Stomatologic Aspects of Cutaneous Diseases Multiple Hamartoma Syndrome (Cowden’s Syndrome)
Announcement The Fourth Annual Westwood Conference on Clini cal Dermatology will be held at the Sheraton InnEast, Buffalo, New York, May 20-23, 1979. The Westwood Western Conference on Clinical Dermatology will be held at the Marriott Hotel, Newport Beach, California, October 14-17, 1979. The Second Annual Westwood Carolina Conference on Clinical Dermatology will be held at the Hyatt Hotel, Hilton Head Island, South Carolina, October 25-28, 1979. For further information, please write Richard L. Dobson, M.D., 50 High Street, Buffalo, New York 14203.
Announcement Practical Skin Pathology August 26-31, 1979 Grossinger’s, Grossinger, N.Y. This is a rigorous course designed as a comprehen sive review of dermatopathology for pathologists and dermatologists. Microscopes and extensive syl labus material will be provided. The program will include 28 formal lectures and 4 question and an swer periods (16 hours), 4 microscope sessions of 400 unknown Workshop Slides and 100 Test Slides (12 hours) and 2 in-depth discussion periods of the Test Slides (8 hours). The course directors are Loren E. Golitz, M.D., and Martin H. Brownstein, M.D. For further information write: Skin Pathology Course, 2 Jordan Drive, Great Neck, N.Y. 10021 or phone: (516) 829-8578 (Dr. Brownstein).
ROBERT J. GORLIN, D.D.S., M.S., AND HEDDIE O. SEDANO, D.D.S., Dr.O.
m u l t i p l e h a m a r t o m a s y n d r o m e is inherited dominantly, but whether autosomally or X-linked has not yet been determined. It is characterized particu larly by hamartomatous neoplasia in the breasts, thyroid, skin, and mucous membranes. The cutaneous lesions of this condition clinically resemble those of adenoma sebaceum and Darter’s disease (Fig. 1). Papillomatous and lichenoid lesions of the lateral as pects of the neck, of most of the face, and of the ears are common. The lips and the oral and pharyngeal mucosae may be studded with papular lesions (Fig. 2). Lesions around the commissures of the lip, are similar to those of acanthosis nigricans. When the tongue is involved it becomes pebbly and fissured (Fig. 3). Affected women may develop hypertrophy or fibroadenomatosis of the breasts. Carcinoma of the breast occasionally supervenes. Thyroid anomalies have been fetal adenomas and follicular adenocar cinoma. A large number of other neoplasms have been reported in the syndrome, namely, meningioma of the ear canal, colonic polyposis and diverticulosis, ganglioneuromatosis of the colon, and angiomatous le sions of soft and hard tissues. Biopsy of cutaneous lesions suggests that they are hamartomas of hair folli cle origin (Fig. 4). In clinical differential diagnosis Darier’s disease, tuberous sclerosis, acanthosis nigri cans, and the multiple mucosal neuroma syndrome are contenders.
The
GENERAL BIBLIOGRAPHY Gentry, Jr., W.,C., Eskritt, N. R., and Gorlin, R. J. Multiple hamar toma syndrome (Cowden disease). Arch. Dermatol. 109:521-525, 1974. Gentry, W. C., et al. Cowden syndrome. Birth Defects 11(4): 137— 141, 1975. Gorlin, R. J., et al. Syndromes of the Head and Neck. 2nd ed., New York, McGraw-Hill, 1976. Weary, P. E., et al. Multiple hamartoma syndrome (Cowden’s dis ease). Arch. Dermatol. 106:682-690, 1972. Dr. Gorlin is Professor and Chairman of the Division of Oral Pathology, School of Dentistry, University of Minnesota, Min neapolis, Minnesota. Dr. Sedano is Professor, Division of Oral Pathology, School of Dentistry, University of Minnesota, Minneapolis, Minnesota.
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J. Dermatol. Surg. Oncol. 5:1 January 1979
GORLIN AND SEDANO
FIGURE I. Clinical appear ance o f cutaneous lesions o f Cowden's syndrome about the mouth. Note the similarity to le sions o f acanthosis nigricans.
FIGURE 2. a "P-
Clinical appearance o f lesions on the mucosa o f
FIGURE 3. Clinical appearance o f the fissures and papular lesions on the tongue.
FIGURE 4. Clinical appear ance o f lesions on periorbital skin.
J. Dermatol. Surg. Oncol. 5 :1 January 1979
13
Announcement The Fourth Annual Westwood Conference on Clini cal Dermatology will be held at the Sheraton InnEast, Buffalo, New York, May 20-23, 1979. The Westwood Western Conference on Clinical Dermatology will be held at the Marriott Hotel, Newport Beach, California, October 14-17, 1979. The Second Annual Westwood Carolina Conference on Clinical Dermatology will be held at the Hyatt Hotel, Hilton Head Island, South Carolina, October 25-28, 1979. For further information, please write Richard L. Dobson, M.D., 50 High Street, Buffalo, New York 14203.
Announcement Practical Skin Pathology August 26-31, 1979 Grossinger’s, Grossinger, N.Y. This is a rigorous course designed as a comprehen sive review of dermatopathology for pathologists and dermatologists. Microscopes and extensive syl labus material will be provided. The program will include 28 formal lectures and 4 question and an swer periods (16 hours), 4 microscope sessions of 400 unknown Workshop Slides and 100 Test Slides (12 hours) and 2 in-depth discussion periods of the Test Slides (8 hours). The course directors are Loren E. Golitz, M.D., and Martin H. Brownstein, M.D. For further information write: Skin Pathology Course, 2 Jordan Drive, Great Neck, N.Y. 10021 or phone: (516) 829-8578 (Dr. Brownstein).
ROBERT J. GORLIN, D.D.S., M.S., AND HEDDIE O. SEDANO, D.D.S., Dr.O.
m u l t i p l e h a m a r t o m a s y n d r o m e is inherited dominantly, but whether autosomally or X-linked has not yet been determined. It is characterized particu larly by hamartomatous neoplasia in the breasts, thyroid, skin, and mucous membranes. The cutaneous lesions of this condition clinically resemble those of adenoma sebaceum and Darter’s disease (Fig. 1). Papillomatous and lichenoid lesions of the lateral as pects of the neck, of most of the face, and of the ears are common. The lips and the oral and pharyngeal mucosae may be studded with papular lesions (Fig. 2). Lesions around the commissures of the lip, are similar to those of acanthosis nigricans. When the tongue is involved it becomes pebbly and fissured (Fig. 3). Affected women may develop hypertrophy or fibroadenomatosis of the breasts. Carcinoma of the breast occasionally supervenes. Thyroid anomalies have been fetal adenomas and follicular adenocar cinoma. A large number of other neoplasms have been reported in the syndrome, namely, meningioma of the ear canal, colonic polyposis and diverticulosis, ganglioneuromatosis of the colon, and angiomatous le sions of soft and hard tissues. Biopsy of cutaneous lesions suggests that they are hamartomas of hair folli cle origin (Fig. 4). In clinical differential diagnosis Darier’s disease, tuberous sclerosis, acanthosis nigri cans, and the multiple mucosal neuroma syndrome are contenders.
The
GENERAL BIBLIOGRAPHY Gentry, Jr., W.,C., Eskritt, N. R., and Gorlin, R. J. Multiple hamar toma syndrome (Cowden disease). Arch. Dermatol. 109:521-525, 1974. Gentry, W. C., et al. Cowden syndrome. Birth Defects 11(4): 137— 141, 1975. Gorlin, R. J., et al. Syndromes of the Head and Neck. 2nd ed., New York, McGraw-Hill, 1976. Weary, P. E., et al. Multiple hamartoma syndrome (Cowden’s dis ease). Arch. Dermatol. 106:682-690, 1972. Dr. Gorlin is Professor and Chairman of the Division of Oral Pathology, School of Dentistry, University of Minnesota, Min neapolis, Minnesota. Dr. Sedano is Professor, Division of Oral Pathology, School of Dentistry, University of Minnesota, Minneapolis, Minnesota.
12
J. Dermatol. Surg. Oncol. 5:1 January 1979
GORLIN AND SEDANO
FIGURE I. Clinical appear ance o f cutaneous lesions o f Cowden's syndrome about the mouth. Note the similarity to le sions o f acanthosis nigricans.
FIGURE 2. a "P-
Clinical appearance o f lesions on the mucosa o f
FIGURE 3. Clinical appearance o f the fissures and papular lesions on the tongue.
FIGURE 4. Clinical appear ance o f lesions on periorbital skin.
J. Dermatol. Surg. Oncol. 5 :1 January 1979
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