Lipodystrophia centrifugalis abdominalis infantilis (LCAI) is a rare localized acquired lipodystrophic disorder characterized by progressive focal atrophy of the subcutis, typically of the abdomen and surrounding areas. The pathogenesis of this disease is unknown, but the presence of congenital cases and racial / ethnic predilection have led some authors to suggest a possible genetic predisposition.
LCAI predominantly affects children, with most reported cases from Japan, Korea, and China. However, there have been case reports in White individuals. LCAI more commonly presents in girls, with a reported male-to-female ratio of 1:1.6.
LCAI typically begins in the first 4 years of life, but adult-onset cases have been reported. It presents as an asymptomatic atrophic plaque with an erythematous border, most commonly on the trunk. The primary plaque expands centrifugally, during which time new depressed plaques may appear in up to one-quarter of cases. In early adolescence, erythema and expansion typically cease, with subsequent significant or complete recovery without scarring, atrophy, or depression in two-thirds of affected individuals. Regional lymphadenopathy is observed in two-thirds of active cases.
Lipodystrophia centrifugalis abdominalis infantilis
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Synopsis
Codes
ICD10CM:
E88.1 – Lipodystrophy, not elsewhere classified
SNOMEDCT:
238899009 – Lipodystrophia centrifugalis abdominalis infantalis
E88.1 – Lipodystrophy, not elsewhere classified
SNOMEDCT:
238899009 – Lipodystrophia centrifugalis abdominalis infantalis
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Last Reviewed:09/20/2022
Last Updated:10/17/2022
Last Updated:10/17/2022
Lipodystrophia centrifugalis abdominalis infantilis