Genetics of skin appendage neoplasms and related syndromes

Brooke-Spiegler syndrome

“…Spiradenomas may transform into spiradenocarcinomas or show sarcomatous differentiation. An increased risk of developing tumors of the salivary glands has also been described (basalcell adenomas and adenocarcinomas of the parotid glands and minor salivary glands) (Lee et al, 2005;Kim et al, 2007;Blake and Toro, 2009). …”

“…the folliculo-sebaceous-apocrine germ). They are small skin-colored papules or nodules, with nests of basaloid cells forming cysts containing horn cells (with keratin) (Lee et al, 2005;Kim et al, 2007;Blake and Toro, 2009). …”

Bazex-Dupré-Christol syndrome (BDCS)

“…the folliculo-sebaceous-apocrine germ). They are small skin-colored papules or nodules, with nests of basaloid cells forming cysts containing horn cells (with keratin) (Lee et al, 2005). …”

Inherited skin tumour syndromes

“…3 Fibrofolliculomas and trichodiscomas clinically present as skin/yellow-white coloured dome shaped papules 2-4 mm in diameter (Fig 1 a and Fig 1 b). 4 These lesions usually develop in the third or fourth decade. 4 In the case of fibrofolliculoma, hair specific differentiation is seen, whereas in the case of trichodiscoma, differentiation is to the mesodermal component of the hair disc (Fig 1 c and Fig 1 d) .…”

“…4 These lesions usually develop in the third or fourth decade. 4 In the case of fibrofolliculoma, hair specific differentiation is seen, whereas in the case of trichodiscoma, differentiation is to the mesodermal component of the hair disc (Fig 1 c and Fig 1 d) . 4 Acrochordons are skin tags that were initially described as part of the BHDS; however, they are common in the general population and therefore are not diagnostic of the syndrome.…”