title: Pseudoacromegaly in congenital generalised lipodystrophy (Berardinelli-Seip syndrome).
abstract: Pseudoacromegaly, or acromegaloidism, is characterised by a clinical appearance mimicking acromegaly in the absence of documented hypersomatotropism or past exposure to excess growth hormone. It can develop secondary to a number of congenital and acquired conditions of which severe insulin resistance is an important example. Lipodystrophy syndromes are a group of rare disorders of which autosomal recessive congenital generalised lipodystrophy is the most common type. Patients with this disorder are predisposed to insulin resistance and its associated complications such as diabetes mellitus, hypertriglyceridaemia, fatty liver, polycystic ovaries and acanthosis nigricans. Elevated circulating insulin levels in these patients rarely can give rise to soft tissue and bony overgrowth, with resultant acromegaloidism. We report an adolescent girl presenting with unusual prominence of her hands and feet; a thorough evaluation ultimately revealed a diagnosis of congenital generalised lipodystrophy.
authors.affiliation: Department of Medicine, Midnapore Medical College and Hospital, Midnapore, West Bengal, India.
authors.forename: Partha Pratim
authors.initials: PP
authors.lastname: Chakraborty
authors.affiliation: Department of Medicine, Calcutta National Medical College, Kolkata, West Bengal, India.
authors.forename: Saumik
authors.initials: S
authors.lastname: Datta
authors.affiliation: Department of Endocrinology & Metabolism, Institute of Post-graduate Medical Education & Research/SSKM Hospital, Kolkata, West Bengal, India.
authors.forename: Satinath
authors.initials: S
authors.lastname: Mukhopadhyay
authors.affiliation: Department of Endocrinology & Metabolism, Institute of Post-graduate Medical Education & Research/SSKM Hospital, Kolkata, West Bengal, India.
authors.forename: Subhankar
authors.initials: S
authors.lastname: Chowdhury
mesh_terms: D000172:Acromegaly / Q000151:congenital*; D000293:Adolescent; D005260:Female; D006801:Humans; D007333:Insulin Resistance; D052497:Lipodystrophy, Congenital Generalized / Q000150:complications*