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$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.