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EPITHELIOMA ADENOIDES CYSTICUM: BASAL CELL NEVI, AGENESIS OF THE CORPUS CALLOSUM AND DENTAL CYSTS: A Clinical and Autopsy Study

EPITHELIOMA ADENOIDES CYSTICUM: BASAL CELL NEVI, AGENESIS OF THE CORPUS CALLOSUM AND DENTAL... Abstract THE PRIMARY purpose of this paper is to present features of epithelioma adenoides cysticum which have not usually received adequate attention from dermatologists. From the earliest articles by Brooke1 and others,2 through that by Savatard3 down to the most recent reports, the congenital defects associated with this disease have not been commented on or have received only brief mention. Recently we had the opportunity to observe a patient with lesions of epithelioma adenoides cysticum who also presented other developmental anomalies constituting a bizarre clinical picture. In addition to the cutaneous lesions there were partial agenesis of the corpus callosum (discovered at autopsy) and multiple dental follicular cysts. The latter proved to be the most serious condition, for a fibrosarcoma of the mandible appeared and metastases caused the patient's death. The patient also had mesodermal anomalies consisting of a bifid sixth rib and a fibroma of the ovary. References 1. Brooke, H. G.: Epithelioma Adenoides Cysticum , Brit. J. Dermat. 4:269 ( (Sept.) ) 1892. 2. Fordyce, J. A.: Multiple Benign Cystic Epithelioma of the Skin , J. Cutan. & Genito-Urin. Dis. 10:459 ( (Dec.) ) 1892. 3. Jarisch: Zur Lehre von den Hautgeschwülsten , Arch. f. Dermat. u. Syph. 28:163, 1894. 4. White, J. C.: Multiple Benign Cystic Epithelioma , J. Cutan. & Genito-Urin. Dis. 12:477, 1894. 5. Savatard, L.: Epithelioma Adenoides Cysticum , Brit. J. Dermat. 34:381 ( (Dec.) ) 1922. 6. Butterworth, T., and Wilson, M.: Dermatologic Aspects of Tuberous Sclerosis , Arch. Dermat. & Syph. 43:1 ( (Jan.) ) 1941. 7. Goldman, H. G.: Multiple Benign Cystic Epithelioma , J. A. M. A. 115:2253 ( (Dec. 28) ) 1940. 8. Lever, W. F.: Histopathology of the Skin , Philadelphia, J. B. Lippincott Co., 1949. 9. Hartzell, M. B.: Benign Cystic Epithelioma, and Its Relationship to So-Called Syringocystadenoma, Syringocystoma, and Hemangioendothelioma , Brit. J. Dermat. 16:361, 1904. 10. Sutton, R. L., and Dennie, C. C.: Possible Interrelationship of Acanthoma Adenoides Cysticum (Multiple Benign Cystic Epithelioma) and Syringocystadenoma (Lymphangioma Tuberosum Multiplex) , J. A. M. A. 58:333 ( (Feb. 3) ) 1912. 11. Ingels, A. E.: Epithelioma Adenoides Cysticum with Features of Syringoma , Arch. Dermat. & Syph. 32:75 ( (July) ) 1935. 12. Adamson, H. G.: Two Cases of Multiple Rodent Ulcer, with a Note on the Possible Relationship Between Multiple Rodent Ulcer and Epithelioma Adenoides Cysticum of Brooke , Lancet 2:1133, 1908. 13. Traenkle, H. L.: Epithelioma Adenoides Cysticum, Trichoepithelioma and Basal Cell Cancer: Relationship Between These Diseases, as Shown by Histologic Studies of Multiple Benign Cystic Epithelioma , Arch. Dermat. & Syph. 42:822 ( (Nov.) ) 1940. 14. Nomland, R.: Multiple Basal Cell Epitheliomas Originating from Congenital Pigmented Basal Cell Nevi , Arch. Dermat. & Syph. 25:1002 ( (June) ) 1932. 15. Nesbit, T. W.: Multiple Basal Cell Epitheliomas Originating from Congenital Pigmented Basal Cell Nevi , Arch. Dermat. & Syph. 47:373 ( (March) ) 1943. 16. Pautrier, L. M., and Archambault, G.: Extraordinary Case of Cutaneous Epitheliomatosis: More Than 200 Epitheliomata Appearing over 23 Years on the Entire Skin, Without Metastases, and Showing 5 Histologic Variations , Bull. Assoc. franç. p. l'étude du cancer 16:835 ( (Dec.) ) 1927. 17. Cockayne, E. A.: Inherited Abnormalities of the Skin and Its Appendages , London, Oxford University Press, 1933. 18. This child had many small round papules, varying in color from that of normal skin to a pale brown. They were located on the face, chest, axillas and groins (figs. 1 and 2). Roentgenograms of the skull, sinuses, mandible and chest revealed no disease of the bones or teeth. A psychometric examination revealed normal mentality. Histological examination of skin from the sternal area showed the same changes as were found in a biopsy of a specimen from the mother. There were branching columns of basal cells and small cysts, and in some instances the cell masses were related to hair follicles. The individual cells were oval or fusiform, the nuclei sharply defined. Superficial roentgen therapy of 1,800 r had slight effect on the papules. 19. We wish to acknowledge the loan of roentgenograms from Dr. R. A. Snelson, Dr. E. C. Baker and Dr. H. M. Weber. 20. Summerill, F., and Hutton, J. G.: Multiple Benign Cystic Epithelioma (Epithelioma Adenoides Cysticum) with Summary of Literature , Arch. Dermat. & Syph. 26:854 ( (Nov.) ) 1932. 21. Fliegelman, M. T., and Kruse, W. T.: Hereditary Multiple Benign Cystic Epithelioma , J. Invest. Dermat. 11:189 ( (Sept.) ) 1948. 22. Van der Hoeve, J.: Doyne Memorial Lecture: Eye Symptoms in Phakomatoses , Tr. Ophth. Soc. U. Kingdom 52:380, 1932. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png A.M.A. Archives of Dermatology & Syphilology American Medical Association

EPITHELIOMA ADENOIDES CYSTICUM: BASAL CELL NEVI, AGENESIS OF THE CORPUS CALLOSUM AND DENTAL CYSTS: A Clinical and Autopsy Study

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References (18)

Publisher
American Medical Association
Copyright
Copyright © 1951 American Medical Association. All Rights Reserved.
ISSN
0096-5979
DOI
10.1001/archderm.1951.01570010076006
Publisher site
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Abstract

Abstract THE PRIMARY purpose of this paper is to present features of epithelioma adenoides cysticum which have not usually received adequate attention from dermatologists. From the earliest articles by Brooke1 and others,2 through that by Savatard3 down to the most recent reports, the congenital defects associated with this disease have not been commented on or have received only brief mention. Recently we had the opportunity to observe a patient with lesions of epithelioma adenoides cysticum who also presented other developmental anomalies constituting a bizarre clinical picture. In addition to the cutaneous lesions there were partial agenesis of the corpus callosum (discovered at autopsy) and multiple dental follicular cysts. The latter proved to be the most serious condition, for a fibrosarcoma of the mandible appeared and metastases caused the patient's death. The patient also had mesodermal anomalies consisting of a bifid sixth rib and a fibroma of the ovary. References 1. Brooke, H. G.: Epithelioma Adenoides Cysticum , Brit. J. Dermat. 4:269 ( (Sept.) ) 1892. 2. Fordyce, J. A.: Multiple Benign Cystic Epithelioma of the Skin , J. Cutan. & Genito-Urin. Dis. 10:459 ( (Dec.) ) 1892. 3. Jarisch: Zur Lehre von den Hautgeschwülsten , Arch. f. Dermat. u. Syph. 28:163, 1894. 4. White, J. C.: Multiple Benign Cystic Epithelioma , J. Cutan. & Genito-Urin. Dis. 12:477, 1894. 5. Savatard, L.: Epithelioma Adenoides Cysticum , Brit. J. Dermat. 34:381 ( (Dec.) ) 1922. 6. Butterworth, T., and Wilson, M.: Dermatologic Aspects of Tuberous Sclerosis , Arch. Dermat. & Syph. 43:1 ( (Jan.) ) 1941. 7. Goldman, H. G.: Multiple Benign Cystic Epithelioma , J. A. M. A. 115:2253 ( (Dec. 28) ) 1940. 8. Lever, W. F.: Histopathology of the Skin , Philadelphia, J. B. Lippincott Co., 1949. 9. Hartzell, M. B.: Benign Cystic Epithelioma, and Its Relationship to So-Called Syringocystadenoma, Syringocystoma, and Hemangioendothelioma , Brit. J. Dermat. 16:361, 1904. 10. Sutton, R. L., and Dennie, C. C.: Possible Interrelationship of Acanthoma Adenoides Cysticum (Multiple Benign Cystic Epithelioma) and Syringocystadenoma (Lymphangioma Tuberosum Multiplex) , J. A. M. A. 58:333 ( (Feb. 3) ) 1912. 11. Ingels, A. E.: Epithelioma Adenoides Cysticum with Features of Syringoma , Arch. Dermat. & Syph. 32:75 ( (July) ) 1935. 12. Adamson, H. G.: Two Cases of Multiple Rodent Ulcer, with a Note on the Possible Relationship Between Multiple Rodent Ulcer and Epithelioma Adenoides Cysticum of Brooke , Lancet 2:1133, 1908. 13. Traenkle, H. L.: Epithelioma Adenoides Cysticum, Trichoepithelioma and Basal Cell Cancer: Relationship Between These Diseases, as Shown by Histologic Studies of Multiple Benign Cystic Epithelioma , Arch. Dermat. & Syph. 42:822 ( (Nov.) ) 1940. 14. Nomland, R.: Multiple Basal Cell Epitheliomas Originating from Congenital Pigmented Basal Cell Nevi , Arch. Dermat. & Syph. 25:1002 ( (June) ) 1932. 15. Nesbit, T. W.: Multiple Basal Cell Epitheliomas Originating from Congenital Pigmented Basal Cell Nevi , Arch. Dermat. & Syph. 47:373 ( (March) ) 1943. 16. Pautrier, L. M., and Archambault, G.: Extraordinary Case of Cutaneous Epitheliomatosis: More Than 200 Epitheliomata Appearing over 23 Years on the Entire Skin, Without Metastases, and Showing 5 Histologic Variations , Bull. Assoc. franç. p. l'étude du cancer 16:835 ( (Dec.) ) 1927. 17. Cockayne, E. A.: Inherited Abnormalities of the Skin and Its Appendages , London, Oxford University Press, 1933. 18. This child had many small round papules, varying in color from that of normal skin to a pale brown. They were located on the face, chest, axillas and groins (figs. 1 and 2). Roentgenograms of the skull, sinuses, mandible and chest revealed no disease of the bones or teeth. A psychometric examination revealed normal mentality. Histological examination of skin from the sternal area showed the same changes as were found in a biopsy of a specimen from the mother. There were branching columns of basal cells and small cysts, and in some instances the cell masses were related to hair follicles. The individual cells were oval or fusiform, the nuclei sharply defined. Superficial roentgen therapy of 1,800 r had slight effect on the papules. 19. We wish to acknowledge the loan of roentgenograms from Dr. R. A. Snelson, Dr. E. C. Baker and Dr. H. M. Weber. 20. Summerill, F., and Hutton, J. G.: Multiple Benign Cystic Epithelioma (Epithelioma Adenoides Cysticum) with Summary of Literature , Arch. Dermat. & Syph. 26:854 ( (Nov.) ) 1932. 21. Fliegelman, M. T., and Kruse, W. T.: Hereditary Multiple Benign Cystic Epithelioma , J. Invest. Dermat. 11:189 ( (Sept.) ) 1948. 22. Van der Hoeve, J.: Doyne Memorial Lecture: Eye Symptoms in Phakomatoses , Tr. Ophth. Soc. U. Kingdom 52:380, 1932.

Journal

A.M.A. Archives of Dermatology & Syphilology – American Medical Association

Published: Jan 1, 1951

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