Autosomal recessive hereditary disorder
Connective tissue hereditary disorder
Familial partial lipodystrophy
Hereditary disorder of endocrine system
Hereditary disorder of the integument
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Cell death inducing DFFA like effector C-related familial partial lipodystrophy (disorder)
CIDEC-related familial partial lipodystrophy
Cell death inducing DFFA like effector C-related familial partial lipodystrophy
FPLD5 - familial partial lipodystrophy type 5
A rare genetic lipodystrophy with characteristics of abnormal subcutaneous fat distribution, resulting in preservation of visceral, neck and axillary fat and absence of lower limb and gluteofemoral subcutaneous fat. Additional clinical features are acanthosis nigricans, insulin-resistant type II diabetes mellitus, dyslipidemia, and hypertension, leading to pancreatitis, hepatomegaly and hepatic steatosis.
Id1197749008
StatusPrimitive
Finding siteStructure of endocrine system
Finding siteTrunk structure
Finding siteLimb structure
Associated morphologyDystrophy
Finding siteSubcutaneous fatty tissue
ICD-10 complex map reference set
TargetE88.1
RuleTRUE
AdviceALWAYS E88.1 | POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
CorrelationSNOMED CT source code to target map code correlation not specified