Medicine:Pigmented hairy epidermal nevus syndrome

From HandWiki
Short description: Medical condition
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

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

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.[4]

See also

References

  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 (Medknow) 68 (2): 205–207. doi:10.4103/ijd.ijd_927_22. ISSN 0019-5154. 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. ↑ 4.0 4.1 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 (Springer Science and Business Media LLC) 15 (1): 395. doi:10.1186/s13256-021-02996-y. ISSN 1752-1947. PMID 34365967. 
  5. ↑ Dasegowda, SathyanarayanaB; Basavaraj, GB; Nischal, KC; Swaroop, MR; Umashankar, NP; Swamy, SuchethaS (2014). "Becker′s nevus syndrome". Indian Journal of Dermatology (Medknow) 59 (4): 421. doi:10.4103/0019-5154.135530. ISSN 0019-5154. PMID 25071279. 
  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 (Elsevier BV) 57 (10): 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 (Wiley) 3 (1): 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 (Wiley) 30 (2): 154–156. doi:10.1111/j.1346-8138.2003.tb00365.x. ISSN 0385-2407. PMID 12692385. 
  11. ↑ Urbani, C. E. (2001). "Paradominant inheritance, supernumerary nipples and Becker's nevus: once again!". European Journal of Dermatology 11 (6): 597. ISSN 1167-1122. PMID 11701421. 

Further reading

  • 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. 
Classification
External resources

Template:Skin tumors, epidermis



Retrieved from "https://handwiki.org/wiki/index.php?title=Medicine:Pigmented_hairy_epidermal_nevus_syndrome&oldid=4171111"