title: Familial acral localized late-onset focal dermal elastosis.
abstract: Late-onset focal dermal elastosis is a rare disorder of elastic tissue, characterized by a local accumulation of elastic fibers in the mid and deep reticular dermis. This disorder occurs mainly in elderly people and consists of multiple yellow papules that are typically distributed on the sides of the neck and flexural areas, closely resembling pseudoxanthoma elasticum from the clinical point of view. We report 2 sisters who have presented with a 20-year history of multiple yellow papules, mainly located on the dorsum of their hands. They also had few lesions of similar morphology scattered on the anterior aspects of their wrists, thighs, and lower abdomen. Histopathologic study demonstrated a focal increase in thick, interlacing elastic fibers in the mid and deep reticular dermis. The most striking features of our cases were the early age of clinical presentation, the familial involvement, and the predominantly acral distribution of the lesions.
authors.affiliation: Department of Dermatology, Fundación Jiménez Díaz, Universidad Autónoma, Madrid, Spain.
authors.forename: Diana
authors.initials: D
authors.lastname: Camacho
authors.forename: Salma
authors.initials: S
authors.lastname: Machan
authors.forename: Ursula
authors.initials: U
authors.lastname: Pielasinski
authors.forename: Juan María
authors.initials: JM
authors.lastname: Revelles
authors.forename: María
authors.initials: M
authors.lastname: del Carmen Fariña
authors.forename: Carlos
authors.initials: C
authors.lastname: Santonja
authors.forename: Luis
authors.initials: L
authors.lastname: Requena
mesh_terms: D004547:Elastic Tissue / Q000473:pathology*; D005121:Extremities; D005260:Female; D006801:Humans; D008875:Middle Aged; D011561:Pseudoxanthoma Elasticum / Q000175:diagnosis* / Q000235:genetics; D035781:Siblings; D012871:Skin Diseases / Q000175:diagnosis* / Q000235:genetics