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Connective tissue nevi in Child
Other Resources UpToDate PubMed

Connective tissue nevi in Child

Contributors: Ahmet Yildirim MEd, Erica Ghareeb MD, William Farmer MD, Susan Burgin MD
Other Resources UpToDate PubMed


Connective tissue nevi (CTN) are hamartomas that consist of excessive production of normal tissue, such as collagen (collagenomas), elastin (elastomas), or both, glycosaminoglycans (nevus mucinosis), and fibroblasts (fibroblastic CTN). CTN may be present at birth or arise during childhood and can be found anywhere on the body. They are usually hereditary but may be acquired.

CTN may be isolated or occur as part of a syndrome, such as tuberous sclerosis (collagenomas known as shagreen patches and fibrous forehead plaque), familial cutaneous collagenomas, Proteus syndrome (cerebriform connective tissue nevus), and Buschke-Ollendorff syndrome (elastomas known as dermatofibrosis lenticularis disseminata). Acquired collagenoma (athlete's nodules) may occur at sites of sporting injuries, such as the feet, knuckles, or knees.

CTN have a variable clinical appearance. They may appear as skin-colored, light brown, or yellowish papules or plaques with a smooth, cobblestone, or peau d'orange surface.


D23.9 – Other benign neoplasm of skin, unspecified

400091006 – Connective tissue nevus of skin

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

  • Juvenile fibromatosis
  • Fibrous hamartoma of infancy
  • Infantile myofibromatosis
  • Mastocytoma – Will urticate when stroked.
  • Lipoma – Typically softer than CTN.
  • Morphea
  • Pseudoxanthoma elasticum
  • Nevus sebaceus
  • Nevus lipomatosus superficialis
  • Verruciform xanthoma
Dermatofibrosis lenticularis disseminata:
  • See differential diagnosis of collagenoma above.
  • Dermatofibroma
  • Eruptive vellus hair cyst
  • Osteoma cutis
  • Calcinosis cutis
Mucinous nevus:
  • Nevus lipomatosus superficialis
  • Mucin deposition in Hunter syndrome
  • Lymphangioma circumscriptum
Fibroblastic CTN:
  • Dermatofibroma
  • Morphea
  • Dermatomyofibroma
  • Dermatofibrosarcoma protuberans(DFSP)

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Last Reviewed:05/24/2021
Last Updated:06/01/2021
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Connective tissue nevi in Child
A medical illustration showing key findings of Connective tissue nevi : Chronic duration lasting years, Trunk
Clinical image of Connective tissue nevi - imageId=262620. Click to open in gallery.  caption: 'A large, oval, pink plaque on the lower back.'
A large, oval, pink plaque on the lower back.
Copyright © 2024 VisualDx®. All rights reserved.