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Related Experiment Videos

Elastic globes: electron microscopic and immunohistochemical observations.

H Nakayama1, K Hashimoto, N Kambe

  • 1Department of Dermatology & Syphilology, Wayne State University School of Medicine, Detroit.

Journal of Cutaneous Pathology
|April 1, 1988
PubMed
Summary

Elastic globes found in epidermolysis bullosa patients share immunologic similarities with elastic microfibrils but differ from epidermal keratin. This suggests a distinct composition and origin for these dermal structures.

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Area of Science:

  • Dermatology
  • Pathology
  • Biochemistry

Background:

  • Epidermolysis bullosa (EB) is a group of genetic blistering disorders affecting the skin.
  • The dermal extracellular matrix, including elastic fibers, plays a crucial role in skin structure and integrity.

Observation:

  • Specimens from an EB patient revealed numerous 'elastic globes' within the dermis.
  • Ultrastructural analysis showed these globes comprised amorphous substances, dense round structures, and fine filaments, rarely forming organized elastic fibers.

Findings:

  • Immunohistochemical staining indicated elastic globes react strongly with NKH-1 (elastic microfibrils) and anti-SAP (elastic fiber microfibrils).
  • Elastic globes were negative for EKH-4, which targets amyloid keratin and cytoid bodies, differentiating them from epidermal keratin.

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Implications:

  • Elastic globes exhibit an immunologic profile closely resembling elastic fiber microfibrils.
  • These findings suggest elastic globes are distinct from epidermal or epithelial keratin, offering insights into their composition and potential role in EB pathogenesis.