Ulnar clubhand is much less common than radial clubhand and is more appropriately referred to as ulnar deficiencies of the forearm. Most cases are sporadic in occurrence, although genetic syndromes are associated with ulnar dysplasia. Focal dermal hyperplasia, or Goltz syndrome (also known as Goltz-Gorlin syndrome), is an X-linked dominant condition that consists of ulnar dysplasia, long-bone defects, and split-hand or split-foot, with skin atrophy; anal, vulval, or lip papillomata; microphthalmia; iris coloboma; and mental retardation. Split-hand and split-foot with ulnar dysplasia have an autosomal dominant inheritance. An ulnar defect with mammary gland aplasia syndrome is associated with polydactyly or camptodactyly.
Synonyms and related keywords: ulnar dysplasia, ulnar aplasia, ulnar deficiencies of the forearm, split hand, split foot, polydactyly, camptodactyly, focal dermal hyperplasia, Goltz syndrome, Goltz-Gorlin syndrome, skin atrophy, anal papillomata, vulval papillomata, lip papillomata, microphthalmia, iris coloboma, mental retardation, mammary gland aplasia syndrome
Patel & Herzenberg 2007