PubMed · 6127536
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L O Simpson. 1982-11-06. The glomerular filter.. https://doi.org/10.1016/s0140-6736(82)90092-7
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There is evidence that basement membrane components control differentiation of liver sinusoids and bile ducts. These processes occur in humans in the 9th gestational week (GW). Distribution of laminin, nidogen, and type IV collagen was studied during human liver development between the 6th and the 10th GW. Laminin and nidogen lined intrahepatic microvessels in the 6th and 7th GW decreasing in quantity at the beginning of the fetal period (9th-10th GW). Type IV collagen was detected in microvessels only from the 9th GW onward. In the early periportal matrix (9th-10th GW) laminin, nidogen, and type IV collagen were diffusely distributed. At these stages, basement membrane zones of larger portal vessels and of early bile ducts were also stained for all three glycoproteins. These results show that laminin and nidogen are localized in microvessels during early human liver development and decrease in concentration at the developmental stage during which microvessels become discontinuous. In contrast, type IV collagen is not present in early microvessels but occurs when laminin and nidogen disappear. The three glycoproteins occur together only in those areas of the developing liver in which, from the 9th GW onward, the differentiation of immature liver cells into biliary epithelium takes place.
Kindler syndrome is characterized by congenital blister formation, photosensitivity, poikiloderma, and cutaneous atrophy in later life. There are few reports about the ultrastructural features of this syndrome, but still there is no consensus about the basic disease. Here we report a case of Kindler syndrome with ultrastructural findings.
This review highlights important considerations in obtaining good skin biopsy specimens to optimize results of direct immunofluorescence (IF) studies and also summarizes the various patterns of cutaneous IF deposition and their associated diagnoses. IF findings of immunobullous diseases, lupus erythematosus, vasculitis, lichen planus, and erythema multiforme are included. The uses of indirect IF studies are also reviewed including newer modifications that are valuable in helping to diagnose epidermolysis bullosa acquisita and paraneoplastic pemphigus.