Connective Tissue Nevus 

  • Author: Steven Brett Sloan, MD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Apr 9, 2010
 

Background

Connective tissue nevi are hamartomas in which one or several components of the dermis is altered. Lesions in which collagen predominates are called collagenomas; lesions in which elastin predominates are called elastomas. A nevus mucinosis is a lesion in which an alteration in the amount of dermal glycosaminoglycan is present. The name nevus mucinosis is also used for lesions in which an alteration in more than one dermal component is present.

Connective tissue nevi may be solitary or multiple, sporadic or inherited. They may occur as isolated skin lesions, or they may be associated with a number of syndromes. One report described a collagenoma that occurred on the bulbar conjunctiva.[1]

Zosteriform connective tissue nevus is considered to be a separate entity because of its distribution and histopathologic characteristics.

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Pathophysiology

The cause of connective tissue nevi is unknown. However, note that osteopoikilosis with or without the skin manifestations of Buschke-Ollendorf syndrome and with or without melorheostosis can be caused by heterozygosity for loss-of-function mutations in LEMD3,[2] also called MAN1, which encodes an inner nuclear membrane protein.

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Epidemiology

Frequency

International

The prevalence of connective tissue nevus is rare worldwide.

Mortality/Morbidity

Connective tissue nevi are benign skin lesions.

Race

No racial predilection has been reported for connective tissue nevi.

Sex

No sexual predilection is described for connective tissue nevus.

Age

The age of onset of a connective tissue nevus depends on the type of lesion.

  • Collagenomas and elastomas generally present during the postpubertal period.
  • In Buschke-Ollendorf syndrome, the skin changes may be delayed until adulthood.
  • Nevus mucinosis may present at birth, during childhood, or in adolescence.
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Contributor Information and Disclosures
Author

Steven Brett Sloan, MD  Assistant Professor, Department of Dermatology, University of Connecticut School of Medicine; Director of Nail Disease Clinic and Chief of Dermatology, Newington Veterans Affairs Medical Center

Steven Brett Sloan, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, Connecticut State Medical Society, New England Dermatological Society, and Texas Dermatological Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Daniel J Hogan, MD  Clinical Professor of Internal Medicine (Dermatology), NOVA Southeastern University; Investigator, Hill Top Research, Florida Research Center

Daniel J Hogan, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Contact Dermatitis Society, and Canadian Dermatology Association

Disclosure: Nothing to disclose.

Richard P Vinson, MD  Assistant Clinical Professor, Department of Dermatology, Texas Tech University School of Medicine; Consulting Staff, Mountain View Dermatology, PA

Richard P Vinson, MD is a member of the following medical societies: American Academy of Dermatology, Association of Military Dermatologists, Texas Dermatological Society, and Texas Medical Association

Disclosure: Nothing to disclose.

Paul Krusinski, MD  Director of Dermatology, Professor, Department of Internal Medicine, Fletcher Allen Health Care, University of Vermont

Paul Krusinski, MD is a member of the following medical societies: American Academy of Dermatology, American College of Physicians, and Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Joel M Gelfand, MD, MSCE  Medical Director, Clinical Studies Unit, Assistant Professor, Department of Dermatology, Associate Scholar, Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania

Joel M Gelfand, MD, MSCE is a member of the following medical societies: Society for Investigative Dermatology

Disclosure: AMGEN Consulting fee Consulting; AMGEN Grant/research funds Investigator; Genentech Grant/research funds investigator; Centocor Consulting fee Consulting; Abbott Grant/research funds investigator; Abbott Consulting fee Consulting; Novartis investigator; Pfizer Grant/research funds investigator; Celgene Consulting fee DMC Chair; NIAMS and NHLBI Grant/research funds investigator

Chief Editor

Dirk M Elston, MD  Director, Department of Dermatology, Geisinger Medical Center

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

References
  1. Zarate JO, Pelayes DE, Gioino JM, Piantoni GR. [Giant cell collagenoma of the bulbar conjunctiva]. Arch Soc Esp Oftalmol. Apr 2007;82(4):233-5. [Medline].

  2. Hellemans J, Preobrazhenska O, Willaert A, et al. Loss-of-function mutations in LEMD3 result in osteopoikilosis, Buschke-Ollendorff syndrome and melorheostosis. Nat Genet. Nov 2004;36(11):1213-8. [Medline].

  3. Xia Y, Darling TN. Rapidly growing collagenomas in multiple endocrine neoplasia type I. J Am Acad Dermatol. May 2007;56(5):877-80. [Medline].

  4. Al-Daraji WI, Ramsay HM, Ali RB. Storiform collagenoma as a clue for Cowden disease or PTEN hamartoma tumour syndrome. J Clin Pathol. Jul 2007;60(7):840-2. [Medline].

  5. Twede JV, Turner JT, Biesecker LG, Darling TN. Evolution of skin lesions in Proteus syndrome. J Am Acad Dermatol. May 2005;52(5):834-8. [Medline].

  6. McClung AA, Blumberg MA, Huttenbach Y, Colome-Grimmer MI, Raimer SS. Development of collagenomas during pregnancy. J Am Acad Dermatol. Aug 2005;53(2 Suppl 1):S150-3. [Medline].

  7. Thappa DM, Singh A, Jaisankar TJ, Rao R, Ratnakar C. Pebbling of the skin: a marker of Hunter's syndrome. Pediatr Dermatol. Sep-Oct 1998;15(5):370-3. [Medline].

  8. de Almeida HL Jr, Breunig Jde A, Wolter M, de Castro LA, Rocha NM. Light and electron microscopy of eruptive collagenoma. J Cutan Pathol. Oct 2009;36 Suppl 1:35-8. [Medline].

  9. Sardana K, Bansal S, Garg VK, Khurana N. Linear nodular collagenoma--successful treatment with intralesional triamcinolone acetonide. Pediatr Dermatol. Sep-Oct 2009;26(5):626-8. [Medline].

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Familial cutaneous collagenoma.
Shagreen patch.
Collagenoma
 
 
 
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