Review of the worlds literature reveals several additional cases of structural abnormalities associated with. Becker nevus syndrome is a phenotype characterised by the simultaneous occurrence of. Becker s nevus is a noncancerous, large, brown birthmark occurring mostly in males. Hypertrichosis is not so prevalent in beckers nevus. The presence of a beckers melanosis may be a heretofore unrecog nized marker for underlying structural anomalies. Becker s melanosis is a relatively common, benign hamartoma, which is usually characterized by a hyperpigmented, hypertrichotic patch on the upper trunk or proximal upper extremities of males, with its onset during the peripubertal years.
Commonly, there is an associated smooth muscle hamartoma on histology. Beckers melanosis is a relatively common, benign hamartoma, which is usually characterized by a hyperpigmented, hypertrichotic patch on the upper trunk or proximal upper extremities of males, with its onset during the peripubertal years. Review of the worlds literature reveals several additional cases of structural abnormalities associated with becker s melanosis. Becker nevus syndrome actas dermosifiliograficas english. A lthough case histories of patients al legedly having primary malignant mela noma of the esophagus have been occasionally reported, 38, 11, 16, 19 in the mind of the. Nras mutation is the sole recurrent somatic mutation in large congenital melanocytic nevi. An otherwise normally developed child who had this hamartoma at birth is described in an attempt to clarify the relationship between pilar smoothmuscle hamartomas and becker s melanosis.
Smooth muscle hamartoma associated with bilateral beckers. Research article hypertrichosis is not so prevalent in becker s nevus. The congenital melanocytic nevus is a type of melanocytic nevus or mole found in infants at birth. In another survey among, school children, the prevalence of becker s nevus was %. Beckers nevus melanosis is usually found in the region of the shoulder girdle of young men as unilateral, hyperpigmented areas of somewhat thickened skin. They hypothesize that these mutations may interfere with hedgehog pathway signaling. Becker s nevus melanosis is usually found in the region of the shoulder girdle of young men as unilateral, hyperpigmented areas of somewhat thickened skin. We herein report a rare case of bilateral, symmetrical, nonsyndromic becker melanosis and we discuss possible pathogenesis and. Beckers nevus syndrome pigmentary hairy epidermal nevus. It develops during childhood or adolescence on the shoulders or upper trunk, occasionally elsewhere. Research article hypertrichosis is not so prevalent in.
It is characterized by peripubertal development, male preponderance, hypertrichosis, occasional development of acneform lesions within the patch, and rare association with accessory scrotum in the genital region. Becker nevus becker nevus also known as becker melanosis or becker pigmentary hamartoma is characterized by a circumscribed, brownish patch with geographical borders and sometimes hypertrichosis. Pdf becker nevus is a hyperpigmented hamartoma with an irregular outline and often hairy. Becker nevus syndrome is also an androgendependent disorder as is becker s nevus and is therefore more commonly found in adolescence due to the increased hyperpigmentation of the nevus and the hair. Since becker s original article, many more cases have been reported. Beckers nevus and lethal betaactin mutations journal of. Their findings have important implications for clinical genetics, and they open new aspects in hair. It is usually located on the shoulders, anterior chest or scapular region.
Pdf beckers melanosis and associated abnormalities. Further studies on the association of beckers nevus and hamartoma formation will be needed. Economia principios y aplicaciones monchon y beker 4ta edicion. Beckers nevus bn is a cutaneous hamartoma characterized by circumscribed hyperpigmentation with hypertrichosis, which was described for the first time by becker in 1949. Thus the pathophysiology of becker s nevus remains unclear. Nord gratefully acknowledges harper price, md, department of dermatology, phoenix childrens hospital, and heather etchevers, phd, national institutes for health and medical research of france inserm, for the preparation of this report. Concurrent melanosis and hypertrichosis in distribution of nevus unius lateris. Mar 24, 2017 beckers nevus is a cutaneous hamartoma affecting approximately 1 in 200 individuals tymen et al. To the editor beckers nevus is a concurrent melanosis and hypertrichosis in the distribution of nevus unius lateris, occurring in the second or third decade of life, mostly in men, quite frequently, no hypertrichosis is seen in the nevus. The purpose of this study was to identify and analyze the common manifestations of the bns. C case 1 a 19yearold east indian man presented with a 2year history of a dark lesion on his chest with excessive hair on its surface. A re view of the worlds literature reveals that this is not an extraordinary finding. Androgen receptor expression patterns in becker s nevi.
We propose that these two entities belong at different poles of the same developmental spectrum of hamartomatous change. While it is generally considered an acquired rather than congenital disorder, there. They proposed that beckers nevus could be considered a component of the androgen receptor hypersensitivity syndrome spectrum. We here report a case of a 15 yearold female who presented with bilateral beckers nevus over her groins, thighs, vulva, and in front of the neck from the age of 5 years. A lthough case histories of patients al legedly having primary malignant mela noma of. Becker nevus syndrome and ipsilateral breast hypoplasia. Androgen receptor expression patterns in beckers nevi. Beckers nevus genetic and rare diseases information center. Concurrent melanosis and hypertrichosis in a distribution of nevus unius lateris. Postzygotic mutations in betaactin are associated with.
Beckers nevus coexistent with connectivetissue nevus. We here report a case of a 15 yearold female who presented with bilateral becker s nevus over her groins, thighs, vulva, and in front of the neck from the age of 5 years. Genotypic and gene expression studies in congenital melanocytic nevi. Beckers nevus coexistent with connectivetissue nevus jama. Becker nevus syndrome, american journal of medical genetics. It can be present at birth, but is usually first noticed around puberty. As becker nevi are generally singular in a given individual, there are very few reported cases of multiple or bilateral lesions. Research article hypertrichosis is not so prevalent in becker. Becker nevus syndrome becker nevus syndrome happle, rudolf.
Becker nevus itself is typically localized on the upper trunk, scapular or upper arm unilaterally. This type of birthmark occurs in an estimated 1% of infants worldwide. Becker nevus syndrome refers to a rare disorder comprising the typical pigmented lesion and its associated developmental abnormalities. Becker s nevus is a relatively common cutaneous hamartoma, but is often overlooked or misdiagnosed. While it is generally considered an acquired rather than congenital disorder, there exists at least one case report documenting what researchers claim is a congenital becker s nevus with genetic association. Becker melanosis, also known as becker nevus, is a relatively common cutaneous hamartoma. It is due to an overgrowth of the epidermis upper layers of the skin, pigment cells melanocytes and hair follicles. Beckers nevus appears in childhood as a unilateral tan patch, increasing in thickness, pigmentation, and hair growth during adolescence becker, 1949. Familial beckers nevus familial beckers nevus jain, hem c fisher, benjamin k. Concurrent melanosis and hypertrichosis in distribution of nevus unis. Pathology outlines beckers nevus beckers melanosis. It typically occurs on one shoulder and upper trunk but occasionally occurs elsewhere on the body. Beckers nevus is a noncancerous, large, brown birthmark occurring mostly in males. In this report, a rare case of multiple becker nevi arranged in a.
A rare case of checkerboardlike becker nevi with a unique. Pdf becker s melanosis in association with ipsilateral. Becker nevus syndrome, american journal of medical. We had the opportunity to evaluate two patients with typical beckers melanosis who also had several developmental abnormalities. Grande sarpa h, harris r, hansen cd, callis duffin kp, florell sr, hadley ml.
An otherwise normally developed child who had this hamartoma at birth is described in an attempt to clarify the relationship between pilar smoothmuscle hamartomas and beckers melanosis. All of three cases developed cancerous lesions over the area of becker nevus, which was a sunprotected and atypical site in a lowrisk patient 9, 10. Files are available under licenses specified on their description page. Beckers nevus syndrome is an association of beckers nevus with unilateral breast hypoplasia and muscle, skin, andor skeletal abnormalities. Skin nonmelanocytic tumor becker s nevus becker s melanosis first described by becker in 1948 arch dermatol 1948. Thus the pathophysiology of beckers nevus remains unclear. Medical knowledge and documentation of this disorder is poor, likely due to a combination of factors including recent discovery, low prevalence, and the more or less aesthetic nature of the effects of the skin disorder. Congenital melanocytic nevi cmn are visible pigmented melanocytic proliferations in the skin that are. Beckers nevus and lethal betaactin mutations journal.
Mediumsized congenital melanocytic nevus is defined as having a diameter more than 2 cm 0. Economia principios y aplicaciones monchon y beker 4ta. It develops during childhood or adolescence on the shoulders or upper. The purpose of this study was to determine the prevalence of lesional hypertrichosis. All structured data from the file and property namespaces is available under the creative commons cc0 license. Beckers nevus genetic and rare diseases information. To our knowledge, there are no studies assessing lightbased treatment of hypertrichosis in beckers nevus, aside from a case report using ruby laser in 1998 10. Although the becker nevus syndrome bns is primarily defined as a becker nevus bn in association with ipsilateral breast hypoplasia or musculoskeletal malformation, there are case reports about various associated malformations. Histologically, epidermal acanthosis is accompanied by irregularly. Beckers nevus is a cutaneous hamartoma affecting approximately 1 in 200 individuals tymen et al. We report herein two cases with extensive lesions accompanied by subjacent and distant soft tissue and bony abnormalities. William becker in 1949 as a localized hypermelanosis and hypertrichosis is a relatively common benign smooth muscle hamartoma. Becker s nevus syndrome is an association of becker s nevus with unilateral breast hypoplasia and muscle, skin, andor skeletal abnormalities.
This nevus usually begins during the second decade of life as a circumscribed, hyperpigmented patch with irregular outline that gradually enlarges with associated hypertrichosis, developing several years later. Introduction becker nevus, also called becker melanosis or pigmented hairy epidermal nevus, is a benign cutaneous hamartoma that develops as a light or dark brown macule with welldefined but irregular borders and can present hypertrichosis. Becker nevus syndrome is a phenotype characterized by the fundamental presence of beckers nevus with ipsilateral hypoplasia of the. Becker nevus syndrome is a phenotype characterized by the fundamental presence of becker s nevus with ipsilateral hypoplasia of the breast or other skin, skeletal. Beckers nevus bn has been reported in association with smoothmuscle hamartoma, and an acneiform eruption and underlying structural anomalies of bone and soft tissues. Beckers nevus is a relatively common cutaneous hamartoma, but is often overlooked or misdiagnosed. Jun 16, 2017 although the becker nevus syndrome bns is primarily defined as a becker nevus bn in association with ipsilateral breast hypoplasia or musculoskeletal malformation, there are case reports about various associated malformations. The etiology of beckers nevus and beckers nevus syndrome has been until now unknown. Histologically, epidermal acanthosis is accompanied by irregularly dispersed ectopic smooth muscle. The patient was in excellent health and the lesion was asymptomatic. We report a case of bn coexistent with a large connectivetissue nevus ctn on the right shoulder of a 74yearold man. Becker melanosis is an acquired melanosis and hypertrichosis usually in a unilateral distribution, first described in 1948 by s. A becker naevus nevus in american spelling is a lateonset epidermal naevus or birthmark occurring mostly in males. Select print from the file menu and choose the printer named win2pdf, and.
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