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The papillary and reticular dermis are the two layers of the dermis. They are made of connective tissue with fibers of collagen extending from one to the other, making the border between the two somewhat indistinct. The dermal papillae extending into the epidermis belong to the papillary layer, whereas the dense collagen fiber bundles below belong to the reticular layer.
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Connective tissues are one of the four main tissue types in humans that are extensively present in the body. They are characterized by cells embedded in an extracellular matrix (ECM) composed of a ground substance and three main types of protein fibers— collagen, elastic, and reticular fibers. The ground substance of connective tissues can range from a watery and jelly-like consistency to mineralized and hard. The wide variety of cells in the connective tissues include fibroblasts,...
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[Extensive connective tissue nevus in children].

O Lafargue1, S Fraitag2, O Boccara3

  • 1Service de dermatologie, CHU de Caen, avenue de la Côte-de-Nacre, CS 30001, 14033 Caen cedex 9, France.

Annales De Dermatologie Et De Venereologie
|August 2, 2017
PubMed
Summary

Connective tissue nevus (CTN) is a rare skin condition. This report details two pediatric cases of extensive CTN, highlighting diagnostic considerations and potential complications like joint stiffening.

Keywords:
ChildrenConnective tissue nevusEnfantHamartome conjonctif

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Area of Science:

  • Dermatology
  • Genetics
  • Pathology

Background:

  • Connective tissue nevus (CTN) is a rare disorder affecting extracellular matrix components.
  • It typically presents as papules of normal skin color and can be syndromic or sporadic.

Observation:

  • Two pediatric cases of extensive and infiltrative CTN were reported.
  • Pathology revealed mixed hamartoma and collagenoma in these isolated cases.

Findings:

  • Early CTN onset is often subtle and difficult to ascertain.
  • Extensive CTN requires differentiation from eosinophilic fasciitis and morphea via skin biopsy.
  • CTN can be associated with Buschke-Ollendorf syndrome, characterized by osteopoikilosis detected via X-ray.

Implications:

  • Prompt diagnosis and differentiation are crucial for managing extensive CTN.
  • Understanding CTN's association with skeletal abnormalities aids in comprehensive patient assessment.
  • Symptomatic care is the primary management strategy for CTN and associated skeletal findings.