Multiple Symmetric Lipomatosis: New Classification System Based on the Largest German Patient Cohort

被引:21
作者
Schiltz, Daniel [1 ]
Anker, Alexandra [1 ]
Ortner, Christine [2 ]
Tschernitz, Sebastian [2 ]
Koller, Michael [3 ]
Klein, Silvan [1 ]
Felthaus, Oliver [1 ]
Schreml, Julia [4 ]
Schreml, Stephan [2 ]
Prantl, Lukas [1 ]
机构
[1] Univ Hosp Regensburg, Dept Plast Hand & Reconstruct Surg, Franz Josef Strauss Allee 11, D-93053 Regensburg, Germany
[2] Univ Hosp Regensburg, Dept Dermatol, Regensburg, Germany
[3] Univ Hosp Regensburg, Dept Clin Studies, Regensburg, Germany
[4] Univ Hosp Cologne, Inst Human Genet, Cologne, Germany
关键词
D O I
10.1097/GOX.0000000000001722
中图分类号
R61 [外科手术学];
学科分类号
摘要
Background: Multiple symmetric lipomatosis (MSL) is defined as a disorder of nonencapsulated adipose tissue growth. Its prevalence is indicated as 1: 25,000 and affects, as stated in the literature, mainly Mediterranean males (male: female ratio of 15: 1). Phenotypes are still classified as defined by Donhauser in 1991. We report clinical and phenotypic data of the largest patient cohort investigated in Germany so far. Methods: Forty-five patients diagnosed with MSL at the University Hospital Regensburg between 2007 and 2017 were photographed, clinically examined, and blood samples were taken. Based on the photographs (n = 33), 11 independent observers assessed patients using the Donhauser classification. Furthermore, the bodies of all patients were subdivided into 12 body areas, and the viewers had to indicate all MSL-affected areas per patient. Prevalence was calculated, comorbidities were assessed, and blood samples were analyzed. Results: According to the established Donhauser classification, less than 50% of the patients could be classified. Therefore, based on the constellations of MSL-affected body areas, a new classification that divides phenotypes of MSL into 5 types (Ia, Ib, Ic, II, and III) was set up and was able to cover 100% of our patients. The male to female ratio was found to be 1:2.5 (male: female). Prevalence of MSL in the catchment area was found to be 1:25,000. Hypercholesterinemia and hypothyroidism were frequent comorbidities, and blood analyses were normal besides a hypercholesterinemia. Discussion: The new proposed classification system describes 5 subtypes and allowed to classify all assessed patients. Male to female ratio (1:2.5) contradicted most previous publications.
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