Vulvar involvement was found in ten (18%) of 53 females with von Recklinghausen's disease. Vulvectomy was necessary in only two of these ten patients. In distinguishing elephantiasis nervorum of the vulva from an intersex problem, the presence of café au lait spots and a familial history of neurofibromatosis are helpful clues.
MICHAEL M. SCHREIBER. Vulvar von Recklinghausen's Disease. Arch Dermatol. 1963;88(3):320–321. doi:10.1001/archderm.1963.01590210078011