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Submitted: 22 September 2016 Modified: 16 June 2017
HERDIN Record #: PCHRD16092214082631

A case of acquired partial lipodystrophy (Barraquer-Simons syndrome) in a 41-year-old Filipino woman.

Coreen Mae G. Copuyoc,
Mia Angela C. Verzosa

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Acquired partial lipodystrophy, also known as Barraquer-Simons syndrome, is a rare disease characterized by progressive atrophy of adipose tissue primarily on the face, leading to a gaunt appearance. Usually manifesting in childhood and in women, the evolution of fat loss occurs in a cephalocaudad manner. The lower extremities are spared and may present with paradoxical hypertrophy. Common findings are C3 hypocomplementemia and positive C3 nephritic factor (C3Nef) but the pathogenesis remains unknown. The prognosis depends on presence of co-morbidities such as membranoproliferative glomerulonephritis. We report a 41-year-old woman who presented with a 23 year history of progressive fat atrophy on the face, trunk, and upper extremities with no skin changes or other symptoms. Subsequently, a three-year history of fat hypertrophy in both lower extremities was noted. Histopathologic findings and decreased C3 ultimately led to a diagnosis of acquired partial lipodystrophy. A thorough approach to this case is reported, along with a review of current literature on pathogenetic mechanisms suspected in the disease. Amongst the lipodystrophy syndromes, acquired partial lipodystrophy portends a relatively good prognosis. However, the cosmetic disfigurement and risk for membranoproliferative glomerulonephritis necessitate a multidisciplinary form of supportive management from a medical, surgical, and psychological point of view. With watchful supportive therapy, patients may lead normal, fulfilling lives.

Publication Type
Journal
Publication Sub Type
Journal Article, Original
Title
Journal of the Philippine Dermatological Society
Frequency
Semi-Annual
Publication Date
November 2015
Volume
24
Issue
2
Page(s)
74-78
LocationLocation CodeAvailable FormatAvailability
Philippine Council for Health Research and Development Library Box no. 98-A Fulltext pdf (Request Document)