rinkage. The whole body
may be involved, and each joint may be fixed as the skin over it
becomes rigid. The muscles may be implicated independently of the skin,
or simultaneously, and they give the resemblance of rigor mortis. The
whole skin is so hard as to suggest the idea of a frozen corpse,
without the coldness, the temperature being only slightly subnormal.
The skin can neither be pitted nor pinched. As Crocker has well put it,
when the face is affected it is gorgonized, so to speak, both to the
eye and to the touch. The mouth cannot be opened; the lids usually
escape, but if involved they are half closed, and in either case
immovable. The effect of the disease on the chest-walls is to seriously
interfere with the respiration and to flatten and almost obliterate the
breasts; as to the limbs, from the shortening of the distended skin the
joints are fixed in a more or less rigid position. The mucous membranes
may be affected, and the secretion of both sweat and sebum is
diminished in proportion to the degree of the affection, and may be
quite absent. The atrophic type of scleroderma is preceded by an edema,
and from pressure-atrophy of the fat and muscles the skin of the face
is strained over the bones; the lips are shortened, the gums shrink
from the teeth and lead to caries, and the nostrils are compressed. The
strained skin and the emotionless features (relieved only by
telangiectatic striae) give the countenance a ghastly, corpse-like
aspect. The etiology and pathology of this disease are quite obscure.
Happily the prognosis is good, as there is a tendency to spontaneous
recovery, although the convalescence may be extended.
Although regarded by many as a disease distinct from scleroderma,
morphea is best described as a circumscribed scleroderma, and presents
itself in two clinical aspects: patches and bands, the patches being
the more common.
Scleroderma neonatorum is an induration of the skin, congenital and
occurring soon after birth, and is invariably fatal. A disease somewhat
analogous is edema neonatorum, which is a subcutaneous edema with
induration affecting the new-born. If complete it is invariably fatal,
but in a few cases in which the process has been incomplete recovery
has occurred. Gerard reports recovery from a case of sclerema
neonatorum in an infant five weeks old, which seemed in perfect health
but for this skin-affection. The back presented a remarkable induration
which involved the entire
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