfort, in addition to metabolic complications and disorders related to chronic alcoholism. In our case, blood urea and cholesterol levels as well as liver tests were normal, and the patient did not have any history of alcoholism and was devoid of complications of alcohol abuse.

The treatment of LBS is mainly surgery resection and/or liposuction since being the most effective treatments.[8] This surgical approach should be considered after alcohol withdrawal which seems delaying the recurrence.[910]

Medical treatment includes correcting metabolic disorders and overweight. The surgical procedures are often time-consuming due to the volume of fat masses, dissemination of lipomas and their infiltration within surrounding tissue, since being non-encapsulated. Conventional surgery is especially indicated in highly vascularized and innervated regions; incision is indicated whenever necessary for tissue lifting. Otherwise, liposuction is preferred despite the risk of recurrence.[11]

In our patient, for the absence of alcohol, the patient was proposed directly for liposuction of the inguinal lipoma annoying walking.

Conclusion

LBS is difficult to treat, but the psychological and functional impacts highly support the care especially desperate patients.

Coherent medical and surgical management approach should be case by case based and allows treating patients in better conditions. All patients should benefit of specialized, personalized medical care before surgery; it is required to include alcohol withdrawal, corrections of metabolic disorders, and introduction of hygienic and dietary rules. Indeed, surgery is considered only after alcohol withdrawal. It is planned, taking into account the fractional complications bleeding and patient history.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.