Three case of xeroderma pigmentosum (Kaposi) or atrophoderma pigmentosum / by H. Radcliffe Crocker.

  • Crocker, H. Radcliffe (Henry Radcliffe), 1845-1909.
Date:
[1884]
Catalogue details

Licence: Public Domain Mark

Credit: Three case of xeroderma pigmentosum (Kaposi) or atrophoderma pigmentosum / by H. Radcliffe Crocker. Source: Wellcome Collection.

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    rare, though it probably occurred iu Hebra’s first patient, who died of cancer of the peritoneum when twenty-seven years old ; but as the tumours multiply and fungate the health of the patient, which has hitherto been so remark¬ ably good that no general symptoms belong to the disease, gives way at last from the continuous discharge and he dies marasmic and exhausted. Vidal divides the disease into three periods : 1. The onset and development of the erythema and pig¬ ment spots. 2. The atrophy of the skin with its consequent dryness, wrinkling exfoliation, and accompanying telangiectasis and superficial ulcers. 3. The formation of verrucous projections whence the fungating tumours develop. Diagnosis.—Few will dispute that the disease is sui generis, but errors may arise in its diagnosis. At the com¬ mencement the red spots have been mistaken for measles and the pigment spots for ordinary freckles; time and the surrounding circumstances will clear this up. Later on difficulties have arisen from laying too much stress on one lesion to the exclusion of the rest; thus the telangiectases have led to its being regarded as a rare form of naevus. From the scabbed ulcers and cicatricial aspect it has been pronounced to be a form of lupus; but what was it before the ulceration commenced, and what kind of lupus begins as superficial sores scattered irregularly over the face and without evident preceding infiltration of the skin ? But the disease that most closely resembles it is the atrophic stage of scleroderma universalis. Here we have immobility, white atrophy, telangiectases, and sometimes pigmentation, but in scleroderma the immobility is much greater, the telangiectases are rarely so conspicuous, and the pigmentation is not in lentiginous spots but in streaks, while the early history is totally different. Indeed, error is almost impossible if the existence of the disease is borne in mind and all the lesions and the his¬ tory of their development known.