An unusual presentation of Madelung's disease in a Hispanic 49-year-old male with pruritus

被引:0
|
作者
Johnsen, Nicole [1 ]
Nesbit, Shannon [1 ]
Zhang, Qiang [1 ]
Pillutla, Pranati [2 ]
Shiba, Travis [2 ]
机构
[1] Univ Calif Los Angeles, David Geffen Sch Med, 10833 Le Conte Ave, Los Angeles, CA 90095 USA
[2] Olive View UCLA Med Ctr, Dept Head & Neck Surg, Los Angeles, CA USA
关键词
SYMMETRIC LIPOMATOSIS;
D O I
10.1016/j.xocr.2024.100649
中图分类号
R76 [耳鼻咽喉科学];
学科分类号
100213 ;
摘要
A 49-year-old Hispanic male presented with a 3-month history of mildly pruritic, bilateral nodules on the suboccipital neck. Examination revealed bilateral sub- occipital nodules measuring 3 x 5 cm, alongside additional bilateral nodules in the preauricular and submandibular regions, as well as in the central submental and substernal areas, each approximately 2 x 3 cm. Laboratory tests indicated hypercholesterolemia and elevated transaminases, suggestive of alcoholic hepatitis. Fine- needle aspiration (FNA) cytology of the substernal and suboccipital masses revealed fibrous adipose tissue and spindle cell proliferation, and magnetic resonance imaging confirmed adipose-like density tumors. The diagnosis of Madelung's disease (also known as multiple symmetric lipomatosis, MSL) was established based on nodular distribution, laboratory findings, cytology, and imaging results. This rare disorder is characterized by non-encapsulated symmetric lipomas and is commonly associated with alcohol use disorder and metabolic abnormalities. This case is notable for its atypical presentation in a Hispanic male, as MSL typically affects Mediterranean or European populations, and the presence of pruritus, which has not been previously reported. Topical hydrocortisone was prescribed, resulting in pruritis improvement. The patient was counseled on alcohol cessation and offered surgical removal of the masses; however, he declined surgery. Providers should remain vigilant for MSL in patients with bilateral lipomas and a history of alcohol use disorder or metabolic abnormalities. A comprehensive workup should include FNA to exclude malignancy and imaging to assess for compression of underlying structures. Treatment should involve counseling on alcohol cessation, consideration of surgery, and topical hydrocortisone may be considered to manage pruritus.
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