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. 2001 Feb;158(2):617–625. doi: 10.1016/S0002-9440(10)64003-5

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Copyright © 2001, American Society for Investigative Pathology

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Figure 3. — Electron microscopy. a: Keratinocytes: High-power view of junctional aspect (lateral borders of two basal keratinocytes). The cell on the left showed one fairly normal hemidesmosome (HD) with correctly inserting tonofilament bundles (tf); the cell on the right showed some hypoplastic (arrow) as well as one fairly normal looking hemidesmosome. This cell lacked correctly inserting tonofilaments on its whole cellular base (right side not shown). Note small areas of dilated lamina lucida (arrowheads), which were seen frequently. Dilated intercellular space between lateral borders of keratinocytes is marked by an asterisk. Anchoring fibrils appeared to be normal. Scale bar, 0.5 μm. b: Low-power view of patient’s muscle tissue (musculus vastus lateralis). Note the circular arrangement of filament bundles. Some Z-bands are lacking. Scale bar, 5 μm. c: Muscular tissue (musculus vastus lateralis). Note the disorientation of the filament bundle system (asterisk), the uneven diameter of filament bundles, discrete Z-band alterations (arrows) as well as accumulation of altered mitochondria. Scale bar, 2.5 μm. d: Muscular tissue (musculus vastus lateralis). Altered, degenerating, or necrotizing mitochondria could be observed (cristolysis, left upper corner; disappearance of cristae and densification of inner mitochondrial matrix). Scale bar, 0.5 μm. e: Intramuscular myelinated nerves (musculus vastus lateralis, low-power view). Slightly altered, at places vacuolized adaxonal bodies (v), and small axons (a, cross-section) were seen. Scale bar, 1 μm. f: Intramuscular myelinated nerves (musculus vastus lateralis, high-power view). Slightly altered adaxonal bodies (arrow), one of them (asterisk) lipidous, displaying myelin-like lamellar material were observed; the myelin-sheath showed ordinary periodicity of lamellae. The axon was triangle shaped (a, cross-section). Scale bar, 0.5 μm. g: Lateral part of neuromuscular junction (musculus vastus lateralis, high-power view). A slightly altered postsynaptic cleft system was observed. Basement membrane material, at places, had floccular appearance. Note calcospherite inclusion body in the upper left corner (incrustation was partially lost during sectioning because of poor resin penetration). Interstitial space is shown at the right side. Scale bar, 0.5 μm.

Figure 3. — Electron microscopy. a: Keratinocytes: High-power view of junctional aspect (lateral borders of two basal keratinocytes). The cell on the left showed one fairly normal hemidesmosome (HD) with correctly inserting tonofilament bundles (tf); the cell on the right showed some hypoplastic (arrow) as well as one fairly normal looking hemidesmosome. This cell lacked correctly inserting tonofilaments on its whole cellular base (right side not shown). Note small areas of dilated lamina lucida (arrowheads), which were seen frequently. Dilated intercellular space between lateral borders of keratinocytes is marked by an asterisk. Anchoring fibrils appeared to be normal. Scale bar, 0.5 μm. b: Low-power view of patient’s muscle tissue (musculus vastus lateralis). Note the circular arrangement of filament bundles. Some Z-bands are lacking. Scale bar, 5 μm. c: Muscular tissue (musculus vastus lateralis). Note the disorientation of the filament bundle system (asterisk), the uneven diameter of filament bundles, discrete Z-band alterations (arrows) as well as accumulation of altered mitochondria. Scale bar, 2.5 μm. d: Muscular tissue (musculus vastus lateralis). Altered, degenerating, or necrotizing mitochondria could be observed (cristolysis, left upper corner; disappearance of cristae and densification of inner mitochondrial matrix). Scale bar, 0.5 μm. e: Intramuscular myelinated nerves (musculus vastus lateralis, low-power view). Slightly altered, at places vacuolized adaxonal bodies (v), and small axons (a, cross-section) were seen. Scale bar, 1 μm. f: Intramuscular myelinated nerves (musculus vastus lateralis, high-power view). Slightly altered adaxonal bodies (arrow), one of them (asterisk) lipidous, displaying myelin-like lamellar material were observed; the myelin-sheath showed ordinary periodicity of lamellae. The axon was triangle shaped (a, cross-section). Scale bar, 0.5 μm. g: Lateral part of neuromuscular junction (musculus vastus lateralis, high-power view). A slightly altered postsynaptic cleft system was observed. Basement membrane material, at places, had floccular appearance. Note calcospherite inclusion body in the upper left corner (incrustation was partially lost during sectioning because of poor resin penetration). Interstitial space is shown at the right side. Scale bar, 0.5 μm.