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      Epithelioma Adenoides Cysticum: A Report of Two Cases in a Family

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          Abstract

          Epithelioma adenoides cysticum or multiple trichoepitheliomas are rare benign hamartomas arising from the hair germ. A 35-year-old female presented with multiple skin-colored firm papules and nodules mainly affecting the central face, scalp, and external ear canal since the age of 9 years. The lesions gradually increased with age. Her 13-year-old son also had similar but smaller lesions on the central face. Histopathology was consistent with trichoepithelioma.

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          Most cited references 5

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          The gene for multiple familial trichoepithelioma maps to chromosome 9p21.

          Multiple familial trichoepithelioma (MFT) is an autosomal dominant skin disease characterized by the presence of many small tumors predominantly on the face. To map the causative gene, we performed linkage analysis with microsatellite markers in three American families. We found a significant linkage of a gene for MFT to chromosome 9p2l. The maximum combined lod score was 3.31 at D9S171 at theta = 0. The disease locus was defined to a 4-cM region between IFNA and D9S126. Because several tumor suppressor genes including p16 and p15 have been mapped to this region, the gene for MFT may also be a tumor suppressor.
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            Epithelioma adenoides cysticum and solitary trichoepithelioma.

             R. Gray,  E B HELWIG (1962)
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              Multiple hereditary trichoepitheliomas and basal cell carcinomas.

              A 45-year-old man with multiple hereditary trichoepitheliomas developed 2 tumors which showed malignant behavior. These tumors contained some undifferentiated basal cell carcinoma-like areas, but also some regions with a high degree of follicular differentiation. In addition he had several small tumors with predominantly undifferentiated features interspersed with the trichoepitheliomas. The relationship between multiple hereditary trichoepitheliomas and basal cell carcinomas is reviewed.
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                Author and article information

                Journal
                Int J Trichology
                Int J Trichology
                IJT
                International Journal of Trichology
                Medknow Publications & Media Pvt Ltd (India )
                0974-7753
                0974-9241
                Oct-Dec 2016
                : 8
                : 4
                : 165-167
                Affiliations
                Department of Dermatology, Medical College, Calicut, Kerala, India
                [1 ]Department of Dermatology, Palakkad Medical College, Palakkad, Kerala, India
                [2 ]Department of Pediatrics, Medical College, Calicut, Kerala, India
                Author notes
                Address for correspondence: Dr. Bindurani Sudhamani, Vrindhavan House, Near Nehru Auditorium, Chittur PO, Palakkad, Kerala, India. E-mail: drbindurani@ 123456gmail.com
                Article
                IJT-8-165
                10.4103/0974-7753.203177
                5387875
                Copyright: © 2017 International Journal of Trichology

                This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 3.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.

                Categories
                Case Report

                Dermatology

                brooke's tumor, epithelioma adenoides cysticum, trichoepithelioma

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