VON RECKLINGHAUSEN'S disease, or multiple neurofibromatosis, is a form of congenital dysplasia manifested by developmental changes in the nervous system, the skin, the bones, and the muscle. The disease is frequently familial and may be associated with endocrine imbalance, muscle weakness, or paralysis due to involvement of the cerebrospinal system.
Cutaneous lesions may be divided into three general types: (1) café-au-lait spots associated with freckles or hyperpigmentation; (2) molluscum fibrosum—superficial dermal tumors caused by proliferation of the connective tissue of peripheral nerve sheaths, and (3) subcutaneous neurofibroma, distorting the skin contour.
Nervous involvement is due largely to the dominant tissue of the nerve sheath—neurectodermal Schwannian syncytium.1 Although there is a controversy as to the origin of the connective tissue, the majority opinion indicates that the Schwann sheath cells are the source of the connective tissue, causing neurofibrotic growths which may involve one nerve, a sheath or a plexus of
WESTCOTT RJ, ACKERMAN LV. ELEPHANTIASIS NEUROMATOSA: A Manifestation of von Recklinghausen's Disease. Arch Derm Syphilol. 1947;55(2):233–241. doi:10.1001/archderm.1947.01520020088006
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