Keratosis Pilaris and its Subtypes: Associations, New Molecular and Pharmacologic Etiologies, and Therapeutic Options

Am J Clin Dermatol. 2018 Oct;19(5):733-757. doi: 10.1007/s40257-018-0368-3.


Keratosis pilaris is a common skin disorder comprising less common variants and rare subtypes, including keratosis pilaris rubra, erythromelanosis follicularis faciei et colli, and the spectrum of keratosis pilaris atrophicans. Data, and critical analysis of existing data, are lacking, so the etiologies, pathogeneses, disease associations, and treatments of these clinical entities are poorly understood. The present article aims to fill this knowledge gap by reviewing literature in the PubMed, EMBASE, and CINAHL databases and providing a comprehensive, analytical summary of the clinical characteristics and pathophysiology of keratosis pilaris and its subtypes through the lens of disease associations, genetics, and pharmacologic etiologies. Histopathologic, genomic, and epidemiologic evidence points to keratosis pilaris as a primary disorder of the pilosebaceous unit as a result of inherited mutations or acquired disruptions in various biomolecular pathways. Recent data highlight aberrant Ras signaling as an important contributor to the pathophysiology of keratosis pilaris and its subtypes. We also evaluate data on treatments for keratosis pilaris and its subtypes, including topical, systemic, and energy-based therapies. The effectiveness of various types of lasers in treating keratosis pilaris and its subtypes deserves wider recognition.

Publication types

  • Review

MeSH terms

  • Abnormalities, Multiple / diagnosis
  • Abnormalities, Multiple / etiology
  • Abnormalities, Multiple / pathology
  • Abnormalities, Multiple / therapy*
  • Administration, Cutaneous
  • Darier Disease / diagnosis
  • Darier Disease / etiology
  • Darier Disease / pathology
  • Darier Disease / therapy*
  • Dermabrasion / methods*
  • Dermatitis, Atopic / complications
  • Dermatologic Agents / therapeutic use*
  • Diagnosis, Differential
  • Eyebrows / abnormalities*
  • Eyebrows / pathology
  • Filaggrin Proteins
  • Humans
  • Ichthyosis / complications
  • Ichthyosis / genetics
  • Intermediate Filament Proteins / genetics
  • Mutation
  • Phototherapy / methods*
  • Signal Transduction / genetics
  • Skin / pathology
  • Treatment Outcome
  • ras Proteins / genetics
  • ras Proteins / metabolism


  • Dermatologic Agents
  • Filaggrin Proteins
  • Intermediate Filament Proteins
  • ras Proteins

Supplementary concepts

  • Burnett Schwartz Berberian syndrome