Madelung disease: distribution of cervical fat and preoperative findings at sonography, MR, and CT.
[3] Two rare distinct familial types of lipomatosis have been identified, which are FML and multiple symmetric lipomatosis (MSL) also known as
Madelung disease. Clinically, the most prominent difference between these two types of lipomatosis is that FML is marked by discrete lipomas that predominate on the extremities and generally absent from neck and shoulders, whereas MSL is a nonencapsulated, diffuse lipomatosis infiltration of underlying tissue (i.e., often is most prominent in neck and shoulder regions).
[Literature review of
Madelung disease: 1 case report].
The adipose deposits, sparing the distal extremities in this patient, were consistent with so-called multiple symmetric lipomatosis (MSL), also known as
Madelung disease.
Yang, "Madelung disease," Canadian Medical Association Journal, vol.
Goldszmidt, "Case 143: Madelung disease," Radiology, vol.
The patient was diagnosed as having Madelung disease based on the physical examination findings.
Madelung disease (benign symmetric lipomatosis) is a rare disease of uncertain etiology, mainly reported in Mediterranean and eastern European populations.
However, Madelung disease may be associated with serious medical problems: difficulties in limb movements, difficulties in turning of the head, difficulties in deglutition, hoarseness, dyspnea, or sleep apnea caused by airway compression.
Patients with Madelung disease have soft masses in the parotid area ("hamster cheeks"), cervical region ("horse collar"), and back ("hump back").
(9) Lipomas usually occur sporadically, but some are associated with several inherited disorders, including multiple lipomatosis, Gardner syndrome, and
Madelung disease. (3) Recurrences, which have been reported in about 5% of patients, are more common when the lipoma is associated with generalized lipomatosis, when the mass is not completely removed at surgery, or when the tumor is a well-differentiated liposarcoma incorrectly diagnosed as a benign lipoma.
