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