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Updated: Aug 11, 2026
In vitro Synthesis of Native, Fibrous Long Spacing and Segmental Long Spacing Collagen
Published on: September 20, 2012
An ultrastructural study of lipodystrophia centrifugalis abdominalis infantilis, with special reference to fibrous
M Kagoura1, M Toyoda, C Matsui
1Department of Dermatology, Faculty of Medicine, Toyama Medical and Pharmaceutical University, Toyama, Japan. kago@ms.toyama-mpu.ac.jp
Insights
Lipodystrophia centrifugalis abdominalis infantilis (LCAI) involves fat breakdown and inflammation. Fibrous long-spacing collagen (FLSC) may play a role in this rare pediatric condition.
Area of Science:
- Dermatology
- Pediatric Pathology
- Connective Tissue Biology
Background:
- Lipodystrophia centrifugalis abdominalis infantilis (LCAI) is a rare form of pediatric lipodystrophy.
- Understanding the pathogenesis of LCAI is crucial for diagnosis and management.
Observation:
- A case of LCAI in a 2.5-year-old Japanese girl presented with abdominal skin depression and erythema.
- Histopathology revealed subcutaneous fat reduction, lymphocytic infiltrate (CD4+), and septal fibrosis.
- Ultrastructural analysis identified fibrous long-spacing collagen (FLSC) within the affected fatty tissue.
Findings:
- The study identified fibrous long-spacing collagen (FLSC) in the septal area of the subcutaneous tissue in LCAI.
- FLSC structures exhibited specific periodicity (150 nm) and intraperiodic bands.
- These findings suggest a correlation between FLSC and the degradation of fibrillar collagens in LCAI.
Implications:
- The presence of FLSC may indicate a novel mechanism in the pathogenesis of LCAI.
- Further research into FLSC's role could lead to targeted therapeutic strategies for lipodystrophies.
- This case contributes to the understanding of rare pediatric inflammatory lipodystrophies.
Abstract:
Lipodystrophia centrifugalis abdominalis infantilis (LCAI) in a 2.5-year-old Japanese girl is reported. Clinically she had a curved cutaneous depression with a slightly elevated erythematous border on the left abdomen beyond the left groin. The regional lymph node was palpable. Histologic examination showed a decrease of the fat, an inflammatory infiltrate mainly composed of lymphocytes, and septal fibrosis in the subcutaneous tissue. Immunohistochemical analysis found that infiltrating lymphocytes were mainly positive against CD4. Ultrastructurally nuclei in the adipocytes were crescent shaped and located toward the periphery of the cell. Some banded structures with a periodicity of 150 nm, and with intraperiodic bands, referred to as fibrous long-spacing collagen (FLSC), were observed in the septal area of the fatty tissue. These results indicated that FLSC was correlated with the breakdown of fibrillar collagens in LCAI.
