连接组织神经被错误诊断为青少年局部性硬皮病
F Tirelli1, C Giraudo2, M Soliani3
1Rheumatology Unit, Department of Woman and Child Health, University Hospital of Padova, Via Giustiniani 3, Padova, 35128, Italy.
Pediatric rheumatology online journal
|October 17, 2023
概括
儿童的结缔组织瘤 (CTN) 可能被误诊为青少年局部性硬皮病 (JLS),导致不必要的免疫抑制. 区分CTN与JLS取决于非炎症性病变的外观,正常的实验室测试和皮肤活检.
科学领域:
- 皮肤病学 皮肤病学
- 儿科病理学 儿科病理学
- 医学诊断 医学诊断 医学诊断
背景情况:
- 连接组织瘤 (CTN) 是皮肤部件的先天性瘤.
- 在儿童中,CTN可以在临床上模仿青少年局部性硬皮病 (JLS),这是一个需要免疫抑制治疗的自身免疫性疾病.
研究的目的:
- 描述儿童CTN被误诊为JLS的病例.
- 在儿童中识别CTN和JLS之间的区别特征.
主要方法:
- 20多年来,对疑似JLS的儿科患者进行了回顾性分析.
- 收集并比较临床,实验室,组织病理学,核磁共振和热像数据.
- 专注于最终被诊断为CTN的患者.
主要成果:
- 17名患者 (平均年龄为4.6岁) 最初被诊断患有JLS.
- 病变是平的,类似于形状;主要是在下肢 (82.4%).
- 没有炎症标志物或自身抗体;活检证实CTN (非家族性原瘤最常见). 诊断延迟的平均时间为4.8年.
结论:
- CTN可能被误诊为JLS,导致不适当的免疫抑制治疗.
- 对CTN的关键诊断指标包括非炎症性病变,正常的仪器和实验室发现,以及准确的皮肤活检解释.
相关概念视频
Papillary Dermis
2.9K
Dermis
The dermis might be considered the "core" of the integumentary system, as distinct from the epidermis and hypodermis. It contains blood and lymph vessels, nerves, and other structures, such as hair follicles and sweat glands. The dermis is made of two layers of connective tissue that comprise an interconnected mesh of elastin and collagenous fibers, produced by fibroblasts.
Papillary Layer
The papillary layer is made of loose, areolar connective tissue, which means the collagen...
The dermis might be considered the "core" of the integumentary system, as distinct from the epidermis and hypodermis. It contains blood and lymph vessels, nerves, and other structures, such as hair follicles and sweat glands. The dermis is made of two layers of connective tissue that comprise an interconnected mesh of elastin and collagenous fibers, produced by fibroblasts.
Papillary Layer
The papillary layer is made of loose, areolar connective tissue, which means the collagen...
2.9K
Reticular Dermis
2.6K
The papillary and reticular dermis are the two layers of the dermis. They are made of connective tissue with fibers of collagen extending from one to the other, making the border between the two somewhat indistinct. The dermal papillae extending into the epidermis belong to the papillary layer, whereas the dense collagen fiber bundles below belong to the reticular layer.
Reticular Layer
Underlying the papillary layer is the much thicker reticular layer, composed of dense, irregular connective...
Reticular Layer
Underlying the papillary layer is the much thicker reticular layer, composed of dense, irregular connective...
2.6K
Loose Connective Tissue
7.2K
Loose connective tissue is found between many organs. Its main function is to absorb shock and bind tissues together. It also allows water, salts, and various nutrients to diffuse into cells that are embedded in it or present in adjacent tissues.
Adipose Tissue
Adipose tissue consists primarily of fat storage cells called adipocytes and little extracellular matrix. A large number of capillaries present within adipose tissue allow rapid mobilization of lipid molecules. White adipose tissue is...
Adipose Tissue
Adipose tissue consists primarily of fat storage cells called adipocytes and little extracellular matrix. A large number of capillaries present within adipose tissue allow rapid mobilization of lipid molecules. White adipose tissue is...
7.2K
Classification of Connective Tissues
10.7K
The connective tissues have different properties and functions in the human body. They are broadly categorized into proper, supporting, or fluid connective tissues.
Connective Tissue Proper
Connective tissue proper is the most abundant class of connective tissues. As its name implies, it predominantly connects different tissues in the body. Depending on the cell types, ground substance, viscosity, and fiber types in the ECM, connective tissue proper is further categorized into loose and dense....
Connective Tissue Proper
Connective tissue proper is the most abundant class of connective tissues. As its name implies, it predominantly connects different tissues in the body. Depending on the cell types, ground substance, viscosity, and fiber types in the ECM, connective tissue proper is further categorized into loose and dense....
10.7K
Embryonic Connective Tissues
4.4K
During early development, the embryo forms two types of connective tissues— the mesenchyme and mucoid connective tissue.
The mesenchyme is the first connective tissue that emerges in the developing embryo. It consists of loosely arranged multipotent mesenchymal cells and reticular fibers in the extracellular matrix. This loose arrangement allows easy migration of cells, which is essential for germ layer positioning, patterning, and organ morphogenesis during embryonic development.
The mesenchyme is the first connective tissue that emerges in the developing embryo. It consists of loosely arranged multipotent mesenchymal cells and reticular fibers in the extracellular matrix. This loose arrangement allows easy migration of cells, which is essential for germ layer positioning, patterning, and organ morphogenesis during embryonic development.
4.4K
Connective Tissue Cell Types
3.2K
Connective tissue develops from the mesoderm of a developing embryo and consists of cells, fibers, and ground substance: a gel-like material containing large complexes of carbohydrates and proteins. Connective tissue was first identified as a separate tissue family in the 18th century, and Johannes Peter Muller coined the term connective tissue.
Fat cells (adipocytes), smooth muscle cells (myoblasts), and bone cells (osteoblasts) are some connective tissue cell types. Some immune system cells...
Fat cells (adipocytes), smooth muscle cells (myoblasts), and bone cells (osteoblasts) are some connective tissue cell types. Some immune system cells...
3.2K


