basal cell nevus

ba·sal cell ne·vus

a hereditary disease noted in infancy or adolescence, characterized by lesions of the eyelids, nose, cheeks, neck, and axillae, appearing as uneroded flesh-colored papules, some becoming pedunculated, and histologically indistinguishable from basal cell epithelioma; also noted are punctate keratotic lesions of the palms and soles; the lesions usually remain benign, but in some cases ulceration and invasion occur and are evidence of malignant change; autosomal dominant inheritance; caused by mutation in the human PTCH, the homologue of the "patched gene" of Drosophila. PTCH is found on chromosome 9q22.

ba·sal cell ne·vus

(bā'săl sel nē'vŭs)
A hereditary disease noted in infancy or adolescence, characterized by lesions of the eyelids, nose, cheeks, neck, and axillae, appearing as flesh-colored papules histologically indistinguishable from basal cell epithelioma; the lesions usually remain benign, but in some cases malignant change occurs.

ba·sal cell ne·vus

(bā'săl sel nē'vŭs)
A hereditary disease noted in infancy or adolescence, characterized by lesions of the eyelids, nose, cheeks, neck, and axillae, appearing as uneroded flesh-colored papules, some becoming pedunculated, and histologically indistinguishable from basal cell epithelioma.
References in periodicals archive ?
Gorlin Syndrome is known by several names, including Gorlin-Goltz Syndrome, Basal Cell Nevus Syndrome, and Nevoid Basal Cell Carcinoma Syndrome (NBCCS).
"Medulloblastoma occurs in 10%-20% of patients with basal cell nevus syndrome and can be the presenting sign.
Consensus statement from the first international colloquium on basal cell nevus syndrome (BCNS).
In patients with early-onset or numerous BCCs, a syndromic manifestation of a genetic cause (e.g., basal cell nevus syndrome) should be considered [2].
The key role for the sonic hedgehog (SHH) signaling pathway in BCC is highlighted by Gorlin-Goltz syndrome (basal cell nevus syndrome), an autosomal-dominant condition related to germline patched 1 (PTCH1) mutation, characterized by multiple BCCs with a young age of onset, keratocystic odontogenic tumors, medulloblastoma, cardiac and ovarian fibromas, and other skeletal anomalies.
Thirty cases were identified, of which 6 were excluded (2 were not in the English or Portuguese language, 3 had no histological evidence of metastasis, and 1 had a basal cell nevus syndrome), resulting in 24 cases.
Acrochordon-like basal cell carcinomas in patients with basal cell nevus. Dermatol Online J 2007; 13: 21.
Linos et al., "High prevalence of vitamin D deficiency in patients with basal cell nevus syndrome," Archives of Dermatology, vol.
In children, it is usually associated with a genetic defect, such as basal cell nevus syndrome, xeroderma pigmentosum, nevus sebaceous, epidermodysplasia verruciformis, Rombo syndrome, or Bazex syndrome.
Gorlin syndrome or basal cell nevus syndrome (BCNS): A case report.

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