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Pigmented hairy epidermal nevus syndrome

From Wikipedia, the free encyclopedia
Pigmented hairy epidermal nevus syndrome
Other namesBecker's naevus syndrome
SpecialtyDermatology

Pigmented hairy epidermal nevus syndrome, also known as Becker's naevus syndrome,[1] is a cutaneous condition characterized by a Becker nevus, ipsilateral hypoplasia of the breast, and skeletal defects such as scoliosis.[2]: 635 [3]: 776 

Signs and symptoms

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Pigmented hairy epidermal nevus syndrome is defined by the presence of a hypoplastic defect of the muscle, skin, or skeleton, or by ipsilateral breast hypoplasia associated with a Becker's nevus.[4]

Anomalies of the skin such as granuloma annulare, basal cell carcinoma, malignant melanoma, lymphangioma, osteoma cutis, and hypohidrosis have been observed to co-localize with Becker's nevus. There have also been reports of quadriparesis, congenital adrenal hyperplasia, pectus carinatum, spina bifida, and an accessory scrotum.[5]

There have been several reports of ipsilateral developmental anomalies, including lipoatrophy,[6] segmental odontomaxillary dysplasia,[7] limb reduction,[8] aplasia of the pectoralis major muscle,[9] breast hypoplasia,[10] and supernumerary nipples.[11]

Diagnosis

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The majority of the diagnosis is clinical, with a well-defined area of hyperpigmentation and hypoplasia of the ipsilateral breast, areola and/or nipple, limb, muscle, and adipose tissue. Histology results showing high androgen receptor levels in the hypoplastic muscles similar to those expressed in the genitalia further corroborate the diagnosis.[12]

See also

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References

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  1. ^ Kololgi, ShreyasP; Kololgi, PrakashD; Hulmani, Manjunath; Lahari, CS (2023). "Becker's birthmark: A case series of pigmented hairy epidermal naevus". Indian Journal of Dermatology. 68 (2). Medknow: 205. doi:10.4103/ijd.ijd_927_22. ISSN 0019-5154. PMC 10238991. PMID 37275790.
  2. ^ James, William; Berger, Timothy; Elston, Dirk (2005). Andrews' Diseases of the Skin: Clinical Dermatology. (10th ed.). Saunders. ISBN 0-7216-2921-0.
  3. ^ Freedberg, et al. (2003). Fitzpatrick's Dermatology in General Medicine. (6th ed.). McGraw-Hill. ISBN 0-07-138076-0.
  4. ^ Chikani, Ugo N.; Ohuche, Ijeoma N.; Bisi-Onyemaechi, Ada I. (2021-08-09). "Becker's nevus syndrome: a case report". Journal of Medical Case Reports. 15 (1). Springer Science and Business Media LLC: 395. doi:10.1186/s13256-021-02996-y. ISSN 1752-1947. PMC 8351151. PMID 34365967.
  5. ^ Dasegowda, SathyanarayanaB; Basavaraj, GB; Nischal, KC; Swaroop, MR; Umashankar, NP; Swamy, SuchethaS (2014). "Becker′s nevus syndrome". Indian Journal of Dermatology. 59 (4). Medknow: 421. doi:10.4103/0019-5154.135530. ISSN 0019-5154. PMC 4103296.
  6. ^ Van Gerwen, H. J.; Koopman, R. J.; Steijlen, P. M.; Happle, R. (1993). "Becker's naevus with localized lipoatrophy and ipsilateral breast hypoplasia". The British Journal of Dermatology. 129 (2): 213. ISSN 0007-0963. PMID 7654589.
  7. ^ Jones, Anne Cale; Ford, Michael J (1999). "Simultaneous occurrence of segmental odontomaxillary dysplasia and decker's nevus". Journal of Oral and Maxillofacial Surgery. 57 (10). Elsevier BV: 1251–1254. doi:10.1016/s0278-2391(99)90498-6. ISSN 0278-2391. PMID 10513874.
  8. ^ Copeman, P. W.; Jones, E. W. (1965). "Pigmented hairy epidermal nevus (Becker)". Archives of Dermatology. 92 (3): 249–251. doi:10.1001/archderm.1965.01600150039006. ISSN 0003-987X. PMID 11851244.
  9. ^ Moore, Jordan A.; Schosser, Robert H. (1985). "Becker's Melanosis and Hypoplasia of the Breast and Pectoralis Major Muscle". Pediatric Dermatology. 3 (1). Wiley: 34–37. doi:10.1111/j.1525-1470.1985.tb00482.x. ISSN 0736-8046. PMID 4070085.
  10. ^ Jung, Jae Hoon; Kim, You Chan; Park, Hyang Joon; Cinn, Yong Woo (2003). "Becker's Nevus with Ipsilateral Breast Hypoplasia: Improvement with Spironolactone". The Journal of Dermatology. 30 (2). Wiley: 154–156. doi:10.1111/j.1346-8138.2003.tb00365.x. ISSN 0385-2407.
  11. ^ Urbani, C. E. (2001). "Paradominant inheritance, supernumerary nipples and Becker's nevus: once again!". European Journal of Dermatology: EJD. 11 (6): 597. ISSN 1167-1122. PMID 11701421.
  12. ^ Chikani, Ugo N.; Ohuche, Ijeoma N.; Bisi-Onyemaechi, Ada I. (2021-08-09). "Becker's nevus syndrome: a case report". Journal of Medical Case Reports. 15 (1). Springer Science and Business Media LLC: 395. doi:10.1186/s13256-021-02996-y. ISSN 1752-1947. PMC 8351151. PMID 34365967.

Further reading

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  • Danarti, Retno; König, Arne; Salhi, Aïcha; Bittar, Mario; Happle, Rudolf (2004). "Becker's nevus syndrome revisited". Journal of the American Academy of Dermatology. 51 (6): 965–969. doi:10.1016/j.jaad.2004.06.036. PMID 15583590.
  • Torchia, Daniele (2021). "Becker nevus syndrome: A 2020 update". Journal of the American Academy of Dermatology. 85 (2): e101–e103. doi:10.1016/j.jaad.2021.03.095. PMID 33819536.
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