Lipomatoses

Abstract

Lipomatoses are benign proliferations of adipose tissue, often with typical distribution patterns, which usually occur without clear causes. In contrast to circumscribed lipomas, they develop diffusely and symmetrically and are not surrounded by a fibrous pseudocapsule. The most common form is benign symmetric lipomatosis (BSL; Launois-Bensaude syndrome), of which four types are distinguished based on the distribution pattern of hyperplastic adipose tissue. The etiology and pathogenesis of the disease are still largely unknown, although some forms appear to have a hereditary basis or to be associated with increased alcohol consumption. In some cases, mitochondrial dysfunctions have been detected. Lipomatoses may be solitary, but may also be associated with other symptoms or illnesses (comorbidity). Effective drug therapies are not known, but surgical procedures can alleviate the symptoms. Lipomatosis dolorosa, which can be distinguished from BSL, occurs predominantly in middle-aged women and is characterized by proliferation of adipose tissue and severe pain in the affected areas. Various differential diagnoses and concomitant illnesses need to be considered, in particular depression. There are no evidence-based recommendations for therapy. Repetitive lidocaine infusions can achieve temporary improvement. Recurrence after surgical intervention is not uncommon, but long-term success is also possible.

Application of lateral tarsoconjunctival flap to correct ocular complications of Madelung's disease

“…In contrast to obesity, exercise or diet control does not affect the fatty tissue proliferation in lipomatosis. [ 2 ] Hence, surgical treatment such as lipectomies and/or liposuction is the mainstay therapy for BSL. [ 2 , 8 , 16 ] In a large retrospective study published in 2017 involving 59 patients who underwent surgical treatment for Madelung's disease, an average of 4 surgeries were performed for each patient during a 10-year period, in which 39% of these patients still had disease recurrence.…”

“…[ 2 ] Hence, surgical treatment such as lipectomies and/or liposuction is the mainstay therapy for BSL. [ 2 , 8 , 16 ] In a large retrospective study published in 2017 involving 59 patients who underwent surgical treatment for Madelung's disease, an average of 4 surgeries were performed for each patient during a 10-year period, in which 39% of these patients still had disease recurrence. [ 8 ] Overall, a total of 230 procedures were performed with 83.5% lipectomies, 13% liposuction alone, and 3.5% lipectomy combined with liposuction.…”

“…[ 1 ] The pathogenesis remains unclear, but it is closely related to genetic predisposition with triggering factors, such as metabolic diseases and alcohol consumption, and has been reported to be associated with alcoholic liver dysfunction. [ 2 ]…”

Rare Forms of Lipomatosis: Dercum’s Disease and Roch-Leri Mesosomatous Lipomatosis

“…This pain is usually chronic, debilitating, and resistant to conventional analgesics; there is increased exquisite tenderness with deep palpation of the lipomas. Mild irritation can trigger the pain (e.g., temperature changes, rubbing of clothes) [26]. It is located mainly on the trunk, the proximal extremities of the limbs, the buttocks, and the pelvis (Figure 1).…”

Metabolic and immunological phenotype of rare lipomatoses: Dercum’s disease and Roch-Leri mesosomatic lipomatosis

“…Lipodystrophy syndromes are rare diseases characterized by a limited capacity of subcutaneous adipose tissue to store triglycerides, which results in metabolic abnormalities such as insulin resistance, hypertriglyceridemia, fatty liver disease and polycystic ovary syndrome. Apart from these syndromes that are usually associated with partial or generalized lipoatrophy, lipomatosis is de ned by the presence of multiple lipomas on the body, without lipoatrophy (1,2,3). Different entities accompanied by multiple lipomas have been described, including:…”