$Unique_ID{BRK03686} $Pretitle{} $Title{Dysplastic Nevus Syndrome} $Subject{Dysplastic Nevus Syndrome B-K Mole Syndrome CMM Cutaneous Malignant Melanoma, Hereditary DNS, Hereditary Familial Atypical Mole-Malignant Melanoma Syndrome FAMMM HCMM Malignant Melanoma Basal Cell Carcinoma Xeroderma Pigmentosum} $Volume{} $Log{} Copyright (C) 1989 National Organization for Rare Disorders, Inc. 633: Dysplastic Nevus Syndrome ** IMPORTANT ** It is possible that the main title of this article (Dysplastic Nevus Syndrome) is not the name you expected. Please check the SYNONYM list to find the alternate names and disorder subdivisions covered by this article. Synonyms B-K Mole Syndrome CMM Cutaneous Malignant Melanoma, Hereditary DNS, Hereditary Familial Atypical Mole-Malignant Melanoma Syndrome FAMMM HCMM Malignant Melanoma Information on the following disorders can be found in the Related Disorders section of this report: Basal Cell Carcinoma Xeroderma Pigmentosum General Discussion ** REMINDER ** The information contained in the Rare Disease Database is provided for educational purposes only. It should not be used for diagnostic or treatment purposes. If you wish to obtain more information about this disorder, please contact your physician and/or the agencies listed in the "Resources" section of this report. Dysplastic Nevus Syndrome is a malignant genetic skin disorder characterized by mole-like tumors. These tumors may appear in different sizes, shapes, and shades of color (usually reddish-brown to pink). The tumors have a variable ability for spreading to adjacent parts of the skin, or through the blood and lymph circulation to other organs. Dysplastic Nevus Syndrome may later evolve into Malignant Melanoma, a common form of skin cancer. Symptoms Symptoms of Dysplastic Nevus Syndrome usually start during adulthood. The disorder is characterized by large moles, variable in number, which are reddish-brown to pink in color. The moles have an irregular border. The presence of dust-like melanin which gives the moles their color, and abnormally large nuclei of skin cells called melanocytes, all visible under the microscope, are characteristic of Dysplastic Nevus Syndrome. The mole- like tumors may spread to adjacent parts of the skin, or through the blood and lymph circulation, to other organs. Certain changes in the melanocyte nuclei indicate when Dysplastic Nevus Syndrome may be changing to Malignant Melanoma. Causes Dysplastic Nevus Syndrome is a disorder inherited through autosomal dominant genes. (Human traits, including the classic genetic diseases, are the product of the interaction of two genes for that condition, one received from the father and one from the mother. In dominant disorders, a single copy of the disease gene (received from either the mother or father) will be expressed "dominating" the other normal gene and resulting in appearance of the disease. The risk of transmitting the disorder from affected parent to offspring is 50 percent for each pregnancy regardless of the sex of the resulting child.) Affected Population Symptoms of Dysplastic Nevus Syndrome usually appear in adulthood. Males and females are affected in equal numbers. Related Disorders Symptoms of the following disorders can be similar to those of Dysplastic Nevus Syndrome. Comparisons may be useful for a differential diagnosis: Basal Cell Carcinomas are a common form of skin cancer that may appear as small, shiny, firm nodules; ulcerated, crusted lesions; flat, scar-like hardened plaques; or lesions difficult to differentiate from psoriasis or localized dermatitis. Xeroderma Pigmentosum is a rare autosomal recessive hereditary skin disorder which begins during early childhood. It is characterized by a defect in the ability of certain connective tissue cells (fibroblasts) to repair skin damaged by ultraviolet rays. The skin of people with Xeroderma Pigmentosum is markedly hypersensitive to sunlight. (For more information, choose "Xeroderma" as your search term in the Rare Disease Data Base.) Therapies: Standard Treatment of Dysplastic Nevus Syndrome involves Vitamin-A derivatives (retinoids) used as a topical ointment. In some patients remission of the lesions may result. In other cases the lesions have regressed to a less severe form. Use of sunscreen when the skin is exposed to sunlight is recommended. Repeated examinations of the moles is advisable in order to detect changes which may signal malignancy. Therapies: Investigational This disease entry is based upon medical information available through April 1989. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder. Resources For more information on Dysplastic Nevus Syndrome, please contact: National Organization for Rare Disorders (NORD) P.O. Box 8923 New Fairfield, CT 06812-1783 (203) 746-6518 Nevus Network 1400 S. Joyce St., #C1201 Arlington, VA 22202 (703) 920-2349 (405) 377-3403 Giant Congenital Pigmented Nevus Support Group 12 Twixt Hill Rd. Ridgefield, CT 06877 (203) 438-3863 Nevus Support Group 58 Necton Rd. Wheathampstead, Herts AL4 8AU England Skin Cancer Foundation 245 Fifth Avenue, Suite 2402 New York, NY 10016 (212) 725-5176 Melanoma Foundation 750 Menlo Avenue, Suite 250 Menlo Park, CA 94025 (415) 326-3974 American Cancer Society 1599 Clifton Rd., NE Atlanta, GA 30329 (404) 320-3333 NIH/National Cancer Institute 9000 Rockville Pike, Bldg. 31, Rm. 1A2A Bethesda, MD 20892 1-800-4-CANCER The National Cancer Institute has developed PDQ (Physician Data Query), a computerized database designed to give the public, cancer patients and families, and health professionals quick and easy access to many types of information vital to patients with this and many other types of cancer. To gain access to this service, call: Cancer Information Service (CIS) 1-800-4-CANCER In Washington, DC and suburbs in Maryland and Virginia, 636-5700 In Alaska, 1-800-638-6070 In Oahu, Hawaii, (808) 524-1234 (Neighbor islands call collect) For genetic information and genetic counseling referrals, please contact: March of Dimes Birth Defects Foundation 1275 Mamaroneck Avenue White Plains, NY 10605 (914) 428-7100 Alliance of Genetic Support Groups 35 Wisconsin Circle, Suite 440 Chevy Chase, MD 20815 (800) 336-GENE (301) 652-5553 References DYSPLASTIC NEVUS SYNDROME: ULTRAVIOLET HYPERMUTABILITY CONFIRMED IN VITRO BY ELEVATED SISTER CHROMATID EXCHANGES: E.G. Jung, et al.; Dermatologica (1986: issue 173(6)). Pp. 297-300 ROLE OF TOPICAL TRETINOIN IN MELANOMA AND DYSPLASTIC NEVI: F.L. Meyskens Jr., et al.; Journal American Acad Dermatol (October 1986: issue 15(4 Pt 2)). Pp. 822-825. THE EFFICACY OF HISTOPATHOLOGICAL CRITERIA REQUIRED FOR DIAGNOSING DYSPLASTIC NAEVI: P.M. Steijlen, et al.; Histopathology (March 1988: issue 12(3)). Pp. 289-300. MENDELIAN INHERITANCE IN MAN, 8th ed.: Victor A. McKusick; Johns Hopkins University Press, 1988. Pp. 485-486.