Information on the following diseases can be found in the Related Disorders section of this report:

• Gingival Fibromatosis with Hypertrichosis
• Epilepsy

Laband Syndrome is an extremely rare inherited disorder characterized by abnormalities of the head and face (craniofacial) area and the fingers and toes, particularly the thumbs and great toes. Abnormalities affecting the fingers and toes may be apparent at birth (congenital); other symptoms may not become apparent until later during childhood. The range and severity of symptoms vary from case to case.

Most children with Laband Syndrome develop abnormally large gums (gingival fibromatosis). This condition usually occurs when the primary (deciduous) teeth erupt through the gums. Complications resulting from gingival fibromatosis may include teeth that do not meet properly (malocclusion), chewing (mastication) problems, excessive drooling (salivation), difficulty swallowing, the development of sores at the corners of the mouth, repeated gum infections, abnormal dryness of the mouth (xerostomia), premature loss (exfoliation) of teeth, and/or speech problems. In rare cases, the gums may completely cover the teeth and protrude from the mouth.

Individuals with Laband Syndrome may also exhibit facial abnormalities that may become apparent during early childhood and progress throughout adolescence. These may include an abnormally narrow facial appearance and/or overgrowth of the tongue, lips, nose, and/or ears. The cartilage of the ears and nose may also be abnormally soft. In addition, some children with this disorder may exhibit excessive hair growth (hypertrichosis).

In most cases, individuals with Laband Syndrome also have malformations of the hands and feet including abnormally long, slender fingers and toes (arachnodactyly) that may be large and swollen at the tips (distal phalanges). In some cases, the bones of the fingertips may be malformed and/or the joints of the fingers and hands (metacarpophalangeal joints) may be unusually flexible (hyperextensive). In addition, the nails of the hands and feet, particularly the thumbs and great toes, may be malformed (dysplastic) or absent.

In some cases, individuals with Laband Syndrome may exhibit other physical characteristics including additional skeletal abnormalities, an unusually large spleen (splenomegaly), and/or and abnormally large liver (hepatomegaly). Mental retardation ranging from mild to severe has been reported in some cases.
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Laband Syndrome is inherited as an autosomal dominant trait. Human traits, including the classic genetic diseases, are the product of the interaction of two genes, 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 the 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. The risk is the same for each pregnancy.

The location and nature of the genetic defect responsible for Laband Syndrome are not yet known. However, ongoing research studies are being conducted to help determine such information.
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Laband Syndrome is an extremely rare inherited disorder that affects males and females in equal numbers. It appears that the disease most frequently affects individuals who are of Eastern Indian ancestry from India and the West Indies. However, the disorder has also been reported in individuals of European descent. More than 30 cases have been reported in the medical literature.

Abnormalities affecting the fingers and toes may be apparent at birth (congenital); however, other symptoms may not become apparent until childhood. Gingival fibromatosis may not occur until a child's primary (deciduous) teeth begin to appear.
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Symptoms of the following disorder can be similar to those of Laband Syndrome. Comparisons may be useful for a differential diagnosis:

Gingival Fibromatosis with Hypertrichosis is an extremely rare disorder characterized by abnormally large gums (gingival fibromatosis) and excessive hair growth (hypertrichosis). Gingival fibromatosis usually occurs when an affected child's primary (deciduous) teeth erupt from the gums. Complications resulting from gingival fibromatosis may include teeth that do not meet properly (malocclusion), chewing (mastication) problems, excessive drooling (salivation), difficulty swallowing, the development of sores at the corners of the mouth, repeated gum infections, and/or speech problems. Other physical characteristics may include abnormal dryness of the mouth (xerostomia), failure of the teeth to erupt, and/or loss of teeth (exfoliation). Affected individuals may also exhibit mental retardation and/or diminished muscle tone (hypotonia). Approximately half of affected individuals may also exhibit seizure activity (epilepsy) and/or impaired intellectual function (oligophrenia). Gingival Fibromatosis with Hypertrichosis is thought to be inherited as an autosomal dominant genetic trait.

The following disorders may be associated with Laband Syndrome as secondary characteristics. They are not necessary for a differential diagnosis:

Individuals who exhibit abnormally large gums (gingival fibromatosis) and excessive hair growth (hypertrichosis) have had epilepsy and mental retardation.

Epilepsy is a group of disorders of the central nervous system characterized by repeated (paroxysmal) convulsive electrical disturbances in the brain. The major symptoms may include convulsions, spasms, sensory confusion, disturbances in the nerves that control involuntary body functions (autonomic nervous system), and/or loss of consciousness (syncope). In some instances, gingival hypertrophy may be secondary to anticonvulsant drugs that may be used to treat epilepsy. (For more information on this disorder, choose "Epilepsy" as your search term in the Rare Disease Database.)
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Diagnosis
In most cases, Laband Syndrome may be diagnosed during early childhood. The diagnosis may be confirmed based upon a thorough clinical evaluation, a detailed patient history, and a variety of specialized tests. X-ray studies of the fingers and/or toes (digits) and clinical examination of the nose, ears, lips, and tongue may be helpful in identifying the disorder. Malformation or absence of the nails may be apparent at birth (congenital). Confirmation of Laband Syndrome may not be made until gingival fibromatosis is observed when the primary teeth appear.

Treatment
The treatment of Laband Syndrome is directed toward the specific symptoms that are apparent in each affected individual. Pediatricians; specialists who assess and correct irregularities of the teeth (dentists and orthodontists); specialists who diagnose and treat skeletal abnormalities (orthopedists); orthopedic and dental surgeons; specialists who treat disorders affecting the tissues supporting and surrounding the teeth (periodontists); and/or other health care professionals may need to work together to ensure a comprehensive, systematic approach to treatment.

Specific therapies for the treatment of Laband Syndrome are symptomatic and supportive. In some cases, proper oral hygiene may postpone the appearance of abnormalities in the gums and may reduce their severity. Gum abnormalities may also be treated by surgical means (excision). Despite these efforts, overgrowth of the gums may recur.

Because affected children may be at risk for abnormal enlargement of the liver or spleen, prompt diagnosis of Laband Syndrome is critical to ensure appropriate early treatment.

Individuals with gingival fibromatosis and hypertrichosis may be at an increased risk for convulsive seizures (epilepsy). Epilepsy may be treated with anticonvulsant drugs that may help to control or prevent seizure activity.

Early intervention is important in ensuring that children with Laband Syndrome reach their potential. Special services that may be beneficial may include remedial education, physical therapy, speech therapy, and other medical, social, and/or vocational services.

Genetic counseling will be of benefit for affected individuals and their families. Other treatment is symptomatic and supportive.
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Research on genetic disorders and their causes is ongoing. The National Institutes of Health (NIH) is sponsoring the Human Genome Project which is aimed at mapping every gene in the human body and learning why they sometimes malfunction. It is hoped that this new knowledge will lead to prevention and treatment of genetic disorders in the future.

McKusick VA, ed. Online Mendelian Inheritance in Man (OMIM). Baltimore. MD: The Johns Hopkins University; Entry No:135500, Last Update:9/24/94; Entry Number 135400, Last Update: 9/24/94.

TEXTBOOKS
Champion RH, et al., eds. Textbook of Dermatology. 5th ed. Cambridge, MA: Blackwell Scientific Publications; 1992:2705.

Buyce ML, ed. Birth Defects Encyclopedia. Dover, MA: Blackwell Scientific Publications; For: The Center for Birth Defects Information Services Inc; 1990:775, 779-782.

JOURNAL ARTICLES
Dumic M, et al. Zimmerman-Laband syndrome: an unusual early presentation in a newborn girl. Croat Med J. 1999;40:102-03.

Robertson SP, et al. Zimmerman-Laband syndrome in an adult. Long-term follow-up of a patient with vascular and cardiac complications. Am J Med Genet. 1998;78:160-64.

Van Buggenhout GJ, et al. Zimmerman-Laband syndrome in a patient with severe mental retardation. Genet Cous. 1995;6:321-7.

Chadwick B, et al., Laband syndrome. Report of two cases, review of the literature, and identification of additional manifestations. Oral Surg Oral Med Oral Pathol. 1994;78:57-63.

Lacombe D, et al. Congenital marked hypertrichosis and Laband syndrome in a child: overlap between the gingival-hypertrichosis and Laband syndromes. Genet Couns. 1994;5:251-56.

Bakaeen G, et al. Hereditary gingival fibromatosis in a family with the Zimmermann-Laband syndrome. J Oral Pathol Med. 1991;20:457-9.

Bazopoulou-Kyrkanidou E, et al., Laband syndrome: a case report. J Oral Pathol Med. 1990;19:385-87.

de Pina Neto JM, et al. A new case of Zimmermann-Laband syndrome with mild mental retardation, asymmetry of limbs, and hypertrichosis. Am J Med Genet. 1988;31:691-95.

Chodirker BN, et al. Zimmermann-Laband syndrome and profound mental retardation. Am J Med Genet. 1986;25:543-47.

Witkop Jr. CJ. Heterogeneity in gingival fibromatosis. Birth Defects. 1971;7:210-21.

Oikawa K, et al. Laband syndrome: report of case. J Oral Surg. 1979;37:120-22.