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Epidermolysis Bullosa Simplex: A New Histologic Subgroup

Epidermolysis Bullosa Simplex: A New Histologic Subgroup Abstract • Two patients with a new mechanobullous disease are described. The trauma-induced bullae were present at birth. The nails were deformed in both cases. Both patients were isolated cases; so far, the inheritance pattern is not known. The histologic picture was unique. The keratinocytes were dyskeratotic, enlarged, and had atypical mitoses on light microscopy of neonatal biopsy specimens. Electron microscopically the defect occurred in the tonofilaments, which formed round clumps. The blister formation took place in the lower part of the epidermis. (Arch Dermatol 1983;119:138-141) References 1. Gedde-Dahl T Jr: Classification of epidermolysis bullosa , in Herzberg JJ, Korting GW (eds): Pädiatrische Dermatologie . New York, FK Schattauer Verlag, 1978, pp 65-91. 2. Ishibashi Y, Klingmüller G: Erythrodermia ichthyosiformis congenita bullosa Brocq . Arch Dermatol Forsch 1968;232:205-224. 3. Pearson RW: Mechanobullous diseases , in Fitzpatrick TB, Arndt KA, Clark WH, et al (eds): Dermatology in General Medicine . New York, McGraw-Hill Book Co, 1971, pp 621-643. 4. Dowling GB, Meara RH: Epidermolysis bullosa resembling juvenile dermatitis herpetiformis . Br J Dermatol 1954;66:139-143.Crossref 5. Pearson RW, Spargo B: Electron microscopic studies of dermal-epidermal separation in human skin . J Invest Dermatol 1961;36:213-224. 6. Gedde-Dahl T Jr, Anton-Lamprecht I: Epidermolysis bullosa , in Emery EAH, Rimoin DL (eds): Principles and Practice in Medical Genetics . Edinburgh, Churchill Livingstone, 1981, chap 3.25C. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Dermatology American Medical Association

Epidermolysis Bullosa Simplex: A New Histologic Subgroup

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References (6)

Publisher
American Medical Association
Copyright
Copyright © 1983 American Medical Association. All Rights Reserved.
ISSN
0003-987X
eISSN
1538-3652
DOI
10.1001/archderm.1983.01650260046016
Publisher site
See Article on Publisher Site

Abstract

Abstract • Two patients with a new mechanobullous disease are described. The trauma-induced bullae were present at birth. The nails were deformed in both cases. Both patients were isolated cases; so far, the inheritance pattern is not known. The histologic picture was unique. The keratinocytes were dyskeratotic, enlarged, and had atypical mitoses on light microscopy of neonatal biopsy specimens. Electron microscopically the defect occurred in the tonofilaments, which formed round clumps. The blister formation took place in the lower part of the epidermis. (Arch Dermatol 1983;119:138-141) References 1. Gedde-Dahl T Jr: Classification of epidermolysis bullosa , in Herzberg JJ, Korting GW (eds): Pädiatrische Dermatologie . New York, FK Schattauer Verlag, 1978, pp 65-91. 2. Ishibashi Y, Klingmüller G: Erythrodermia ichthyosiformis congenita bullosa Brocq . Arch Dermatol Forsch 1968;232:205-224. 3. Pearson RW: Mechanobullous diseases , in Fitzpatrick TB, Arndt KA, Clark WH, et al (eds): Dermatology in General Medicine . New York, McGraw-Hill Book Co, 1971, pp 621-643. 4. Dowling GB, Meara RH: Epidermolysis bullosa resembling juvenile dermatitis herpetiformis . Br J Dermatol 1954;66:139-143.Crossref 5. Pearson RW, Spargo B: Electron microscopic studies of dermal-epidermal separation in human skin . J Invest Dermatol 1961;36:213-224. 6. Gedde-Dahl T Jr, Anton-Lamprecht I: Epidermolysis bullosa , in Emery EAH, Rimoin DL (eds): Principles and Practice in Medical Genetics . Edinburgh, Churchill Livingstone, 1981, chap 3.25C.

Journal

Archives of Dermatology – American Medical Association

Published: Feb 1, 1983

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