Doença de Madelung

Abstract

Resumo / SummaryDoença de Madelung ou lipomatose simétrica múltipla é uma doença rara, que é caracterizada por acúmulo de gordura não encapsulada, geralmente localizada simetricamente ao redor do pescoço e ombros. A etiologia é desconhecida, mas é freqüentemente observada em pacientes com história de alcoolismo crônico e é acompanhada por anormalidades metabólicas. Os autores apresentam um caso de lipomatose cervical associada a polineuropatia e alcoolismo. A remoção cirúrgica do tecido adiposo por lipectomia ou lipoaspiração é terapia paliativa, já que ele não é encapsulado, a recorrência é comum.

Multiple symmetrical lipomatosis (Madelung’s disease)✰✰Please cite this article as: Ikumi-Shibasaki I, Ikuo-Shibasaki H, Nakamoto TS, Baccan FS, Raposo LS. Multiple symmetrical lipomatosis (Madelung's disease). Braz J Otorhinolaryngol. 2014;80:90–1.

“…Some studies have described a clinical treatment with β2-agonist, in order to increase the adrenergic lipolysis and reduce the accumulation of fat, but with questionable efficacy. 2 Although weight loss and alcohol withdrawal appear to have no effect on disease progression, these measures associated with surgery can decrease the recurrence rate, 6 as a longitudinal study showed that the amount of alcohol consumed is directly associated with the onset of the disease. 3…”

Doença de Madelung: relato de caso e revisão da literatura

“…In type II, lipomatosis is diffuse, with clinical appearance of simple obesity, and without the symptoms of deep involvement present in type I (6) . Typically, the masses do not respect cleavage planes, penetrating all levels of the tissue, so their surgical exeresis is more difficult (4) .…”

“…Such types include multiple symmetric lipomatosis, congenital infiltrative lipomatosis of the face, encephalocraniocutaneous lipomatosis, scapular waist lipomatosis, adiposis dolorosa, pelvic lipomatosis and mediastinal lipomatosis (5) . The clinical diagnosis is based on the recognition of the unique appearance of the patient, but in atypical cases, there may be confusion with lymphoproliferative disorders and other conditions (4) .…”