The Bazex-Dupré-Christol Syndrome

Goeteyn, Marleen

doi:10.1001/archderm.1994.01690030069011

Cited by 67 publications

(53 citation statements)

References 4 publications

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“…[58][59][60][61][62] In BDCS, milia typically arise during infancy or early childhood, often within the first weeks or months of life, and may be the presenting sign of the disease. 61,62 Milia are especially prominent on the face, limbs, trunk, axillae, and groin and may improve significantly at puberty.…”

Section: Bazex-dupre-christol Syndrome (Online Mendelian Inheritance mentioning

confidence: 99%

Milia: A review and classification

Berk

Bayliss

2008

Journal of the American Academy of Dermatology

110

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Section: Bazex-dupre-christol Syndrome (Online Mendelian Inheritance mentioning

confidence: 99%

Milia: A review and classification

Berk

Bayliss

2008

Journal of the American Academy of Dermatology

110

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“…They appear as rm, white, discrete, super cial papules about 1-2 mm in diameter. [10][11][12] These are characterized by multiple tumours derived from the epidermis and its appendages, such as cutaneous cylindromas, trichoepitheliomas, spiradenomas and basal cell carcinomas. 6,8,9 They may be seen connected by a cord of undifferentiated epithelial cells to the structure from which it arose -a hair follicle or eccrine sweat gland.…”

Section: Discussionmentioning

confidence: 99%

Multiple milia due to radiotherapy

Lee

Griffiths

2002

Journal of Dermatological Treatment

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“…12 Puede afectar solamente a la cara o ser generalizada. En el caso presentado, además de nuestra paciente, también referían hipohidrosis su madre y tres tíos maternos.…”

Section: Caso Clínicounclassified

Síndrome Bazex-Dupré-Christol: serie de casos

et al. 2015

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RESUMENEl síndrome de Bazex-Dupré-Christol es una genodermatosis ligada al cromosoma X, la cual se caracteriza por presentar hipotricosis congénita, hipohidrosis, atrofodermia folicular, múltiples quistes de millium y carcinomas basocelulares. Presentamos a una niña y su familia con este síndrome. La paciente y sus hermanos de 5 meses de edad y de 17 años de edad presentaban múltiples quistes de millium e hipotricosis de las cejas y el cuero cabelludo. Su hermano de 8 años presentaba quistes de millium y atrofodermia folicular. Su madre presentaba hipohidrosis, hipotricosis congénita del cuero cabelludo y las cejas, así como también una lesión tumoral en la región paranasal derecha compatible con carcinoma basocelular. Destacamos la importancia del diagnóstico y del seguimiento clínico de estos niños por la posibilidad de desarrollar carcinomas basocelulares. Palabras clave: síndrome de Bazex-Dupré-Christol, hipotricosis, hipohidrosis, atrofodermia folicular y carcinoma basocelular.ABSTRACT Bazex-Dupré-Christol syndrome is an X-linked dominantly inherited disorder characterized by congenital hypotrichosis, hypohidrosis, follicular atrophoderma, multiple milia and basal cell carcinomas. We present a girl and her family with this syndrome. Our patient, her 5 month old brother and her 17 year old brother had multiple milia and scalp and eyebrows hypotrichosis. Her 8 year old brother had multiple milia and follicular atrophoderma. Her mother had hypohidrosis and congenital scalp and eyebrows hypotrichosis, as well as a right paranasal lesion suggestive of basal cell carcinoma. We emphasize the importance of precise diagnosis and clinical follow up of these patients due to the possibility of developing basal cell carcinomas.

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The Bazex-Dupré-Christol Syndrome

Cited by 67 publications

References 4 publications

Milia: A review and classification

Milia: A review and classification

Multiple milia due to radiotherapy

Síndrome Bazex-Dupré-Christol: serie de casos

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