Electron microscopy and morphometry enhances differentiation of epidermolysis bullosa subtypes. With normal values for 24 parameters in skin |
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Authors: | A. E. Jaunzems Anthony E. Woods Alan Staples |
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Affiliation: | (1) Department of Histopathology, Women’s Children’s Hospital, 72 King William Road, North Adelaide, South Australia 5006 Tel. (08) 8204 6528; Fax (08) 8204 7022, AU;(2) Centre for Advanced Biomedical Studies, University of South Australia, Adelaide, South Australia, AU;(3) Department of Medical Genetics, Women’s and Children’s Hospital, North Adelaide, South Australia, AU |
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Abstract: | Electron microscopy combined with morphometry was used to establish values for 24 parameters in normal skin. These results were compared with those similarly obtained from samples of epidermolysis bullosa with a view to facilitating classification of the disease. Six of the eight subtypes of epidermolysis bullosa investigated could be differentiated. Four subtypes showed values for structural components in intact skin which were outside the normal range: (1) epidermolysis bullosa simplex generalisata gravis (hemidesmosomes); (2) epidermolysis bullosa dystrophica Pasini and (3) Cockayne-Touraine (anchoring fibrils); and (4) epidermolysis bullosa acquisita (anchoring fibrils, hemidesmosomes, and lamina lucida and lamina densa aspects of the dermoepidermal junction). Two subtypes revealed specific features which could be assessed qualitatively: distinctive, circumscribed, clumped tonofilament bodies were present in basal keratinocytes from epidermolysis bullosa herpetiformis Dowling-Meara and thick (30 nm diameter) cross-striated anchoring fibrils were absent in epidermolysis bullosa dystrophica generalisata gravis. Epidermolysis bullosa simplex Köbner and Weber-Cockayne forms could not be distinguished. |
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Keywords: | Diagnosis Mechanobullous disease Skin Ultrastructure |
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