David Walsh, Sydney Stephenson
1899 The Lancet  
THE following case is one of such rarity as perhaps t4 warrant a short account in the pages of THE LANCET. S, far as we can ascertain few similar cases have hithert( been recorded in this country. Fortunately there cai be no doubt as to the diagnosis, for the patient wal shown at the Dermatological Society of Great Britair and Ireland on June 29th, 1898. The diagnosis was there confirmed by the President (Dr. Radcliffe Crocker) and other members present. Dr. Crocker agreed that the case,came
more » ... at the case,came within the same category as those called by Mr. Hutchinson " lupus lymphaticus " but which he himself called lymphangiectodes " or lymphangioma circumscriptum." The case is briefly as follows. A single woman, aged 18 years, who was seen by both of us in May, 1898, complained of a disfiguring condition of the right eyeball and lids which had existed since infancy but which had not got worse. The patient's general health was good and she was well nourished. No family history was available. As regards the right eye, externally there was apparent lengthening of the eyebrow, and the eyeball was prominent. The palpebral fissure measured 38 millimetres, as against 26 millimetres in the left eye. The lids were swollen and thickened but could be distinctly pitted on moderately firm pressure. The intermarginal space was twice as broad as was that of the other eye. The cornea was small, the globe was of less size than was that of the sound eye, and there was an external strabismus of 25°. The ocular conjunctiva showed a nasvoid and vesicular condition a% we have described later in detail in this note. The vision of the left eye equalled (Jaeger). With the right eye the patient could make out the number of fingers held up against a strong light at a distance of one metre. The disc of the right eye was ill-defined, the retinal vessels were large and rather tortuous. Both eyes showed dotted opacities in the lens, less marked in the left eye. Several strands of persistent pupillary membrane were present in the right eye, which presented an external nasvoid condition. There were an enlarged caruncle and an enlarged semilunar fold which had a pale cedematous look. The fold was merged above and below in thickened conjunctiva and externally it was continuous with a translucent and vascular growth on the ocular conjunctiva. This I growth consisted of a number of jelly-like elevations, arranged roughly in the shape of a ring round the cornea, leaving a clear space of about one-eighth of an inch at the corneal margin except at its upper segment where the growth impinged on the cornea. These yellowish translucent elevations had numerous punctate hasmorrbagic injections and fine blood-vessels. In places the jelly-like bodies were about one-eighth of an inch in thickness and were pouch-like. The patient stated that the eyelid got black at times and that there used to be a lump sometimes at the inner angle of the orbit. The capillaries on the cheeks were dilated. The chief interest of this condition lies in its exact resemblance to Mr. Jonathan Hutchinson's I lupus lympbaticus." The jelly-like pouches on the eyeball were clearly varicose lymphatics. It seems possible from the history and from the dilated surface capillaries and congested retinal vessels that the eyelids and orbital contents generally may be affected with a mixed vascular and lymphatic nsevus. In that case it is interesting to note the differing appearances of what we must assume to be a common underlying pathological condition. The .elephantiasis of the eyelid and the nasvus lymphaticus of the eyeball, if this view of common origin be accepted, are possibly two distinct expressions of some congenital local affection of angio-lymphatic type. The present lesion is congenital, non-infective, chronic, and associated with congenital naevoid manifestations. Mr. Hutchinson figures a patch at the back of the tongue in a young girl and remarks that the aspect of the mucous membrane exactly resembles that of the skin. The present case does not exactly tally with Mr. Hutchinson's lupus lymphaticus inasmuch as it is apparently non-infective and is not associated with attacks of erysipelatous swelling.
doi:10.1016/s0140-6736(01)50908-3 fatcat:ublcqkcmsfconcc5g5j3at2o5i