Synonym(s)
Progressive partial lipodystrophy; Barra-quer-Simons syndrome; acquired partial lipodystrophy; cephalothoracic dystrophy; acquired progressive lipodystrophy
Definition
Disorder characterized by progressive and symmetric loss of subcutaneous fat
Pathogenesis
May be associated with glomerulonephritis, low C3 levels, and the presence of a C3 nephritic factor
Clinical manifestation
Onset between 5 and 15 years of age; slow, insidious loss of subcutaneous fat, initially limited to the face, sometimes extending to the upper portion of the body, giving the patient a cachetic appearance
Differential diagnosis
HIV-associated lipodystrophy; Cockayne syndrome; generalized lipodystrophies such as Berardinelli-Seip syndrome; Werner syndrome; hypothalamus tumor
Therapy
Subcutaneous fat injections from unaffected areas; temporal muscle flaps; silicone filling material
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References
Ketterings C (1988) Lipodystrophy and its treatment. Annals of Plastic Surgery 21(6):536–543
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© 2004 Springer-Verlag
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(2004). Progressive lipodystrophy. In: Levine, N., Levine, C.C. (eds) Dermatology Therapy. A to Z Essentials. Springer, Berlin, Heidelberg. https://doi.org/10.1007/3-540-29668-9_2230
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DOI: https://doi.org/10.1007/3-540-29668-9_2230
Publisher Name: Springer, Berlin, Heidelberg
Print ISBN: 978-3-540-00864-4
Online ISBN: 978-3-540-29668-3
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