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anomalies and curiosities of medicine-第293章

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es。 Two members; brothers; aged twenty…two and fourteen; were examined by Geoffroy…Saint…Hilaire。 This thickening of the epidermis and hair was the effect of some morbid predisposition which was transmitted from father to son; the daughters not being affected。 Five generations could be reckoned which had been affected in the manner described。

The 〃porcupine…man〃 seen by Baker contracted small…pox; and his skin was temporarily freed from the squamae; but these reappeared shortly afterward。 There are several older records of prickly men or porcupine…men。 Ascanius mentions a porcupine…man; as do Buffon and Schreber。 Autenreith speaks of a porcupine…man who was covered with innumerable verrucae。 Martin described a remarkable variety of ichthyosis in which the skin was covered with strong hairs like the bristles of a boar。 When numerous and thick the scales sometimes assumed a greenish…black hue。 An example of this condition was the individual who exhibited under the name of the 〃alligator…boy。〃 Figure 286 represents an 〃alligator…boy〃 exhibited by C。 T。 Taylor。 The skin affected in this case resembled in color and consistency that of a young alligator。 It was remarked that his olfactory sense was intact。

The harlequin fetus; of which there are specimens in Guy's Hospital; London Hospital; and the Royal College of Surgeons Museum; is the result of ichthyosis congenita。 According to Crocker either after the removal of the vernix caseosa; which may be thick; or as the skin dries it is noticeably red; smooth; shiny; and in the more severe cases covered with actual plates。 In the harlequin fetus the whole surface of the body is thickly covered with fatty epidermic plates; about 1/16 inch in thickness; which are broken up by horizontal and vertical fissures; and arranged transversely to the surface of the body like a loosely…built stone wall。 After birth these fissures may extend down into the corium; and on movement produce much pain。 The skin is so stiff and contracted that the eyes cannot be completely opened or shut; the lips are too stiff to permit of sucking; and are often inverted; the nose and ears are atrophied; the toes are contracted and cramped; and; if not born dead; the child soon dies from starvation and loss of heat。 When the disease is less severe the child may survive some time。 Crocker had a patient; a male child one month old; who survived three months。 Hallopeau and Elliot also report similar cases。

Contagious follicular keratosis is an extremely rare affection in which there are peculiar; spine…like outgrowths; consisting in exudations of the mouths of the sebaceous glands。 Leloir and Vidal shorten the name to acne cornee。

Erasmus Wilson speaks of it as ichthyosis sebacea cornea。 H。 G。 Brooke describes a case in a girl of six。 The first sign had been an eruption of little black spots on the nape of the neck。 These spots gradually developed into papules; and the whole skin took on a dirty yellow color。 Soon afterward the same appearances occurred on both shoulders; and; in the same order; spread gradually down the outer sides of the armsfirst black specks; then papules; and lastly pigmentation。 The black specks soon began to project; and comedo…like plugs and small; spine…like growths were produced。 Both the spines and plugs were very hard and firmly…rooted。 They resisted firm pressure with the forceps; and when placed on sheets of paper rattled like scraps of metal。 A direct history of contagion was traced from this case to others。

Mibelli describes an uncommon form of keratodermia (porokeratosis)。 The patient was a man of twenty…one; and exhibited the following changes in his skin: On the left side of the neck; beyond a few centimeters below the lobe of the ear; there were about ten small warty patches; irregularly scattered; yellowish…brown in color; irregular in outline; and varying in size from a lentil to a half…franc piece; or rather more。 Similar patches were seen on other portions of the face。 Patches of varying size and form; sharply limited by a kind of small; peripheral 〃dike;〃 sinuous but uninterrupted; of a color varying from red to whitish…red; dirty white; and to a hue but little different from that of the healthy skin。 Similar patches were seen on the right hand; and again on the back of the right hand was a wide space; prolonged upward in the form of a broad band on the posterior surface of the forearm to just below the olecranon; where the skin was a little smoother and thinner than the surrounding skin; and altogether bare of hairs。 The disease was noticed at the age of two; and gradually progressed。 The patient always enjoyed the most perfect health; but had contracted syphilis three years before。 A brother of the patient; aged twenty…four; for sixteen years has had the same skin…affection as this patient; on the back of the hand; and the sister and father had noticed similar lesions。

Diffuse symmetric scleroderma; or hide…bound disease; is quite rare; and presents itself in two phases: that of infiltration (more properly called hypertrophy) and atrophy; caused by shrinkage。 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
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