Connective tissue nevus

Connective tissue nevi are hamartomatous malformations of the skin containing elements of dermal connective tissue. These elements include collagen, elastin, and glycosaminoglycans.

Lesions with a predominantly collagen makeup may occur as familial multiple connective tissue nevi, as the shagreen patch seen in tuberous sclerosus, or as knuckle pads.

Elastic tissue predominates in connective tissue nevi associated with pseudoxanthoma elasticum (the Buschke-Ollendorf syndrome) and perforating elastoma.

Ground substance accumulates in the dermal nodules of Hunter's syndrome.

Other lesions showing connective tissue proliferation are fibrous papule of the nose, periungual fibromas associated with tuberous sclerosis and pearly papules of the penis.

Treatment:

• If symptomatic, lesions can be surgically excised.
• It is important to examine the patient for an associated syndrome.

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