Vulvar von Recklinghausen's Disease

Schreiber, Michael M.

doi:10.1001/archderm.1963.01590210078011

Cited by 25 publications

(3 citation statements)

References 2 publications

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“…More commonly, it presents as a part of generalised neurofibromatosis. Schreiber 4 described 53 women with peripheral neurofibromatosis and found 10 cases (18%) with vulvar lesions (vulvar elephantiasis nervorum).…”

Section: Discussionmentioning

confidence: 99%

Giant neurofibroma of the vulva

Maheshwari

Darshit

Rekha

et al. 2005

Aust NZ J Obst Gynaeco

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Section: Discussionmentioning

confidence: 99%

Giant neurofibroma of the vulva

Maheshwari

Darshit

Rekha

et al. 2005

Aust NZ J Obst Gynaeco

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“…Genital involvement of neurofibromatosis seems to be more common than previously reported. Schreiber studied 53 women with neurofibromatosis type I and found 10 (18%) with lesions of the vulva 13 . Over 20 cases of neurofibromatosis with vulval lesions have been described.…”

Section: Discussionmentioning

confidence: 99%

Neurofibromatosis of atypical presentation

Tato¹,

Sáez²,

Recuero³

et al. 2005

Acad Dermatol Venereol

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Neurofibromatosis (NF) is considered to be a heterogeneous neuroectodermal disease clinically defined by the presence of neurofibromas, multiple café-au-lait spots, intertriginous freckles and Lisch nodules. Mosaicism explains atypical presentations of the disease. Early mutations, before tissue differentiation, give rise to generalized disease. We report an atypical presentation of neurofibromatosis with an unusual distribution of neurofibromas, a peculiar, clinically and pathologically, neurofibroma on the trunk and the association with an ovarian serous cystoadenofibroma.

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“…A 25-month-old Caucasian girl was seen for congeni- Craven and Bresnahan, 1983Diekman et al, 1967Franceschini et al, 1978Greer and Pederson, Griebel et al, 1991Haddad and Jones, 1960Haraoka et al, 1988Hoffman, 1962Kaneti et al, 1988Kearse and Ritchey, Kenny et al, 1966Knudson and Amromin, Labardini et al, 1968 McKeown and Frazer, Messina and Strauss, 198119931961Nogita et al, 1990Nonomura et al, 1992Petsche and Radlinger, 1954Ravikumar and Lakshmanan, 1983Rink and Mitchell, 1983 Schepel and Tolhurst, Schreiber, 1963Thomas et al, 1989Van Buskirk et al, 1964Vitse et al, 1968 tal clitoromegaly that had slowly increased in size. Pregnancy and delivery were unremarkable.…”

Section: Patientmentioning

confidence: 99%

Clitoromegaly in neurofibromatosis

1995

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Genitourinary neurofibromas are rare and clitoral involvement in neurofibromatosis (NF) has been reported infrequently. However, when it occurs, clitoromegaly is often the presenting sign. In many cases, it is congenital. In 236 families with type 1 neurofibromatosis (NF-1) evaluated through the USF Regional Genetics Program between January 1982 and September 1993, four patients had clitoral involvement. In three, involvement was limited to the clitoris. Biopsy/surgical excision in two of them showed a neurofibroma in one and non-specific hamartomatous soft tissue overgrowth in the other. In the fourth patient, the involvement was asymmetric and extended to the labia majora and mons pubis. Endocrine studies and chromosomes in all patients were normal; there was no exposure to androgens, progestins, or coumadin. There was no gestational history of maternal luteomas. Review of the literature documented 26 patients with NF and clitoral involvement. Clitoral involvement in NF-1 appears to be more common than previously reported and the differential diagnosis of ambiguous genitalia should include clitoromegaly due to NF. Pathogenesis of clitoral lesions appears similar to other lesions of NF. Biopsy of such lesions appears to be justified only when malignancy is suspected.

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Vulvar von Recklinghausen's Disease

Cited by 25 publications

References 2 publications

Giant neurofibroma of the vulva

Giant neurofibroma of the vulva

Neurofibromatosis of atypical presentation

Clitoromegaly in neurofibromatosis

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