Fróðskaparrit - 01.01.1965, Page 105

Dupuytrens Contracture
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stitutional inherent disease or collagen disease associated with
rheumatic disorders.
There appears to be no end to the possible aetiological factors
which have been discussed by various authors. However there
are many aspects for consideration. The condition is prevalent
in males. The condition becomes apparent mostly during the
middle and latter period of life. It is a condition which may
be multicentric in origin and may affect any part of the fascia
of the hand, although commoner to the ulnar two fingers. It
is a progressive disease. In a number of patients a strong family
history has been obtained. Although heredity is thought to be
a factor the degree of its importance and its mode of action
remains unknown.
While the information is suggestive of a familiar factor this
does not contribute much to the understanding of the inheri-
tance of Dupuytrens contracture.
Family History.
The family history is summarized in the family pedigree
Fig. 1, based on an investigation of all the inhabitants of the
islands of Sandoy and Skúvoy (population 31.12.1965: — 1699)
and of the parish register for these islands since 1783, 6 succes-
sive generations of affected individuals, including males and
females suggest autosomal dominant inheritance. The members
of the sixth generation below the age of 25 were not included,
neither were children of the previous generation who died
before they reached the same age. Two (2) of the female
members of the second generation 1 and 6 married and left for
elsewhere and it was not possible to trace the descendants.
On the average in this type of inheritance, one half
of the children of the affected parents can be expected to be
similarly affected. However, it seems that more than the ex-
pected number of the affected family members are found in
this pedigree. The finding of unaffected children from affected
parents might be considered as evidence of a reduced or low
expressivity of the mutant gene which has led to Dupuytrens
contracture in others.
9 — Fróðskaparrit 1965