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Benign Familial Chronic Pemphigus

Benign Familial Chronic Pemphigus Abstract In 1939 Hailey and Hailey1 described 4 patients with an apparently new and previously undescribed skin disorder. They stated that they had not found any report of a cutaneous condition characteristic of their cases. They named the condition "benign familial chronic pemphigus." Benign familial chronic pemphigus is characterized by vesicular plaques on the nape and sides of the neck, the axillae, the groin, and the intercrural, genital, perianal, and periumbilical areas, with occasional spread to the scalp and larger areas of the glabrous skin (Figs. 1, 2 and 3). The term "pemphigus" describes the clinical and histopathologic vesicular quality of the disease, but it should not connote a severe generalized blistering disease as is usually indicated by the term. The primary lesion is a vesicle, arising on apparently normal skin. It enlarges rapidlyto become a flaccid bulla. Nikolsky's sign may be positive. The vesicle ruptures, leaving an eroded base which exudes serum and results in a crust formation that strongly resembles impetigo. The lesions tend to spread peripherally, leaving a dry center and producing References 1. Hailey, H., and Hailey, H.: Familial Benign Chronic Pemphigus , Arch. Derm. Syph. 39:679-685 ( (April) ) 1939.Crossref 2. Lyles, T. W.; Knox, J. M., and Richardson, J. B.: Atypical Features in Familial Benign Chronic Pemphigus , A.M.A. Arch. Derm. 78:446-453 ( (Oct.) ) 1958.Crossref 3. Jewell, E. W., and Key, M. M.: Familial Benign Chronic Pemphigus (Hailey-Hailey) in Negroes , A.M.A. Arch. Derm. 75:715-719 ( (May) ) 1957.Crossref 4. Hailey, H., and Hailey, H.: Familial Benign Chronic Pemphigus: Report of 13 Cases in 4 Generations of a Family and Report of 9 Additional Cases in 4 Generations of a Family , Southern Med. J. 33:477-480 ( (May) ) 1940.Crossref 5. Raaschou-Nielsen, W., and Reymann, F.: Familial Benign Chronic Pemphigus , Acta Dermatovener. (Stockh.) 39:280-291, 1959. 6. Becker, S. W., and Obermayer, M. E.: Bullous Disease: Bullous Darier's Disease (Pels and Goodman); Familial Benign Pemphigus (Hailey and Hailey); Herpetiform Dermatitis Repens (Ayers and Anderson) , Arch. Derm. Syph. 41:1170-1171 ( (June) ) 1940. 7. Coles, R. B., and Thomson, S.: Familial Benign Chronic Pemphigus (Hailey-Hailey) , Brit. J. Derm. 62:265-266 ( (June) ) 1950.Crossref 8. Pels, I. R., and Goodman, M. H.: Criteria for the Histologic Diagnosis of Keratosis Follicularis (Darier): Report of a Case with Vesiculation , Arch. Derm. Syph. 39:438-455 ( (March) ) 1939.Crossref 9. Ayres, S., Jr., and Anderson, N. P.: Recurrent Herpetiform Dermatitis Repens , Arch. Derm. Syph. 40:402-413 ( (Sept.) ) 1939.Crossref 10. Pinkus, H., and Epstein, S.: Familial Benign Chronic Pemphigus: Report of a Case , Arch. Derm. Syph. 53:119-124 ( (Feb.) ) 1946.Crossref 11. Ellis, F. A.: Vesicular Darier's Disease (So-Called Benign Familial Pemphigus): A Variant of Darier's Disease , Arch. Derm. Syph. 61:715-736 ( (May) ) 1950.Crossref 12. Becker, S. W.: Dyskeratosis Bullosa (SoCalled Benign Familial Pemphigus) , Arch. Derm. Syph. 48:217 ( (Aug.) ) 1943.Crossref 13. Frank, S. B., and Rein, C. R.: Dyskeratoid Dermatosis , Arch. Derm. Syph. 45:129-151 ( (Jan.) ) 1942.Crossref 14. Sachs, W.; Hyman, A. B., and Gray, M. B.: Epidermolysis Bullosa: A Recently Described Variant , Arch. Derm. Syph. 55:91-100 ( (Jan.) ) 1947.Crossref 15. Mali, J. W. H., and Zoon, J. J.: Hailey's Disease , Dermatologica (Basel) 96:401-409, 1948.Crossref 16. Cremer, G., and Prakken, J. R.: Hailey's Disease: Familial Benign Chronic Pemphigus; Recurrent Herpetiform Dermatitis Repens , Dermatologica (Basel) 94:207-213, 1947.Crossref 17. Pöhlmann, A.: Dariersche Erkrankung in drei Generationen , Arch. Derm. Syph. 97:195-202, 1909.Crossref 18. Bukovský, J.: Psorospermosis Follicularis Vegetans Darier , Arch. Derm. Syph. 75:279-302, 1905.Crossref 19. Spitzer, R.: Zur Kenntnis der Darierschen Krankheit , Arch. Derm. Syph. 135:362-376, 1921.Crossref 20. Gougerot, H.: La Priorité du pemphigus chronique familial héréditaire bénin , Ann. Derm. Syph. (Par.) s. 8, 10:361-363 ( (July) -Aug.) 1950. 21. Gougerot, H., and Allée: Forme de transition entre la dermatite polymorphe douloureuse de BrocqDühring, et le pemphigus congénital familial héréditaire , Arch. Derm. Syph. Clin. Hop. (Par.) 5:255-257 ( (June) ) 1933. 22. Degos, R.: Dermatologie , Paris, Ernest Flammarion, 1958, p. 446. 23. Ingram, J. T., and Collins, D. H.: Familial Benign Chronic Pemphigus (Hailey-Hailey) , Acta Dermatovener. (Stockh.) 34:284-292, 1954. 24. Jablónska, S., and Chorzelski, T.: Zur Klassifikation des Pemphigus Hailey-Hailey: Seine Beziehung zum Pemphigus vulgaris und zur vesikulösen Variante des Morbus Darier , Dermatologica (Basel) 117:24-38, 1958.Crossref 25. Pearse, A. G. E.: The Ferric Ferricyanide Method for SH (Chèvremont and Frèderic, 1943) , in Histochemistry: Theoretical and Applied , Ed. 2, Boston, Little, Brown & Company, 1960, p. 803. 26. Pearse, A. G. E.: The DDD Reaction for SH Groups (Barrnett and Seligman, 1952) , in Histochemistry: Theoretical and Applied , Ed. 2, Boston, Little, Brown & Company, 1960, pp. 807-808. 27. Loewenthal, L. J. A.: Familial Benign Chronic Pemphigus: The Role of Pyogenic Bacteria , A.M.A. Arch. Derm. 80:318-326 ( (Sept.) ) 1959.Crossref 28. Haber, H., and Russell, B.: Sisters with Familial Benign Chronic Pemphigus (Gougerot, Hailey and Hailey) , Proc. Roy. Soc. Med. 43:558-560 ( (July) ) 1950. 29. Forman, L., and Calnan, L. D.: Chronic Familial Benign Pemphigus (Hailey and Hailey) , Proc. 10th Int. Cong. Derm. (Lond.) 6:277, 1952. 30. Storck, H.: Experimentelle Untersuchungen zur Frage der Bedeutung von Mikroben in der Ekzemgenese , Dermatologica (Basel) 96:177-262, 1948.Crossref 31. Rebell, G. C.: Staphylococci and Hemolytic Streptococci from Normal Skin and from Cutaneous Infections , J. Invest. Derm. 8:13-24 ( (Jan.) ) 1947. 32. Pillsbury, D. M., and Kligman, A. M.: Modern Trends in Dermatology , New York, Paul B. Hoeber, Inc., 1954, pp. 199-200. 33. Hailey, H.: Familial Benign Chronic Pemphigus: Report 13 Years After First Observation of a New Entity , South. Med. J. 46:763-765 ( (Aug.) ) 1953.Crossref 34. Lynch, F. W.: Benign Pemphigus (Hailey and Hailey): Treated with Sulfathiazole (2-Sulfanilamidothiazole) , Arch. Derm. Syph. 43:736 ( (April) ) 1941.Crossref 35. Carpenter, C. C.: Treatment of Familial Benign Chronic Pemphigus: Rapid Improvement With Penicillin Therapy , Arch. Derm. Syph. 58: 80-83 ( (July) ) 1948.Crossref 36. Salsberg, R. H.: Treatment of Familial Benign Chronic Pemphigus (Hailey and Hailey Syndrome): Rapid Healing with Aureomycin , Arch. Derm. Syph. 62:568-570 ( (Oct.) ) 1950.Crossref 37. Shelley, W. B., and Pillsbury, D. M.: Specific Systemic Antibiotic Therapy in Familial Benign Chronic Pemphigus , A.M.A. Arch. Derm. 80:554-556 ( (Nov.) ) 1959.Crossref 38. Finnerud, C. W., and Szymanski, F. J.: Chronic Benign Familial Pemphigus: A Probable Vesicular Variant of Keratosis Follicularis , Arch. Derm. Syph. 61:737-749 ( (May) ) 1950.Crossref 39. Brunauer, S. R.: Morbus Darier , in Handbuch der Haut und Geschlechtskrankheiten , Berlin, Springer-Verlag, 1931, Vol. 8, pt. (2) , pp. 210-280. 40. Svendsen, I. B., and Albrectsen, B.: The Prevalence of Dyskeratosis Follicularis (Darier's Disease) in Denmark: An Investigation of the Heredity in 22 Families , Acta Dermatovener. (Stockh.) 39:256-269, 1959. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Dermatology American Medical Association

Benign Familial Chronic Pemphigus

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References (39)

Publisher
American Medical Association
Copyright
Copyright © 1962 American Medical Association. All Rights Reserved.
ISSN
0003-987X
eISSN
1538-3652
DOI
10.1001/archderm.1962.01590100107020
Publisher site
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Abstract

Abstract In 1939 Hailey and Hailey1 described 4 patients with an apparently new and previously undescribed skin disorder. They stated that they had not found any report of a cutaneous condition characteristic of their cases. They named the condition "benign familial chronic pemphigus." Benign familial chronic pemphigus is characterized by vesicular plaques on the nape and sides of the neck, the axillae, the groin, and the intercrural, genital, perianal, and periumbilical areas, with occasional spread to the scalp and larger areas of the glabrous skin (Figs. 1, 2 and 3). The term "pemphigus" describes the clinical and histopathologic vesicular quality of the disease, but it should not connote a severe generalized blistering disease as is usually indicated by the term. The primary lesion is a vesicle, arising on apparently normal skin. It enlarges rapidlyto become a flaccid bulla. Nikolsky's sign may be positive. The vesicle ruptures, leaving an eroded base which exudes serum and results in a crust formation that strongly resembles impetigo. The lesions tend to spread peripherally, leaving a dry center and producing References 1. Hailey, H., and Hailey, H.: Familial Benign Chronic Pemphigus , Arch. Derm. Syph. 39:679-685 ( (April) ) 1939.Crossref 2. Lyles, T. W.; Knox, J. M., and Richardson, J. B.: Atypical Features in Familial Benign Chronic Pemphigus , A.M.A. Arch. Derm. 78:446-453 ( (Oct.) ) 1958.Crossref 3. Jewell, E. W., and Key, M. M.: Familial Benign Chronic Pemphigus (Hailey-Hailey) in Negroes , A.M.A. Arch. Derm. 75:715-719 ( (May) ) 1957.Crossref 4. Hailey, H., and Hailey, H.: Familial Benign Chronic Pemphigus: Report of 13 Cases in 4 Generations of a Family and Report of 9 Additional Cases in 4 Generations of a Family , Southern Med. J. 33:477-480 ( (May) ) 1940.Crossref 5. Raaschou-Nielsen, W., and Reymann, F.: Familial Benign Chronic Pemphigus , Acta Dermatovener. (Stockh.) 39:280-291, 1959. 6. Becker, S. W., and Obermayer, M. E.: Bullous Disease: Bullous Darier's Disease (Pels and Goodman); Familial Benign Pemphigus (Hailey and Hailey); Herpetiform Dermatitis Repens (Ayers and Anderson) , Arch. Derm. Syph. 41:1170-1171 ( (June) ) 1940. 7. Coles, R. B., and Thomson, S.: Familial Benign Chronic Pemphigus (Hailey-Hailey) , Brit. J. Derm. 62:265-266 ( (June) ) 1950.Crossref 8. Pels, I. R., and Goodman, M. H.: Criteria for the Histologic Diagnosis of Keratosis Follicularis (Darier): Report of a Case with Vesiculation , Arch. Derm. Syph. 39:438-455 ( (March) ) 1939.Crossref 9. Ayres, S., Jr., and Anderson, N. P.: Recurrent Herpetiform Dermatitis Repens , Arch. Derm. Syph. 40:402-413 ( (Sept.) ) 1939.Crossref 10. Pinkus, H., and Epstein, S.: Familial Benign Chronic Pemphigus: Report of a Case , Arch. Derm. Syph. 53:119-124 ( (Feb.) ) 1946.Crossref 11. Ellis, F. A.: Vesicular Darier's Disease (So-Called Benign Familial Pemphigus): A Variant of Darier's Disease , Arch. Derm. Syph. 61:715-736 ( (May) ) 1950.Crossref 12. Becker, S. W.: Dyskeratosis Bullosa (SoCalled Benign Familial Pemphigus) , Arch. Derm. Syph. 48:217 ( (Aug.) ) 1943.Crossref 13. Frank, S. B., and Rein, C. R.: Dyskeratoid Dermatosis , Arch. Derm. Syph. 45:129-151 ( (Jan.) ) 1942.Crossref 14. Sachs, W.; Hyman, A. B., and Gray, M. B.: Epidermolysis Bullosa: A Recently Described Variant , Arch. Derm. Syph. 55:91-100 ( (Jan.) ) 1947.Crossref 15. Mali, J. W. H., and Zoon, J. J.: Hailey's Disease , Dermatologica (Basel) 96:401-409, 1948.Crossref 16. Cremer, G., and Prakken, J. R.: Hailey's Disease: Familial Benign Chronic Pemphigus; Recurrent Herpetiform Dermatitis Repens , Dermatologica (Basel) 94:207-213, 1947.Crossref 17. Pöhlmann, A.: Dariersche Erkrankung in drei Generationen , Arch. Derm. Syph. 97:195-202, 1909.Crossref 18. Bukovský, J.: Psorospermosis Follicularis Vegetans Darier , Arch. Derm. Syph. 75:279-302, 1905.Crossref 19. Spitzer, R.: Zur Kenntnis der Darierschen Krankheit , Arch. Derm. Syph. 135:362-376, 1921.Crossref 20. Gougerot, H.: La Priorité du pemphigus chronique familial héréditaire bénin , Ann. Derm. Syph. (Par.) s. 8, 10:361-363 ( (July) -Aug.) 1950. 21. Gougerot, H., and Allée: Forme de transition entre la dermatite polymorphe douloureuse de BrocqDühring, et le pemphigus congénital familial héréditaire , Arch. Derm. Syph. Clin. Hop. (Par.) 5:255-257 ( (June) ) 1933. 22. Degos, R.: Dermatologie , Paris, Ernest Flammarion, 1958, p. 446. 23. Ingram, J. T., and Collins, D. H.: Familial Benign Chronic Pemphigus (Hailey-Hailey) , Acta Dermatovener. (Stockh.) 34:284-292, 1954. 24. Jablónska, S., and Chorzelski, T.: Zur Klassifikation des Pemphigus Hailey-Hailey: Seine Beziehung zum Pemphigus vulgaris und zur vesikulösen Variante des Morbus Darier , Dermatologica (Basel) 117:24-38, 1958.Crossref 25. Pearse, A. G. E.: The Ferric Ferricyanide Method for SH (Chèvremont and Frèderic, 1943) , in Histochemistry: Theoretical and Applied , Ed. 2, Boston, Little, Brown & Company, 1960, p. 803. 26. Pearse, A. G. E.: The DDD Reaction for SH Groups (Barrnett and Seligman, 1952) , in Histochemistry: Theoretical and Applied , Ed. 2, Boston, Little, Brown & Company, 1960, pp. 807-808. 27. Loewenthal, L. J. A.: Familial Benign Chronic Pemphigus: The Role of Pyogenic Bacteria , A.M.A. Arch. Derm. 80:318-326 ( (Sept.) ) 1959.Crossref 28. Haber, H., and Russell, B.: Sisters with Familial Benign Chronic Pemphigus (Gougerot, Hailey and Hailey) , Proc. Roy. Soc. Med. 43:558-560 ( (July) ) 1950. 29. Forman, L., and Calnan, L. D.: Chronic Familial Benign Pemphigus (Hailey and Hailey) , Proc. 10th Int. Cong. Derm. (Lond.) 6:277, 1952. 30. Storck, H.: Experimentelle Untersuchungen zur Frage der Bedeutung von Mikroben in der Ekzemgenese , Dermatologica (Basel) 96:177-262, 1948.Crossref 31. Rebell, G. C.: Staphylococci and Hemolytic Streptococci from Normal Skin and from Cutaneous Infections , J. Invest. Derm. 8:13-24 ( (Jan.) ) 1947. 32. Pillsbury, D. M., and Kligman, A. M.: Modern Trends in Dermatology , New York, Paul B. Hoeber, Inc., 1954, pp. 199-200. 33. Hailey, H.: Familial Benign Chronic Pemphigus: Report 13 Years After First Observation of a New Entity , South. Med. J. 46:763-765 ( (Aug.) ) 1953.Crossref 34. Lynch, F. W.: Benign Pemphigus (Hailey and Hailey): Treated with Sulfathiazole (2-Sulfanilamidothiazole) , Arch. Derm. Syph. 43:736 ( (April) ) 1941.Crossref 35. Carpenter, C. C.: Treatment of Familial Benign Chronic Pemphigus: Rapid Improvement With Penicillin Therapy , Arch. Derm. Syph. 58: 80-83 ( (July) ) 1948.Crossref 36. Salsberg, R. H.: Treatment of Familial Benign Chronic Pemphigus (Hailey and Hailey Syndrome): Rapid Healing with Aureomycin , Arch. Derm. Syph. 62:568-570 ( (Oct.) ) 1950.Crossref 37. Shelley, W. B., and Pillsbury, D. M.: Specific Systemic Antibiotic Therapy in Familial Benign Chronic Pemphigus , A.M.A. Arch. Derm. 80:554-556 ( (Nov.) ) 1959.Crossref 38. Finnerud, C. W., and Szymanski, F. J.: Chronic Benign Familial Pemphigus: A Probable Vesicular Variant of Keratosis Follicularis , Arch. Derm. Syph. 61:737-749 ( (May) ) 1950.Crossref 39. Brunauer, S. R.: Morbus Darier , in Handbuch der Haut und Geschlechtskrankheiten , Berlin, Springer-Verlag, 1931, Vol. 8, pt. (2) , pp. 210-280. 40. Svendsen, I. B., and Albrectsen, B.: The Prevalence of Dyskeratosis Follicularis (Darier's Disease) in Denmark: An Investigation of the Heredity in 22 Families , Acta Dermatovener. (Stockh.) 39:256-269, 1959.

Journal

Archives of DermatologyAmerican Medical Association

Published: Oct 1, 1962

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