Diagnostic and pathogenetic role of café-au-lait macules in nevoid basal cell carcinoma syndrome
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چکیده
منابع مشابه
Diagnostic and pathogenetic role of café-au-lait macules in nevoid basal cell carcinoma syndrome
Café au lait spots (CALS) are common dermatologic findings that can at the same time arise in a variety of pathologic conditions such as Neurofibromatosis type 1 (NF1), together with numerous hereditary syndromes for which they represent either diagnostic criteria or associated elements (McCune Albright, Silver-Russell, LEOPARD, Ataxia-Telangiectasia). A review of the literature also revealed t...
متن کاملDiagnostic value of the café-au-lait spot in children.
The significance of the cafe-au-lait spot in the diagnosis of certain disorders of childhood is still not as widely appreciated as it should be, partly due to lack of information as to the normal incidence of such pigmentation, and partly to confusion over its relation to other forms of congenital pigmentation. The varieties of congenital melanin pigmentation of the skin consist of a number of ...
متن کاملNevoid Basal Cell Carcinoma Syndrome: Report from the Zurich Nevoid Basal Cell Carcinoma Syndrome Cohort.
BACKGROUND Nevoid basal cell carcinoma syndrome (NBCCS, Gorlin-Goltz syndrome) presents various symptoms and can disfigure patients. The estimated prevalence is around 1:100,000. OBJECTIVE To systematically investigate the clinical manifestations of NBCCS patients of the Zurich register and compare them with those described in 4 epidemiological studies performed in other countries. METHODS ...
متن کاملCommon hyperpigmentation disorders in adults: Part I. Diagnostic approach, café au lait macules, diffuse hyperpigmentation, sun exposure, and phototoxic reactions.
The cause of hyperpigmentation usually is traced to the activity and presence of melanocytes. Café au lait macules may be solitary benign findings or may indicate the presence of neurofibromatosis with its associated complications. Diffuse hyperpigmentation should prompt a search for offending medications or systemic diseases such as hemochromatosis, hyperthyroidism, and Addison's disease. In t...
متن کامل[Gorlin syndrome (nevoid basal cell carcinoma syndrome)].
CLINICAL CASE A 77 year-old male patient with Parkinson's disease and senile dementia had many facial basal cell carcinomas and an ectropion of the left eye. When he experienced respiratory difficulty he was diagnosed to have an ameloblastoma in left nostril requiring surgery. DISCUSSION Gorlin syndrome is an autosomal dominant condition characterized by basal cell carcinomas, and skeletal an...
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ژورنال
عنوان ژورنال: Hereditary Cancer in Clinical Practice
سال: 2012
ISSN: 1897-4287
DOI: 10.1186/1897-4287-10-15