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Epidermolysis Bullosa Simplex (Koebner) Is a Keratin Disorder: Ultrastructural and Immunohistochemical Study

Epidermolysis Bullosa Simplex (Koebner) Is a Keratin Disorder: Ultrastructural and... Abstract • A skin biopsy specimen was obtained from a 1-month-old female with epidermolysis bullosa simplex (Koebner). Histologically, an intraepidermal separation was seen and considered to be formed by cytolysis of the epidermal basal cells. Ultrastructurally, the basal cells were lacking in cytoplasmic tonofilaments, and the initial change of the cytolysis seemed to be cleavages of the cytoplasm. Immunohistochemically, a basal cell keratin was expressed in a suprabasal cell layer but not in the basal cell layer, and a panepithelial keratin was not detected in the basal cell layer. These findings suggest that keratin production of the epidermal cells may be delayed, resulting in a weakness of the basal cells against minor trauma to the skin. (Arch Dermatol. 1991;127:367-372) References 1. Haber RM, Hanna W, Ramsay CA, Boxall LBH. Hereditary epidermolysis bullosa . J Am Acad Dermatol. 1985;13:252-278.Crossref 2. Kero M, Niemi KM. Epidermolysis bullosa . Int J Dermatol. 1986;25:75-82.Crossref 3. Fine JD. Epidermolysis bullosa: clinical aspects, pathology, and recent advances in research . Int JDermatol. 1986;25:143-157.Crossref 4. Koebner H. Hereditare Anlage zur Blasenbildung . Dtsch Med Wochenschr. 1886;12:21-22.Crossref 5. Reynolds ES. The use of lead citrate at high pH as an electron opaque stain in electron microscopy . J Cell Biol. 1963;17:208-212.Crossref 6. Ito M, Tazawa T, Ito K, Shimizu N, Katsuumi K, Sato Y. Immunological characteristics and histological distribution of human hair fibrous proteins studies with anti-hair keratin monoclonal antibodies HKN-2, HKN-4, and HKN-6 . J Histochem Cytochem. 1986; 34:269-275.Crossref 7. Tazawa T, Ito M, Ito K, Shimizu N, Sato Y. Anti-hair keratin monoclonal antibody (HKN-2) . J Dermatol. 1985;12:313-317. 8. Ito M, Tazawa T, Shimizu N, et al. Cell differentiation in human anagen hair and hair follicles studied with anti-hair keratin monoclonal antibodies . J Invest Dermatol. 1986;86:563-569.Crossref 9. Shimizu N, Ito M, Tazawa T, Katsuumi K, Sato Y. Anti-keratin monoclonal antibody against basal cell epithelioma keratin: BKN-1 . J Dermatol. 1987;14:359-363. 10. Shimizu N, Ito M, Tazawa T, Sato Y. Immunohistochemical study on keratin expression in certain cutaneous epithelial neoplasms: basal cell carcinoma, pilomatricoma, and seborrheic keratosis . Am J Dermatopathol. 1989;11:534-540.Crossref 11. Pearson RW, Spargo B. Electron microscope studies of dermal-epidermal separation in human skin . J Invest Dermatol. 1961;36:213-224. 12. Pearson RW. Studies on the pathogenesis of epidermolysis bullosa . J Invest Dermatol. 1962;39:551-575.Crossref 13. Murata J, Suetsuge T, Fujisawa R, Hashimoto Y. A case of epidermolysis bullosa simplex of the hands and feet (Weber-Cockayne) [in Japanese] . Rinsho Dermatol. 1980;34:749-755. 14. Haneke E, Anton-Lamprecht I. Ultrastructure of blister formation in epidermolysis bullosa hereditaria, V: epidermolysis bullosa simplex localisata type Weber-Cockayne . J Invest Dermatol. 1982; 78:219-223.Crossref 15. Olaisen B, Gedde-Dahl T Jr. GPT-epidermolysis bullosa simplex (EBS Ogna) linkage in man . Hum Hered. 1973;23:189-196.Crossref 16. Dowling GB, Meara RH. Epidermolysis bullosa resembling juvenile dermatitis herpetiformis . Br J Dermatol. 1954;66:139-143.Crossref 17. Anton-Lamprecht I, Schnyder UW. Epidermolysis bullosa herpetiformis Dowling-Meara: report of a case and pathomorphogenesis . Dermatologica . 1982;164:221-235.Crossref 18. Niemi KM, Kero M, Kanerva L, Mattila R. Epidermolysis bullosa simplex: a new histologic subgroup . Arch Dermatol. 1983;119:138-141.Crossref 19. Manabe M, Naito K, Ikeda S, Takamori K, Ogawa H. Production of blister in normal human skin in vitro by blister fluids from epidermolysis bullosa . J Invest Dermatol. 1984;82:283-286.Crossref 20. Takamori K, Ikeda S, Naito K, Ogawa H. Proteases are responsible for blister formation in recessive dystrophic epidermolysis bullosa and epidermolysis bullosa simplex . Br J Dermatol. 1985; 112:533-538.Crossref 21. Savolainen ER, Kero M, Pihlajaniemi T, Kivirikko KI. Deficiency of galactosylhydroxylysyl glucosyltransferase, an enzyme of collagen synthesis, in a family with dominant epidermolysis bullosa simplex . N Engl J Med. 1981;304:197-204.Crossref 22. Sanchez G, Seltzer JL, Eisen AZ, Stapler P, Bauer EA. Generalized dominant epidermolysis bullosa simplex: decreased activity of a gelatinolytic protease in cultured fibroblasts as a phenotypic marker . J Invest Dermatol. 1983;81:576-579.Crossref 23. Kitajima Y, Inoue S, Yaoita H. Abnormal organization of keratin intermediate filaments in cultured keratinocytes of epidermolysis bullosa simplex . Arch Dermatol Res. 1989;281:5-10.Crossref 24. Lazarides E. Intermediate filaments as mechanical integrators of cellular space . Nature . 1980;283:249-256.Crossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Dermatology American Medical Association

Epidermolysis Bullosa Simplex (Koebner) Is a Keratin Disorder: Ultrastructural and Immunohistochemical Study

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References (25)

Publisher
American Medical Association
Copyright
Copyright © 1991 American Medical Association. All Rights Reserved.
ISSN
0003-987X
eISSN
1538-3652
DOI
10.1001/archderm.1991.01680030087012
Publisher site
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Abstract

Abstract • A skin biopsy specimen was obtained from a 1-month-old female with epidermolysis bullosa simplex (Koebner). Histologically, an intraepidermal separation was seen and considered to be formed by cytolysis of the epidermal basal cells. Ultrastructurally, the basal cells were lacking in cytoplasmic tonofilaments, and the initial change of the cytolysis seemed to be cleavages of the cytoplasm. Immunohistochemically, a basal cell keratin was expressed in a suprabasal cell layer but not in the basal cell layer, and a panepithelial keratin was not detected in the basal cell layer. These findings suggest that keratin production of the epidermal cells may be delayed, resulting in a weakness of the basal cells against minor trauma to the skin. (Arch Dermatol. 1991;127:367-372) References 1. Haber RM, Hanna W, Ramsay CA, Boxall LBH. Hereditary epidermolysis bullosa . J Am Acad Dermatol. 1985;13:252-278.Crossref 2. Kero M, Niemi KM. Epidermolysis bullosa . Int J Dermatol. 1986;25:75-82.Crossref 3. Fine JD. Epidermolysis bullosa: clinical aspects, pathology, and recent advances in research . Int JDermatol. 1986;25:143-157.Crossref 4. Koebner H. Hereditare Anlage zur Blasenbildung . Dtsch Med Wochenschr. 1886;12:21-22.Crossref 5. Reynolds ES. The use of lead citrate at high pH as an electron opaque stain in electron microscopy . J Cell Biol. 1963;17:208-212.Crossref 6. Ito M, Tazawa T, Ito K, Shimizu N, Katsuumi K, Sato Y. Immunological characteristics and histological distribution of human hair fibrous proteins studies with anti-hair keratin monoclonal antibodies HKN-2, HKN-4, and HKN-6 . J Histochem Cytochem. 1986; 34:269-275.Crossref 7. Tazawa T, Ito M, Ito K, Shimizu N, Sato Y. Anti-hair keratin monoclonal antibody (HKN-2) . J Dermatol. 1985;12:313-317. 8. Ito M, Tazawa T, Shimizu N, et al. Cell differentiation in human anagen hair and hair follicles studied with anti-hair keratin monoclonal antibodies . J Invest Dermatol. 1986;86:563-569.Crossref 9. Shimizu N, Ito M, Tazawa T, Katsuumi K, Sato Y. Anti-keratin monoclonal antibody against basal cell epithelioma keratin: BKN-1 . J Dermatol. 1987;14:359-363. 10. Shimizu N, Ito M, Tazawa T, Sato Y. Immunohistochemical study on keratin expression in certain cutaneous epithelial neoplasms: basal cell carcinoma, pilomatricoma, and seborrheic keratosis . Am J Dermatopathol. 1989;11:534-540.Crossref 11. Pearson RW, Spargo B. Electron microscope studies of dermal-epidermal separation in human skin . J Invest Dermatol. 1961;36:213-224. 12. Pearson RW. Studies on the pathogenesis of epidermolysis bullosa . J Invest Dermatol. 1962;39:551-575.Crossref 13. Murata J, Suetsuge T, Fujisawa R, Hashimoto Y. A case of epidermolysis bullosa simplex of the hands and feet (Weber-Cockayne) [in Japanese] . Rinsho Dermatol. 1980;34:749-755. 14. Haneke E, Anton-Lamprecht I. Ultrastructure of blister formation in epidermolysis bullosa hereditaria, V: epidermolysis bullosa simplex localisata type Weber-Cockayne . J Invest Dermatol. 1982; 78:219-223.Crossref 15. Olaisen B, Gedde-Dahl T Jr. GPT-epidermolysis bullosa simplex (EBS Ogna) linkage in man . Hum Hered. 1973;23:189-196.Crossref 16. Dowling GB, Meara RH. Epidermolysis bullosa resembling juvenile dermatitis herpetiformis . Br J Dermatol. 1954;66:139-143.Crossref 17. Anton-Lamprecht I, Schnyder UW. Epidermolysis bullosa herpetiformis Dowling-Meara: report of a case and pathomorphogenesis . Dermatologica . 1982;164:221-235.Crossref 18. Niemi KM, Kero M, Kanerva L, Mattila R. Epidermolysis bullosa simplex: a new histologic subgroup . Arch Dermatol. 1983;119:138-141.Crossref 19. Manabe M, Naito K, Ikeda S, Takamori K, Ogawa H. Production of blister in normal human skin in vitro by blister fluids from epidermolysis bullosa . J Invest Dermatol. 1984;82:283-286.Crossref 20. Takamori K, Ikeda S, Naito K, Ogawa H. Proteases are responsible for blister formation in recessive dystrophic epidermolysis bullosa and epidermolysis bullosa simplex . Br J Dermatol. 1985; 112:533-538.Crossref 21. Savolainen ER, Kero M, Pihlajaniemi T, Kivirikko KI. Deficiency of galactosylhydroxylysyl glucosyltransferase, an enzyme of collagen synthesis, in a family with dominant epidermolysis bullosa simplex . N Engl J Med. 1981;304:197-204.Crossref 22. Sanchez G, Seltzer JL, Eisen AZ, Stapler P, Bauer EA. Generalized dominant epidermolysis bullosa simplex: decreased activity of a gelatinolytic protease in cultured fibroblasts as a phenotypic marker . J Invest Dermatol. 1983;81:576-579.Crossref 23. Kitajima Y, Inoue S, Yaoita H. Abnormal organization of keratin intermediate filaments in cultured keratinocytes of epidermolysis bullosa simplex . Arch Dermatol Res. 1989;281:5-10.Crossref 24. Lazarides E. Intermediate filaments as mechanical integrators of cellular space . Nature . 1980;283:249-256.Crossref

Journal

Archives of Dermatology – American Medical Association

Published: Mar 1, 1991

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