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- Sporadic Cutaneous Keratocyst without Nevoid Basal Cell Carcinoma Syndrome: Report of 1 Case.
- Hye Won Lee, Ji Young Park, Sun Hee Kang, Misun Choe
- Korean J Pathol. 2011;45(3):322-325.
- DOI: https://doi.org/10.4132/KoreanJPathol.2011.45.3.322
- 4,557 View
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- 3 Crossref
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Abstract
PDF - A cutaneous keratocyst is very rare and is ordinarily associated with nevoid basal cell carcinoma syndrome (NBCCS), also known as Gorlin syndrome. NBCCS is a rare autosomal-dominant disorder that results from the mutation in the patched homologue 1 (PTCH1) gene located on chromosome 9q22.3, with high penetrance and variable expressivity. NBCCS demonstrates multisystem manifestations such as multiple basal cell carcinomas in early age, jaw cysts and pits of the hands and feet. Cutaneous keratocysts are characteristically lined by festooned keratinized squamous epithelium with parakeratosis. The cystic wall contains neither granular cell layer nor skin appendages. To the best of our knowledge, only two cases of cutaneous keratocysts not associated with NBCCS have been reported to date. We report one another case of a histologically confirmed cutaneous keratocyst in a 50-year-old female without a family history and clinical features of NBCCS.
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Citations
Citations to this article as recorded by
- Determining the Relationship Between Cutaneous Keratocysts and Basal Cell Nevus Syndrome
Madelyn M. Class, Claire Rose Kissinger, Sidra Ibad, Aspen Trautz, Lisa Zhai, Farhaan Hafeez
Journal of Cutaneous Pathology.2025;[Epub] CrossRef - Sporadic Cutaneous Keratocyst of the Scalp: A Report of an Extremely Rare Lesion
Rana S AL-Zaidi, Eyad Tantawi, Rahaf AL-Radadi, Asrar Banjar
Cureus.2021;[Epub] CrossRef - A Large Extragnathic Keratocystic Odontogenic Tumour
Soumya Makarla, Radhika M. Bavle, Sudhakara Muniswamappa, Srinath Narasimhamurthy
Case Reports in Pathology.2015; 2015: 1. CrossRef
- Determining the Relationship Between Cutaneous Keratocysts and Basal Cell Nevus Syndrome
- Nevoid Basal Cell Carcinoma Syndrome: Report of a case.
- Hae Ryoun Park, Young Im Han, Mee Young Sol, Sun Kyung Lee
- Korean J Pathol. 1995;29(2):263-267.
- 1,743 View
- 20 Download
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Abstract
PDF
- Nevoid basal cell carcinoma syndrome (NBCC syndrome) is a very rare autosomal dominant dermatopathy characterized by a primary triad with variable secondary anomalies. The chief features include nevoid basal cell carcinomas, one or more skeletal anomalies, and multiple odontogenic keratocysts of the jaw. We report a case of NBCC syndrome in a 43-year old male who had multiple nevoid basal cell carcinomas on the retroauricular area, face, chest wall, and back, which have been present since childhood. Skull x-rays revealed relatively well-circumscribed cystic, radiolucent lesions on bilateral rami of both upper and lower jaws, calcification of the falx cerebri, agenesis of the right coronoid process, and a bifid chin. The cystic lesions were histologically confirmed as keratocysts.
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