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Development of carcinoma carcinoma of the vulva in netherton's syndrome congenital ichthyosiform erythroderma atopic dermatitis trichorrhexis nodosa

, : Development of carcinoma carcinoma of the vulva in netherton's syndrome congenital ichthyosiform erythroderma atopic dermatitis trichorrhexis nodosa. Geburtshilfe und Frauenheilkunde 47(10): 742-744

So far there has been only one incidence of a malignant tumor 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.

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Related references

Wilkinson, R.D.; Curtis, G.H.; Hawk, W.A., 1964: Netherton's Disease; Trichorrhexis Invaginata (Bamboo Hair), Congenital Ichthyosiform Erythroderma And The Atopic Diathesis. A Histopathologic Study. Archives of Dermatology 89: 46-54

Duvina, P.L.; Simoni, M.R.; Pieroni, O., 1978: Netherton's disease: a case of congenital ichthyosiform non-bullous erythroderma (Brocq) with trichorrhexis invaginata. The authors report the case of a seven year old girl originally diagnosed as a case of congenital ichthyosiform non-bullous erythroderma (Brocq). Further specific examination of the hair led to a final rare dual diagnosis of congenital ichthyosifo...

Anonymous, 1964: Nethertons disease Trichorrhexis invaginata , congenital ichthyosiform erythroderma and the atopic diathesis A histopathologic study

Mizuno, Y.; Suga, Y.; Haruna, K.; Muramatsu, S.; Hasegawa, T.; Kohroh, K.; Shimizu, T.; Komatsu, N.; Ogawa, H.; Ikeda, S., 2006: A case of a Japanese neonate with congenital ichthyosiform erythroderma diagnosed as Netherton syndrome. We report a 6-day-old Japanese girl showing generalized erythroderma accompanied by yellowish, exfoliative scaling that was accentuated on the face and scalp. Histological analysis showed psoriasiform dermatitis with acanthotic epidermis and prema...

Michalowski, R.; Urban, J.; Kucharska, D., 1974: Ichthyosiform erythrodermia and trichorrhexis invaginata (Netherton's syndrome). Annales Universitatis Mariae Curie-Sklodowska. Sectio D: Medicina 29: 199-207

Sprecher, E.; Tesfaye-Kedjela, A.; Ratajczak, P.; Bergman, R.; Richard, G., 2004: Deleterious mutations in SPINK5 in a patient with congenital ichthyosiform erythroderma: molecular testing as a helpful diagnostic tool for Netherton syndrome. The congenital erythrodermas represent a heterogeneous group of inherited and acquired disorders often accompanied by systemic infections, impaired epidermal barrier function and concomitant life-threatening fluid and electrolyte imbalance. In the...

Orbaneja, J.G.; Iglesias, L.; Moro, H., 1972: A case of Netherton's disease, of trichorrhexis invaginata, associated with a circumflex linear ichthyosis and an atopic dermatitis. Bulletin de la Societe Francaise de Dermatologie et de Syphiligraphie 79(3): 244-244

Arita, K.; Akiyama, M.; Tsuji, Y.; Iwao, F.; Kodama, K.; Shimizu, H., 2003: Squamous cell carcinoma in a patient with non-bullous congenital ichthyosiform erythroderma. British Journal of Dermatology 148(2): 367-369

Sommer, A., 1999: The syndrome of Comel-Netherton (trichorrhexis invaginata, ichthyosis linearis circumflexa and atopic eczema). Nederlands Tijdschrift Voor Geneeskunde 142(49): 2709-2710

Samitz, M.H., 1948: Primary exfoliative dermatitis; Leiner's disease; congenital ichthyosiform erythroderma. Archives of Dermatology and Syphilology 57(4): 776-778