Cutaneous pigmentation as an incomplete form of Recklinghausen's disease : with remarks on the classification of incomplete and anomalous forms of Recklinghausen's disease / by F. Parkes Weber.

  • Weber, Frederick Parkes, 1863-1962.
Date:
[1909]
Catalogue details

Licence: In copyright

Credit: Cutaneous pigmentation as an incomplete form of Recklinghausen's disease : with remarks on the classification of incomplete and anomalous forms of Recklinghausen's disease / by F. Parkes Weber. Source: Wellcome Collection.

Provider: This material has been provided by The Royal College of Surgeons of England. The original may be consulted at The Royal College of Surgeons of England.

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    I li0 patient s general nutrition was fairly good, and menstruation had lately commenced. 1 hough she looked rather pale, examination of the blood showed nothing of pathological significance. The systolic brachial blood-pressure was found to be about 130 mm. mercury. Examination of the thoracic and abdominal viscera and of the urine showed nothing abnormal. Cutaneous sensation, knee- jerks and plantar reflexes were natural. The mental development and general intelligence were up to the average. There was no pigmentation in the mucous membrane of the mouth. The only noteworthy point in the past history of the patient was that she had always been liable to headache and bilious vomiting. But there was no tendency to fainting, such as to suggest Addison’s disease. The attacks of vomiting seemed rather to have been induced by certain articles of food, such as rich, fatty things, and, at one time, raw apples. Moreover, the patient’s mother, as a child and young woman, had been subject to so-called “ bilious attacks,” and one or two other members of the family had been inclined to similar troubles. Apparently no abnormal pigmentation had been observed in the family, except in the patient herself. In her case, from the diagnostic point of view, it was to be noted that the pigmentation affected chiefly the covered parts of the body, and that there was no special liability to freckling from exposure to the sun. This was the reverse of what one would expect in Kaposi’s “Xerodermia pigmentosa.” Moreover, in the present case, the variety in the colour and form of the areas of pigmentation and their situation (mainly on the trunk) made it seem most probable that the case was an incomplete form (“forme fruste”) of Recklinghausen’s disease—in fact, that it was a case of neuro-fibro- matosis, with typical but excessive cutaneous pigmentation, though, as yet, practically without any (superficial) tumours. There was only a single, small, flaccid, molluscous tumour to be found. This was situated in the skin on the lower part of the back, and had first been observed about three years previously. Probably no other satisfactory explanation for the pigmentation could be offered except that given, namely, that it represented an early stage or incomplete form of Recklinghausen’s disease—an explanation which was first suggested in a conversation by Dr. A. Whitfield. When I last saw the patient, in July, 1908, the previous diagnosis