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Connective tissue nevi are benign, slightly elevated, well-circumscribed
plaques that are often seen as an isolated skin finding, but can
also be associated with other systemic disease (Table 11-1).
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Synonyms Nevus elasticus, juvenile
elastoma, collagenoma, collagen hamartomas.
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Age Present at birth or childhood.
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Genetics May have an autosomal
dominant inherited form. Buschke-Ollendorf syndrome has mutation
in the LEMD3 gene.
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Connective tissue nevi are localized malformations of dermal
collagen and/or elastic fibers.
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Connective tissue nevi appear in childhood or adolescence and
are asymptomatic but can be disfiguring.
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Type Slightly raised plaque (Fig.
11-1).
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Color Flesh-colored to yellow.
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Size Few millimeters to several
centimeters.
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Number Solitary or multiple.
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Distribution Symmetrically over
abdomen, back, buttocks, arms, thighs.
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Can be associated with systemic disease (Table 11-1).
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Differential
Diagnosis
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Connective tissue nevi can be diagnosed clinically and confirmed
by skin biopsy. They can be confused with other dermal or subcutaneous
processes such as fibromatoses, fibrous hamartoma of infancy, infantile
myofibromatosis, dermatofibromas, lipomas, scars, keloids, pseudoxanthoma elasticum,
or mucopolysaccharidoses.
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Laboratory Examinations
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Dermatopathology Skin biopsy reveals
disorganized collagen and/or elastin fibers. Typically,
there is an increase in collagen and a decrease or normal amount
of elastin. Biopsies of the lesion can be easily mistaken for normal
skin.
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Connective tissue nevi are benign. They persist for life and
can increase in number during pregnancy. They are typically asymptomatic
but can be cosmetically troublesome. The presence of connective
tissue nevi should alert the clinician to check carefully for other
signs of tuberous sclerosis (Table 11-1).
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Treatment for connective tissue nevi is not necessary. Early
recognition and evaluation for possible associated ...