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Ultraviolet-Induced Acantholysis in Familial Benign Chronic Pemphigus: Detection of the Forme Fruste

Ultraviolet-Induced Acantholysis in Familial Benign Chronic Pemphigus: Detection of the Forme Fruste Abstract Familial benign chronic pemphigus (FBCP) is an uncommon hereditary acantholytic disorder transmitted as an irregular autosomal dominant trait. The lesions appear primarily on flexural skin areas, but the disease also exists in a latent form in normal appearing skin. Latent acantholysis can be made manifest by subjecting the normal appearing skin to various noxious stimuli. The demonstration that ultraviolet light can easily induce this acantholytic phenomenon in patients with FBCP prompted the study of a family with this disease in an effort to identify clinically normal "carriers" of the gene. Two members in the family were discovered by means of a simple phototest method, which appears not only to identify the clinically normal heterozygote of FBCP but also to be useful in the diagnosis of suspected disease in remission when clinical lesions are not apparent. References 1. Hailey, H., and Hailey, H.: Familial Benign Chronic Pemphigus , Arch Derm 39:679-685 ( (April) ) 1939.Crossref 2. Chorzelski, T.: Experimentally Induced Acantholysis in Hailey's Benign Pemphigus , Dermatologica 124:21-30, 1962.Crossref 3. Loewenthal, L.J.A.: Familial Benign Chronic Pemphigus: The Role of Pyogenic Bacteria , Arch Derm 80:318-326 ( (Sept) ) 1959.Crossref 4. Morales, A.; Livingood, C.S.; and Hu, F.: Familial Benign Chronic Pemphigus , Arch Derm 93:324-328 ( (March) ) 1966.Crossref 5. Cram, D.L., and Winkelmann, R.K.: Ultraviolet-Induced Acantholysis in Pemphigus , Arch Derm 92:7-13 ( (July) ) 1965.Crossref 6. Cram, D.L.: Photo-Induced Acantholysis in Pemphigus, thesis, University of Minnesota Graduate School, 1966. 7. Curwen, W.L., and Jillson, O.F.: Light Hypersensitivity: A Simplified Office Procedure for Diagnosis of Contact Photodermatitis , J Invest Derm 34:207-212 ( (March) ) 1960. 8. Olson, R.L.; Sayre, R.M.; and Everett, M.A.: Effect of Anatomic Location and Time on Ultraviolet Erythema , Arch Derm 93:211-215 ( (Feb) ) 1966.Crossref 9. Raaschou-Nielsen, W., and Reymann, F.: Familial Benign Chronic Pemphigus , Acta Dermatovener 39:280-291, 1959. 10. Cullen, D.R.: Genetic Features of Familial Benign Pemphigus , Brit J Derm 77:20-23 ( (Jan) ) 1965.Crossref 11. Hailey, H.: Familial Benign Chronic Pemphigus: Report Thirteen Years After First Observation of a New Entity , Southern Med J 46:763-765 ( (Aug) ) 1953.Crossref 12. Herzberg, J.J.: Pemphigus Gougerot/Hailey-Hailey , Arch Klin Exp Derm 202:21-44 ( (Dec 21) ) 1955.Crossref 13. Palmer, D.D., and Perry, H.O.: Benign Familial Chronic Pemphigus , Arch Derm 86:493-502 ( (Oct) ) 1962.Crossref 14. Wilgram, G.N.; Caulfield, J.B.; and Lever, W.F.: An Electromicroscopic Study of Acantholysis and Dyskeratosis in Hailey and Hailey's Disease , J Invest Derm 39:373-381 ( (Nov) ) 1962.Crossref 15. Wutzdorff, E., and Kobner, H.: Zur Aetiologie der Psoriasis: Zuschriften an die Redaction , Vjschr Derm 9:203, 1877. 16. Fitzpatrick, T.B., et al: Abnormal Reactions of Man to Light , Ann Rev Med 14:195-214, 1963.Crossref 17. Baden, H.P., and Pearlman, C.: The Effect of Ultraviolet Light on Protein and Nucleic Acid Synthesis in the Epidermis , J Invest Derm 43:71-75 ( (July) ) 1964.Crossref 18. McLaren, A.D., and Luse, R.A.: Mechanism of Inactivation of Enzyme Proteins by Ultraviolet Light , Science 134:836 ( (Sept 22) ) 1961.Crossref 19. Norins, A.L.: Free Radical Formation in the Skin Following Exposure to Ultraviolet Light , J Invest Derm 39:445-447 ( (Nov) ) 1962.Crossref 20. Miescher, G.: Zur Histologie der lichtbedingten Reaktionen , Dermatologica 115:345-357, 1957.Crossref 21. Nix, T.E., Jr., et al: Ultrastructural Changes Induced by Ultraviolet Light in Human Epidermis: Basal and Spinous Layers , J Invest Derm 45:52-64 ( (July) ) 1965.Crossref 22. Beutner, E.H., et al: Autoantibodies in Pemphigus Vulgaris: Response to an Intercellular Substance of Epidermis , JAMA 192:682-688 ( (May 24) ) 1965.Crossref 23. Waldorf, D.S.; Smith, C.W.; and Strauss, A.J.L.: Immunofluorescent Studies in Pemphigus Vulgaris: Confirmatory Observations and Evaluation of Technical Considerations , Arch Derm 93:28-33 ( (Jan.) ) 1966.Crossref 24. Wright, E.T.; Epps, R.L.; and Newcomer, V.D.: Fluorescent Antibody Studies in Pemphigus Vulgaris , Arch Derm 93:562-569 ( (May) ) 1966.Crossref 25. Chorzelski, T.P.; Von Weiss, J.F.; and Lever, W.F.: Clinical Significance of Autoantibodies in Pemphigus , Arch Derm 93:570-576 ( (May) ) 1966.Crossref 26. Wilgram, G.F.; Caulfield, J.B.; and Madgic, E.V.: An Electron Microscopic Study of Acantholysis and Dyskeratosis in Pemphigus Foliaceus: With a Special Note on Peculiar Intracytoplasmic Bodies , J Invest Derm 43:287-299 ( (Nov) ) 1964.Crossref 27. Braun-Falco, O., and Vogell, W.: Elektronenmikroskopische Untersuchungen zur Dynamik der Akantholyse bei Pemphigus vulgaris: II. Mitteilung, Die akantholytische Blase , Arch Klin Exp Derm 223:533-550 ( (Dec 16) ) 1965.Crossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Dermatology American Medical Association

Ultraviolet-Induced Acantholysis in Familial Benign Chronic Pemphigus: Detection of the Forme Fruste

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Publisher
American Medical Association
Copyright
Copyright © 1967 American Medical Association. All Rights Reserved.
ISSN
0003-987X
eISSN
1538-3652
DOI
10.1001/archderm.1967.01610060030003
Publisher site
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Abstract

Abstract Familial benign chronic pemphigus (FBCP) is an uncommon hereditary acantholytic disorder transmitted as an irregular autosomal dominant trait. The lesions appear primarily on flexural skin areas, but the disease also exists in a latent form in normal appearing skin. Latent acantholysis can be made manifest by subjecting the normal appearing skin to various noxious stimuli. The demonstration that ultraviolet light can easily induce this acantholytic phenomenon in patients with FBCP prompted the study of a family with this disease in an effort to identify clinically normal "carriers" of the gene. Two members in the family were discovered by means of a simple phototest method, which appears not only to identify the clinically normal heterozygote of FBCP but also to be useful in the diagnosis of suspected disease in remission when clinical lesions are not apparent. References 1. Hailey, H., and Hailey, H.: Familial Benign Chronic Pemphigus , Arch Derm 39:679-685 ( (April) ) 1939.Crossref 2. Chorzelski, T.: Experimentally Induced Acantholysis in Hailey's Benign Pemphigus , Dermatologica 124:21-30, 1962.Crossref 3. Loewenthal, L.J.A.: Familial Benign Chronic Pemphigus: The Role of Pyogenic Bacteria , Arch Derm 80:318-326 ( (Sept) ) 1959.Crossref 4. Morales, A.; Livingood, C.S.; and Hu, F.: Familial Benign Chronic Pemphigus , Arch Derm 93:324-328 ( (March) ) 1966.Crossref 5. Cram, D.L., and Winkelmann, R.K.: Ultraviolet-Induced Acantholysis in Pemphigus , Arch Derm 92:7-13 ( (July) ) 1965.Crossref 6. Cram, D.L.: Photo-Induced Acantholysis in Pemphigus, thesis, University of Minnesota Graduate School, 1966. 7. Curwen, W.L., and Jillson, O.F.: Light Hypersensitivity: A Simplified Office Procedure for Diagnosis of Contact Photodermatitis , J Invest Derm 34:207-212 ( (March) ) 1960. 8. Olson, R.L.; Sayre, R.M.; and Everett, M.A.: Effect of Anatomic Location and Time on Ultraviolet Erythema , Arch Derm 93:211-215 ( (Feb) ) 1966.Crossref 9. Raaschou-Nielsen, W., and Reymann, F.: Familial Benign Chronic Pemphigus , Acta Dermatovener 39:280-291, 1959. 10. Cullen, D.R.: Genetic Features of Familial Benign Pemphigus , Brit J Derm 77:20-23 ( (Jan) ) 1965.Crossref 11. Hailey, H.: Familial Benign Chronic Pemphigus: Report Thirteen Years After First Observation of a New Entity , Southern Med J 46:763-765 ( (Aug) ) 1953.Crossref 12. Herzberg, J.J.: Pemphigus Gougerot/Hailey-Hailey , Arch Klin Exp Derm 202:21-44 ( (Dec 21) ) 1955.Crossref 13. Palmer, D.D., and Perry, H.O.: Benign Familial Chronic Pemphigus , Arch Derm 86:493-502 ( (Oct) ) 1962.Crossref 14. Wilgram, G.N.; Caulfield, J.B.; and Lever, W.F.: An Electromicroscopic Study of Acantholysis and Dyskeratosis in Hailey and Hailey's Disease , J Invest Derm 39:373-381 ( (Nov) ) 1962.Crossref 15. Wutzdorff, E., and Kobner, H.: Zur Aetiologie der Psoriasis: Zuschriften an die Redaction , Vjschr Derm 9:203, 1877. 16. Fitzpatrick, T.B., et al: Abnormal Reactions of Man to Light , Ann Rev Med 14:195-214, 1963.Crossref 17. Baden, H.P., and Pearlman, C.: The Effect of Ultraviolet Light on Protein and Nucleic Acid Synthesis in the Epidermis , J Invest Derm 43:71-75 ( (July) ) 1964.Crossref 18. McLaren, A.D., and Luse, R.A.: Mechanism of Inactivation of Enzyme Proteins by Ultraviolet Light , Science 134:836 ( (Sept 22) ) 1961.Crossref 19. Norins, A.L.: Free Radical Formation in the Skin Following Exposure to Ultraviolet Light , J Invest Derm 39:445-447 ( (Nov) ) 1962.Crossref 20. Miescher, G.: Zur Histologie der lichtbedingten Reaktionen , Dermatologica 115:345-357, 1957.Crossref 21. Nix, T.E., Jr., et al: Ultrastructural Changes Induced by Ultraviolet Light in Human Epidermis: Basal and Spinous Layers , J Invest Derm 45:52-64 ( (July) ) 1965.Crossref 22. Beutner, E.H., et al: Autoantibodies in Pemphigus Vulgaris: Response to an Intercellular Substance of Epidermis , JAMA 192:682-688 ( (May 24) ) 1965.Crossref 23. Waldorf, D.S.; Smith, C.W.; and Strauss, A.J.L.: Immunofluorescent Studies in Pemphigus Vulgaris: Confirmatory Observations and Evaluation of Technical Considerations , Arch Derm 93:28-33 ( (Jan.) ) 1966.Crossref 24. Wright, E.T.; Epps, R.L.; and Newcomer, V.D.: Fluorescent Antibody Studies in Pemphigus Vulgaris , Arch Derm 93:562-569 ( (May) ) 1966.Crossref 25. Chorzelski, T.P.; Von Weiss, J.F.; and Lever, W.F.: Clinical Significance of Autoantibodies in Pemphigus , Arch Derm 93:570-576 ( (May) ) 1966.Crossref 26. Wilgram, G.F.; Caulfield, J.B.; and Madgic, E.V.: An Electron Microscopic Study of Acantholysis and Dyskeratosis in Pemphigus Foliaceus: With a Special Note on Peculiar Intracytoplasmic Bodies , J Invest Derm 43:287-299 ( (Nov) ) 1964.Crossref 27. Braun-Falco, O., and Vogell, W.: Elektronenmikroskopische Untersuchungen zur Dynamik der Akantholyse bei Pemphigus vulgaris: II. Mitteilung, Die akantholytische Blase , Arch Klin Exp Derm 223:533-550 ( (Dec 16) ) 1965.Crossref

Journal

Archives of DermatologyAmerican Medical Association

Published: Dec 1, 1967

References