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Brooke–Spiegler Syndrome
Published in Dongyou Liu, Handbook of Tumor Syndromes, 2020
Trichoepithelioma is a benign neoplasm with follicular differentiation and may exist in a solitary or familial form. The solitary form occurs in the head and neck region as well as any portion of hair bearing skin, while the familial/multiple form emerges in adolescence or adulthood and shows a predilection for central facial distribution [5].
Trichoepithelioma
Published in Longo Caterina, Diagnosing the Less Common Skin Tumors, 2019
Trichoepithelioma is an uncommon benign tumor deriving from the hair germ, which may be mainly seen in adults as a solitary skin-colored papule, up to 0.5 cm in diameter, and mainly located on the nose, upper lip and cheeks.1 Besides the less uncommon solitary form, two other variants have been described: multiple and desmoplastic. The latter is now considered as a distinct clinicopathologic entity with specific histopathologic features and is discussed separately in Chapter 17; on the contrary, multiple trichoepitheliomas are identical to the solitary form on histopathologic examination but typically involve younger individuals with an autosomal dominant inheritance. In most of these cases a mutation in the CYLD gene may be found, which is a tumor-suppressor gene located on chromosome 16q12–q13.2–4 Different mutations in this gene have been reported to produce three distinct phenotypic variants. In multiple familial trichoepitheliomas (Online Mendelian Inheritance in Man [OMIM] 601,606), only multiple trichoepitheliomas can be found; in Brooke–Spiegler syndrome (OMIM 605,041), multiple trichoepitheliomas are associated with cylindromas and spiradenomas; in familial cylindromatosis (OMIM 132,700), which is the rarest condition, only multiple cylindromas are present (see Chapter 26).5 On histopathologic examination, trichoepithelioma appears as a well-demarcated dermal tumor composed of nests of uniform basaloid (hair germ) cells, in which hair germ/papilla and bulbar differentiation are present. In addition, there are usually branching nests of basaloid cells and the presence of infundibulocystic structures. The stroma is loosely arranged and contains fibroblasts occasionally aggregated in abortive papillary-mesenchymal bodies.6 The main differential diagnosis of trichoepithelioma involves basal cell carcinoma (BCC) and other adnexal tumors, in particular, trichoadenoma and trichoblastoma.1
Efficacy and safety of lasers in treating syringomas: a review of the literature
Published in Journal of Dermatological Treatment, 2022
Syringomas are benign adnexal neoplasms caused by an overgrowth of cells from sweat glands of eccrine origin (1). These lesions are small, multiple, asymptomatic, smooth, firm, skin-to-yellow-colored papules that frequently develop in clusters in a bilateral, symmetrical distribution (2). Based on clinical features and associations, Friedman and Butler classified syringomas as a localized form, a familial form, a type linked with Down syndrome, and a generalized form with numerous and eruptive syringomas (3). Syringomas are predominantly present around the periorbital region, especially the lower eyelids and are less prevalent on the forehead, scalp, cheeks, abdomen, extremities, axilla, buttocks, and genitalia (4). Although the appearance of syringomas is most frequent in the third and fourth decades of life, studies have also shown that they can occur in any age group before or after puberty (5). Syringomas are more common in women (6) and patients with Down syndrome (7). The female preponderance may be explained by the influence of hormones. The racial predilection for syringoma is not widely reported. However, eruptive syringomas are thought to be more common in African Americans and Asians (8). The mechanism for the formation of the lesions in syringoma is largely unknown. Clinical presentation of eruptive syringomas can be confused with acne vulgaris, milia, sebaceous hyperplasia, lichen planus, urticaria pigmentosa, hidrocystoma, and eruptive xanthoma on the face. Further, syringomas on the face may resemble sclerosing basal cell carcinoma and desmoplastic trichoepithelioma, while lesions on the eyelids may resemble xanthelasma. Syringomas have specific histopathological features, hence histological investigation can be used to provide a definitive diagnosis (2).
Carbon dioxide laser ablation for trichoepitheliomas: The largest reported series
Published in Journal of Cosmetic and Laser Therapy, 2018
Khushboo Sinha, Raj Mallipeddi, Nisith Sheth, Firas Al-Niaimi
At six months, four patients required more than a single session of treatment. It is important to note that these patients had a larger distribution of trichoepithelioma to begin with, and therefore, the treatment is performed in two stages in selected cases. In terms of outcome, all nine patients expressed a high level of satisfaction in terms of cosmetic outcome at year one.
Morpheaform basal cell carcinoma of the nasal ala associated with multiple familial trichoepithelioma reconstructed by anterolateral thigh flap: a case report
Published in Case Reports in Plastic Surgery and Hand Surgery, 2023
Masakatsu Hihara, Yuuki Kouchi, Tsugumi Takao, Maako Fujita, Natsuko Kakudo
Multiple familial trichoepithelioma, a disease caused by a genetic mutation, extremely rarely co-occurs with malignant tumor (e.g. basal cell carcinoma [BCC]). We report a case in which morpheaform BCC with extensive defects in the nasal ala was constructed using a free anterolateral thigh (ALT) flap.