Becker′s nevus syndrome

Abstract

Becker's nevus is a cutaneous hamartoma characterized by circumscribed hyperpigmentation with hypertrichosis. Becker's nevus syndrome is an association of Becker's nevus with unilateral breast hypoplasia and muscle, skin, and/or skeletal abnormalities. We here report a case of a 15 year-old 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. She had associated mental retardation, delayed development of mile stones, delayed puberty, dwarfism, depressed nasal bridge, long slender digits, crowding of lateral toes, valgus deformity of first metatarsophalangeal joint, mitral valve prolapse, muddy conjunctiva with hypertrophic and hyperpigmented caruncle of both eyes, ichthyosis, brownish hair, and absence of axillary and pubic hair. On histopathological examination collagen hamartoma underneath the Becker's nevus was found.

Becker's nevus syndrome: a report of a rare disease with unusual associations

“…Most of the previously reported anomalies tend to show a regional correspondence to the nevus and are generally ipsilateral. 4,10 The presence of bilateral pectoralis muscle hypoplasia in our patient represented another rare manifestation. We would also like to emphasize that a thorough clinical examination, including internal organ assessment, is very important in any patient presenting with Becker's nevus.…”

“…reported a case of BNS with neurofibromatosis type 1 . Intellectual handicap, delayed achievement of milestones, delayed puberty, dwarfism, a depressed nasal bridge, long slender digits, crowding of the lateral toes, valgus deformity of the first metatarsophalangeal joint, muddy conjunctiva with hypertrophic and hyperpigmented caruncles in both eyes, ichthyosis, brownish hair, and the absence of axillary and pubic hair were reported by Dasegowda et al . Petraud et al .…”

“…We were unable to find any previous reports of Gilbert's syndrome in association with BNS. Most of the previously reported anomalies tend to show a regional correspondence to the nevus and are generally ipsilateral . The presence of bilateral pectoralis muscle hypoplasia in our patient represented another rare manifestation.…”

Co-occurrence of vitiligo and Becker's nevus: A case report

“…Bazen ipsilateral meme hipoplazisi, skolyoz, pektus ekskavatum, ipsilateral ekstremite hipoplazisi, odonto maksiller hipoplazi gibi kas, iskelet ve/ veya derinin gelişimsel anomalileriyle birlikte görülebilir ve bu durum BN sendromu olarak adlandırılır 3,4 . Ayrıca BN'ye eşlik eden deri lezyonları arasında liken planus, annular granülom, bazal hücreli karsinom, malign melanoma, lenfanjiyoma, osteoma kutis bildirilmiştir 5,6 . Literatürde vitiligo ve BN birlikteliğinin bildirildiği tek olgu bulunmaktadır 6 .…”

“…Bu hastalıkta tutulan deride melanositler ortadan kalkar ve klinik olarak depigmente makül ve yamalar ortaya çıkar 1 . Becker nevüs (BN) ise sıklıkla unilateral dağılım gösteren, keskin ama düzensiz sınırlı hiperpigmente makül, yama veya verrüköz plakların izlendiği, üzerinde değişik derecelerde hipertrikozun bulunduğu bir hastalıktır [2][3][4][5][6] . Biz burada her iki lezyonu birlikte bulunduran bir hastayı sunmayı amaçladık.…”