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Zimmermann-Laband syndrome

Tuesday 15 November 2005

Zimmermann-Laband syndrome (ZLS) is an autosomal dominant disorder characterized by gingival fibromatosis, absent or dysplastic distal phalanges, vertebral defects, hepatosplenomegaly, hypertrichosis and sometimes mental retardation.

It is an inherited disordercharacterized by abnormalities of the head, face, hands, and feet.

Most children with this disorder have generalized hypertrichosis, large gingivae, and hypoplasia of the fingernails and toenails.

Synopsis

- gingival fibromatosis
- absent or dysplastic distal phalanges
- vertebral defects
- hepatosplenomegaly
- hypertrichosis
- mental retardation (sometimes)

- generalized hypertrichosis
- large gingivae

- hypoplasia of the fingernails
- hypoplasia of the toenails

- gingival papillary projections composed of hyperplastic stratified squamous epithelium with different amounts of keratinization (16267629)

References

- Atabek ME, Pirgon O, Sert A, Toy H. Zimmermann-Laband syndrome in an infant with an atypical histologic finding. Pediatr Dev Pathol. 2005 Nov-Dec;8(6):654-7. PMID: 16267629