A case of von Recklinghausen's neurofibromatosis, hospitalized for progressive anemia, secondary to chronic hemorrhage from the digestive tract, is reported. The imponent involvement of the skin (innumerable nodules of different size and volume), skeleton (marked cifoskoliosis giving a monstruous aspect to the patient) and visceral organs (multiple localizations of the digestive and urinary tract) is illustrated. A functional study of endocrine system suggested by a marked somatic hypoevolutism and right testis retention failed to show any pathological change.
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References
1.
BenerosoA., et GoldinE.: Maladie de Recklinghausen à localisation jéjunale.Sem. Hop. Paris, 42: 1270–1272, 1966.
2.
CollinsN. P.: Intestinal hemorrhage in von Recklinghausen's disease.Arch. Surg., 87: 374–378, 1963.
3.
DomartA., WolfrommR., TourneurR., HazardJ., et Le QuintrecY.: Localisations gastro-duodénales et pulmonaires au cours de l'évolution d'une neurofibromatose de Recklinghausen.Presse Med., 68: 1746–1748, 1960.
GlushienA. S., MansuyM. M., and LittmanD. S.: Pheochromocytoma. Its relationship to the neurocutaneous syndromes.Amer. J. Med., 14: 318–327, 1953.
6.
Gonzales-AnguloA., and ReyesH. A.: Neurofibromatosis involving to the lower urinary tract.J. Urol., 89: 804–811, 1963.
7.
KleitschW. P., KehneJ. H., and GutchC. F.: Gastrointestinal hemorrhage due to neurofibromatosis.J.A.M.A., 147: 1434–1436, 1951.
8.
LambertenghiG., e PragaC.: Aspetti ultrastrutturali dei noduli neurofibromatosi in un caso di M. di von Recklinghausen.Arch. It. Anat. Istol. Patol., 41: 447–462, 1967.
9.
LukashW. M., MorganR. I., SennettC. O., and NielsonO. F.: Gastrointestinal neoplasms in von Recklinghausen's disease.Arch. Surg., 92: 905–908, 1966.
10.
SchmittJ.: Les formes viscérales des phacomatoses.Doire Ed., Paris, 1959.
11.
ShawR. C.: Von Recklinghausen's disease of the small intestine associated with skin lesions.Am. J. Surg., 80: 360–363, 1950.
12.
VachonA., et LehmannM.: Les localisations gastro-intestinales de la maladie de Recklinghausen.Rev. Lyonn. Méd., 8: 277–284, 1952.
