Multiple familial trichoepithelioma with malignant transformation

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چکیده

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Malignant transformation of multiple familial trichoepithelioma: case report and literature review.

Patients with the autosomal-dominant form of multiple familial trichoepithelioma develop numerous tumours on the face, neck and upper trunk, beginning in childhood. Malignant transformation of such lesions is quite rare; only one case of "malignant trichoepithelioma" has been reported previously, inferring pilomatrix carcinoma on a histological observation. We report here the case of a patient ...

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Familial Facial disfigurement in Multiple Familial Trichoepithelioma.

Trichoepithelioma is an uncommon, benign hamartomatous tumor of the pilosebaceous follicle. Presenting as multiple papules and nodules on face and neck, they pose a significant cosmetic problem in affected individuals. Familial involvement of this dermatosis occurs in an autosomal dominant pattern, the locus being located on chromosome 9p21, which causes multiple facial lesions in family member...

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Multiple familial trichoepithelioma: confirmation via dermoscopy

Trichoepitheliomas are uncommon benign adnexal neoplasms that originate from the hair follicles. Multiple familial trichoepithelioma constitute an autosomal dominant disease characterized by the appearance of multiple flesh-colored, symmetrical papules, tumors and/or nodules in the central face and occasionally on the scalp. Although clinical diagnosis is usually straightforward in light of the...

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A novel missense mutation in the CYLD gene in a Spanish family with multiple familial trichoepithelioma.

B rooke-Spiegler syndrome (BSS) includes the combination of spiradenomas, cylindromas, and trichoepitheliomas. It has been postulated that BSS results from defects in the regulation of putative stem cells of the folliculosebaceous-apocrine unit. This follicular dysregulation may give rise to 3 different genodermatoses: familial cylindromatosis (FC), multiple familial trichoepithelioma (MFT), or...

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Trichoepithelioma: A Rare but Crucial Dermatologic Issue

BACKGROUND Trichoepithelioma is a rare benign skin lesion that originates from hair follicles. Trichoepitheliomas are mostly seen in the scalp, nose, forehead, and upper lip. METHODS We present a large family of Iranian origin with 15 individuals affected with multiple familial trichoepithelioma in four generations, and treated with three different methods. RESULTS Trichoepithelioma is his...

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ژورنال

عنوان ژورنال: Indian Journal of Dermatology

سال: 2013

ISSN: 0019-5154

DOI: 10.4103/0019-5154.117348