Pigmented hairy epidermal nevus syndrome explained

Synonyms:Becker's naevus syndrome

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] [3]

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.[12]

See also

Further reading

Notes and References

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

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