Connective Tissue Nevi: A Review of the Literature

Am J Dermatopathol. 2017 May;39(5):325-341. doi: 10.1097/DAD.0000000000000638.


Connective tissue nevi (CTN) are hamartomas of the dermis, with the 3 main components being collagen, elastin, and proteoglycans. Each subtype can present as a solitary lesion or multiple lesions. They could present as part of systemic diseases or inherited disorders. This article provides a comprehensive literature review of the different types of CTN, their clinical presentations, associations, and treatment options. Treatment options for 56 lesions were reviewed. Fifty-two percent of lesions were present in males, and the age range at the time of presentation was wide (1.6-80 years). Management varied according to CTN subtypes. Most lesions (14) received topical or intralesional treatment with corticosteroids, followed by surgical removal of lesions (12), whereas the remaining lesions were clinically monitored.

Publication types

  • Review

MeSH terms

  • Biopsy, Needle
  • Connective Tissue Diseases / diagnosis
  • Connective Tissue Diseases / pathology*
  • Connective Tissue Diseases / therapy
  • Cryotherapy / methods
  • Female
  • Humans
  • Immunohistochemistry
  • Injections, Intralesional
  • Male
  • Nevus / diagnosis
  • Nevus / pathology*
  • Nevus / therapy
  • Prognosis
  • Rare Diseases
  • Risk Assessment
  • Skin Neoplasms / diagnosis
  • Skin Neoplasms / pathology*
  • Treatment Outcome
  • Triamcinolone / therapeutic use*


  • Triamcinolone

Supplementary concepts

  • Connective Tissue Nevus