Steatocystoma multiplex with hair shaft abnormalities

Abstract

Steatocystoma multiplex (SM) is an unusual benign disorder of the pilosebaceous duct characterized by multiple cysts with little or no nail and hair involvement. We report a 30-year-old woman with multiple cystic nodules located on the neck, axillae and forearms as well as patchy scalp alopecia. Histopathological examination of the lesions was diagnostic of SM. Trichoscopy revealed pili torti and pili canaliculi. This patient represents an unusual clinical presentation of SM because of the presence of hair abnormalities.

Patient Characteristics, Locations and Histopathological Features of Steatocystoma Multiplex Cases

“…It is an unusual benign disorder that characterized by the development of numerous sebum containing dermal cysts. It is an autosomal dominant inherited disorder, but most cases are sporadic (Cho et al, 2002;Varshney et al, 2011;Gordon et al, 2013;Surej et al, 2014;Pietrzak et al, 2015).…”

“…Lesions are most commonly found on the chest and arms (proximal extremities). Although other sites are also described such as; axillae, neck, abdomen, scalp, vulva and inguinal region (common in women) (Cho et al, 2002;Varshney et al, 2011;Gordon et al, 2013;Surej et al, 2014;Pietrzak et al, 2015;Kromann et al, 2015). However, they can be occur anywhere and have been reported on the oral mucosa and in the subdermal tissue (Kaiser et al, 2016).…”

“…Steatocystoma multiplex (SM) is a rare benign genetic disorder consisted of multiple cystic with little or no nail and hair involvement (Gordon et al, 2013;Pietrzak at al., 2015). It is a hamartomatous malformation of the pilosebaceous unit consisting of dermal cysts filled with a sebum-like material (Cho et al, 2002;Surej et al, 2014;Krishnegowda et al, 2015;Santana et al, 2016).…”

“…It is a hamartomatous malformation of the pilosebaceous unit consisting of dermal cysts filled with a sebum-like material (Cho et al, 2002;Surej et al, 2014;Krishnegowda et al, 2015;Santana et al, 2016). Mutations in the Keratin 17 gene are considered in its pathogenesis (Cho et al, 2002;Gordon et al, 2013;Surej et al, 2014;Pietrzak et al, 2015;Krishnegowda et al, 2015;Kromann et al, 2015;Santana et al, 2016). SM lesions are typically located in areas with sebaceous follicles, although atypical presentations involving sites lacking sebaceous follicles have exceptionally been described (Kromann et al, 2015).…”

Steatocystoma Multiplex Suppurativa: A Case with Unusual Giant Cysts over the Scalp and Neck

“…Characteristically, it presents with multiple skin-colored dermal cysts on the trunk, axilla, groin, and proximal extremities [1]. In the literature, however, there are rare SM presentations reported for the breasts, face [15, 16], and scalp [17, 18], but in most of these atypical cases, the lesions were localized and limited to a single anatomical location.…”