Multiple neurofibromatosis (Von Recklinghausen's disease) / Francis Harbitz.

  • Harbitz, Francis Gottfred, 1867-
Date:
1909
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Licence: In copyright

Credit: Multiple neurofibromatosis (Von Recklinghausen's disease) / Francis Harbitz. 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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    congenital elephantiasis in five generations, a unique or at least extremely rare observation. Nonne9 has reported elephantiasis of the feet in eight members of the same family in three generations. The disease, or at least some of its symptoms, may be congenital. This is particularly true of cases of elephantiasis (Cases 8 and 9). Tumors of the skin have also been observed from birth (Cases 11 and 14) but more often appear later. Congenital tumors of the nerves— multiple or isolated—have not been observed as far as known, although it certainly may be taken for granted that they have a congenital arilage. They sometimes are discovered in childhood (Case 6 and Case B), more often at the age of puberty, but also frequently at the ages of 40, 50 or 60, when prolonged latency must be assumed, as the tendency is congen- ital. As has been stated, the pigmentations may be congenital. The evolution of the disease varies, but as a rule it is very slow, re- quiring many years. Most patients die with the disease, not from it. Ordinarily tumors and pigment spots in the skin begin to appear in small numbers; later new eruptions occur, often separated by intervals; at a more advanced stage tumors in nerve trunks and large cutaneous tumors appear. At a mature age the picture is usually very pronounced with numerous large tumors and extensive pigmentation. This is illus- trated in our Cases 1, 2, 3, 4 and 6. The eruption of new nodules and pigmented areas is sometimes, though rarely, arrested. On the other hand, the fibromata of nerves often cease to grow after having broken out in large numbers, and sometimes even after repeated recurrences after operations new nodules cease to appear, the disease having become sta- tionary (see the two cases, A and B, described in the beginning of this article). A large elephantiasis-like growth may develop slowly and steadily for years, then cease growing and become stationary (Case 8). External influences appear as a rule to exert little influence, though in certain cases the disease seems to have a predilection for places where traumatic insults may be operative. It has also been thought that cold and moisture may call forth new eruptions or hasten their development; likewise chronic irritative conditions of the skin, bad hygienic conditions, mental suffering; all this, however, is very uncertain. But there is reliable evidence that intercurrent infectious diseases and intoxications may have such influence. Experience has shown more rapid progress of the disease especially during certain physiologic periods, such as. puberty, menopause, pregnancy, puerperium, lactation (see Case 6). Of great practical importance is the malignant metamorphosis of the tumors and especially the influence of surgical intervention in the course 9. Nonne: Virchow’s Arch. f. path. Anat. [etc.] Berl., cxxv.