BECKER’S NEVUS (SMOOTH MUSCLE HAMARTOMA) - pediagenosis
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Thursday, October 18, 2018

BECKER’S NEVUS (SMOOTH MUSCLE HAMARTOMA)


BECKER’S NEVUS (SMOOTH MUSCLE HAMARTOMA)
Becker’s nevi most commonly appear on the shoulder or upper limb girdle of prepubescent boys. It is a rather common benign condition that is seen in up to 0.5% of the male population. It is less commonly seen in females. Becker’s nevi are acquired nevi. Most occur before 10 years of age. Becker’s nevus is classified as a smooth muscle hamartoma. It does not contain melanocytic nevus cells and is not considered to be a melanocytic nevus. It was given its name by the dermatologist Samuel Becker, who first described this condition.

BECKER’S NEVUS (SMOOTH MUSCLE HAMARTOMA)

Clinical Findings: Becker’s nevi begin as ill-defined, slightly hyperpigmented macules on the upper limb girdle. Over time (1 year, on average), the hyperpigmented region develops hypertrichosis, resulting in its characteristic appearance. Backer’s nevi may occur anywhere on the human body, but by far the most common locations are on the shoulder, upper chest, and back. The area of hypertrichosis is limited to the underlying hyperpigmented area. The clinical significance of Becker’s nevi is its differentiation from large congenital nevi and café-au-lait macules. Becker’s nevi confer no increased risk for development of melanoma, and they are rarely associated with any underlying abnormalities. The most common underlying abnormality is unilateral hypoplasia of the breast, which has minimal clinical significance. Rarely, a patient with a Becker’s nevus has underlying hypoplasia of bone and soft tissue, the cause of which is unknown. The differential diagnosis includes a giant congenital nevus and a café-aulait macule. These two conditions should be easily differentiated from Baker’s nevus, because they both are typically apparent at birth or soon there- after, whereas Becker’s nevi are typically acquired at about the age of 10 years.
The diagnosis is typically made on clinical findings, but a skin biopsy is sometime needed to confirm the diagnosis if the nevus is in an unusual anatomical location. The punch biopsy is the best method for obtaining tissue.

Histology: The biopsy specimen shows a smooth muscle hamartoma. Multiple smooth muscle fascicles are seen within the dermis. There is an increased ratio of terminal to vellus hairs and a lack of melanocytic nevus cells. The hyperpigmentation results from increased formation of pigmentation within the melanocytes of the stratum basalis. There is no increase in the number of melanocytes. Varying amounts of acanthosis and hyperkeratosis are seen.

Pathogenesis: The pathogenesis of Becker’s nevus is unclear. It is believed to be caused by the dermal presence of hamartomatous smooth muscle tissue. Research has shown that the tissue in Becker’s nevi has an increased number of androgen receptors. It is thought that increased androgen levels at puberty interact with the excessive androgen receptors and cause the clinical findings.
Becker’s nevus is the most common type of smooth muscle hamartoma in the skin. Smooth muscle hamartomas by themselves are rarely found within the skin.
Non-Becker’s smooth muscle hamartomas are usually present at birth or soon thereafter and manifest as a small, flesh-colored plaque located anywhere on the body. All smooth muscle hamartomas may at some point exhibit the pseudo-Darier’s sign. To clinically elicit this sign, one gently rubs the smooth muscle hamartoma; the lesion may fasciculate due to smooth muscle activity, or the region may develop an urticarial appearance. This sign has nothing to do with histamine release; rather, it is caused by a neurally mediated con- traction of the underlying hamartomatous smooth muscle tissue.

Treatment: No therapy is required. Surgical excision is likely to produce a mutilating scar unless the nevus is extraordinarily small. The hypertrichosis can be treated for cosmetic purposes with any of a multitude of therapies including laser removal, shaving, and electrolysis. Most patients prefer to not treat the area.

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