[Development of cancer (vulvar cancer) in the Netherton syndrome (ichthyosis, hair anomalies, atopic diathesis)].
So far there has been only one incidence of a malignant tumour associated with Netherton's syndrome, a rare skin disease consisting of congenital ichthyosiform erythroderma, atopic dermatitis and trichorrhexis nodosa (bamboo hair). The aetiology and clinical consequences are discussed on the occasion of a carcinoma of the vulva occurring in a case of a patient with Netherton's syndrome, both phenomena being interlinked.
Kübler
H C
HC
Abteilung für Gynäkologische Morphologie.
Kühn
W
W
Rummel
H H
HH
Kaufmann
I
I
Kaufmann
M
M
D001706:Biopsy; D002294:Carcinoma, Squamous Cell / Q000473:pathology*; D002471:Cell Transformation, Neoplastic / Q000473:pathology; D003876:Dermatitis, Atopic / Q000473:pathology*; D005260:Female; D006197:Hair / Q000002:abnormalities*; D006801:Humans; D007057:Ichthyosis / Q000473:pathology*; D008875:Middle Aged; D009378:Neoplasms, Multiple Primary / Q000473:pathology*; D010212:Papilloma / Q000473:pathology*; D012867:Skin / Q000473:pathology; D013577:Syndrome; D014844:Vulva / Q000473:pathology; D014846:Vulvar Neoplasms / Q000473:pathology*
Zur Karzinomentstehung (Vulvakarzinom) beim Netherton-Syndrom (Ichthyosis, Haaranomalien, atopische Diathese).