title: Folliculocystic and collagen hamartoma of tuberous sclerosis complex.
abstract: BACKGROUND
  Tuberous sclerosis complex (TSC) is an autosomal dominant disorder characterized by tumors and hamartomas in several organs including the skin.
  
  
  OBJECTIVE
  We sought to describe a new type of complex hamartoma in patients with TSC.
  
  
  METHODS
  This was a retrospective clinical and histopathologic evaluation of 6 cases.
  
  
  RESULTS
  The skin lesions consisted of large, painless, infiltrated plaques that were first noticed at birth or during early infancy on the abdomen, thigh, back, or scalp. In time, the plaques became studded with numerous follicular comedo-like openings and cysts containing and draining a keratinous or purulent material. The main histopathologic features were: abundant collagen deposition in the dermis and extending into the underlying fat; concentric, perifollicular fibrosis surrounding hair follicles; and comedones and keratin-containing cysts lined by infundibular epithelium, some of which were ruptured with secondary granulomatous reaction. Five of the 6 patients had a clinical diagnosis of TSC.
  
  
  CONCLUSIONS
  Genetic testing was performed in only one patient.
  
  
  CONCLUSIONS
  This distinctive folliculocystic and collagen hamartoma has not been recognized previously in association with TSC.
authors.affiliation: Department of Dermatology, Hospital del Niño Jesús, Madrid, Spain. atorrelo@aedv.es
authors.forename: Antonio
authors.initials: A
authors.lastname: Torrelo
authors.forename: Smail
authors.initials: S
authors.lastname: Hadj-Rabia
authors.forename: Isabel
authors.initials: I
authors.lastname: Colmenero
authors.forename: Robert
authors.initials: R
authors.lastname: Piston
authors.forename: Virginia P
authors.initials: VP
authors.lastname: Sybert
authors.forename: Helena
authors.initials: H
authors.lastname: Hilari-Carbonell
authors.forename: Angela
authors.initials: A
authors.lastname: Hernández-Martín
authors.forename: Joan C
authors.initials: JC
authors.lastname: Ferreres
authors.forename: Sergio
authors.initials: S
authors.lastname: Vañó-Galván
authors.forename: Daniel
authors.initials: D
authors.lastname: Azorín
authors.forename: Javier Enríquez
authors.initials: JE
authors.lastname: de Salamanca
authors.forename: Luis
authors.initials: L
authors.lastname: Requena
authors.forename: Christine
authors.initials: C
authors.lastname: Bodemer
authors.forename: Rudolf
authors.initials: R
authors.lastname: Happle
authors.forename: Vicente
authors.initials: V
authors.lastname: García-Patos
authors.forename: Sylvie
authors.initials: S
authors.lastname: Fraitag
mesh_terms: D003094:Collagen / Q000096:biosynthesis; D006222:Hamartoma / Q000209:etiology* / Q000378:metabolism / Q000473:pathology*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D012189:Retrospective Studies; D012871:Skin Diseases / Q000378:metabolism / Q000473:pathology*; D014402:Tuberous Sclerosis / Q000150:complications*
chemical_list: D003094:Collagen