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Pigmented hairy epidermal nevus syndrome
Pigmented hairy epidermal nevus syndrome
Other names	Becker's naevus syndrome
Specialty	Dermatology

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.^[12]

References

^ 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.
^ James, William; Berger, Timothy; Elston, Dirk (2005). Andrews' Diseases of the Skin: Clinical Dermatology. (10th ed.). Saunders. ISBN 0-7216-2921-0.
^ Freedberg, et al. (2003). Fitzpatrick's Dermatology in General Medicine. (6th ed.). McGraw-Hill. ISBN 0-07-138076-0.
^ 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.
^ 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. PMID 25071279.
^ 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.
^ 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.
^ 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.
^ 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.
^ 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.
^ 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.
^ 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.