Zimmermann-Laband syndrome

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An extremely rare inherited syndrome of gingival fibromatosis, associated with hypoplasia of the distal phalanges, nail dysplasia, joint hypermobility, and sometimes hepatosplenomegaly.

Description

An extremely rare inherited syndrome of gingival fibromatosis, associated with hypoplasia of the distal phalanges, nail dysplasia, joint hypermobility, and sometimes hepatosplenomegaly. The nose and pinnas are usually large and poorly structures, giving the affected persons peculiar facial characteristics.

Both sexes affected. Fibromatosis of the gingiva is usually present at birth or appears shortly after. Skeletal defects include clubbed tree frog-like fingers and toes, and hyperextensibility of the metacarpophalangeal joints. The nails are usually absent or dysplastic. Occasionally, mental retardation. Inheritance is autosomal dominant.

Victor A. McKusick, Online Mendelian Inheritance in Man: Laband et al (1964) and Alvander (1965) reported 2 Asiatic Indian families (one living in the Caribbean and one in India) in which gingival fibromatosis occurred in association with 'whittling' of the terminal phalanges and absence or dysplasia of the fingernails. The report by Laband et al described the disorder in a 38-year-old Trinidad woman and 5 of her 7 children. The mother showed large, soft ears, hypertension, hyperextensibility of metacarpophalangeal joints, and splenomegaly. The affected children had soft tissue enlargement of the nose and ears, splenomegaly, skeletal abnormalities, obscure or reduced size of toenails and thumbnails, short terminal phalanges, and hypermobility of several joints. The report by Alvander described 5 affected persons in 3 generations with associated features of thickening of the soft tissues of the nose and ear with softness of the cartilages, hyperextensible joints, and hepatomegaly. Chodirker et al. (1986) reported a case of this syndrome with profound mental retardation. Pina-Neto et al. (1988) also reported a case with mental retardation.

The term Zimmermann-Laband was coined by Carl Jacob Witkop (born 1920) in 1971.

Bibliography

  • K. W. Zimmermann:
    Über Anomalien des Ektoderms.
    Vierteljahresschrift fur Zahnheilkunde, Berlin,1928, 44: 419-434
  • P. F. Laband, G. Habib, G. S. Humphreys:
    Hereditary gingival fibromatosis. Report of an affected family with associated splenomegaly and skeletal and sof- tissue abnormalities.
    Oral Surgery, Oral Medicine, Oral Pathology, St. Louis, March 1964, 17: 339-351.
  • G. Alavandar:
    Elephantiasis gingivae: report of an affected family with associated hepatomegaly, soft tissue and skeletal abnormalities.
    Journal of the All India Dental Association, Bombay, 1965, 37: 349-353.
  • C. J. Witkop Jr, A. E. Chudley:
    Gingival fibromatosis- digital anomalies.
    In: M. L. Buyse, editor. Birth defect encyclopedia. Dover: Center for Birth Defects Information Services; 1990: 779-780.
  • C. J. Witkop Jr:
    Heterogeneity in gingival fibromatosis.
    Birth Defects. Original Article Series, White Plains, NY: June 1971, 7 (7): 210-221.
  • B. N. Chodirker, A. E. Chudley, M. A. Toffler, M. H. Reed:
    Zimmerman-Laband syndrome and profound mental retardation.
    American Journal of Medical Genetics, New York, November 1986, 25 (3): 543-547.
  • J. M. Pina-Neto, L. R. Martelli-Soares, A. H. Oliveira-Souza, E. B. Lopes-Petean, M. A. S. L. Velludo, A. Campos de Freitas, J. P. Ribas:
    A new case of Zimmerman-Laband syndrome with mild mental retardation, asymmetry of limbs, and hypertrichosis. (Letter).
    American Journal of Medical Genetics, 1988, 31: 691-695.

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