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E-CASE REPORT
Year : 2014  |  Volume : 59  |  Issue : 4  |  Page : 421
Becker's nevus syndrome


Department of Dermatology, Adichunchanagiri Institute of Medical Sciences, BG Nagara, Karnataka, India

Date of Web Publication27-Jun-2014

Correspondence Address:
Dr. Sathyanarayana B Dasegowda
Professor and Head, Department of Dermatology, Adichunchanagiri Institute of Medical Sciences, BG Nagara, Karnataka - 571 448
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-5154.135530

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   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.


Keywords: Becker′s nevus, Becker′s nevus syndrome, collagen hamartoma


How to cite this article:
Dasegowda SB, Basavaraj G, Nischal K, Swaroop M, Umashankar N, Swamy SS. Becker's nevus syndrome. Indian J Dermatol 2014;59:421

How to cite this URL:
Dasegowda SB, Basavaraj G, Nischal K, Swaroop M, Umashankar N, Swamy SS. Becker's nevus syndrome. Indian J Dermatol [serial online] 2014 [cited 2019 Nov 14];59:421. Available from: http://www.e-ijd.org/text.asp?2014/59/4/421/135530

What was known?
1. Becker′s nevus syndrome is a part of epidermal nevus syndrome, which is a disease complex of epidermal nevi and developmental abnormalities of different organ systems.
2. Several ipsilateral developmental abnormalities have been described, particularly breast hypoplasia, supernumerary nipples, aplasia of the pectoralis major muscle, limb reduction, segmental odontomaxillary dysplasia, and lipoatrophy.
3. Commonest histopathological association of Becker′s nevus is smooth muscle hamartoma.



   Introduction Top


Becker's nevus syndrome is a part of epidermal nevus syndrome, which is a disease complex of epidermal nevi and developmental abnormalities of different organ systems. [1] Epidermal nevus syndromes include Becker's nevus syndrome, Nevus sebaceous syndrome, Nevus comedonicus syndrome, Proteus syndrome, and CHILD syndrome. [2] Beckers's nevus is a cutaneous hamartoma characterized by circumscribed hyperpigmentation with hypertrichosis, which was described by Becker in 1949. [3] Many authors have observed the association of Becker's nevus with other cutaneous, musculoskeletal, or maxillofacial anomalies. However, it was only in 1995 that the term hairy epidermal nevus syndrome or Becker's nevus syndrome was described by Happle as an association of Becker's nevus with unilateral breast hypoplasia and muscle, skin, and/or skeletal abnormalities. We are presenting a case of Becker's nevus syndrome in a 15-year-old female who presented with bilateral Becker's nevus over her groins, thighs, vulva, in front of the neck from the age of 5 years. The associated features of Becker's nevus in our case are rare and many have not been reported in the literature.


   Case Report Top


A 15-year-old female patient presented with a large dark-colored patch over genitals, upper and inner aspects of both the thighs, and in front of the neck. The lesions started at the age of 5 years. The lesions were smaller in size initially and they gradually increased. History of first degree consanguinity and delayed development of milestones were noted. The patient started walking and speaking at the age of 5 years. She was a dwarf, deaf, and had not attained her menarche.

On local examination, a large hyperpigmented plaque over the groin extending from the suprapubic region to the lower part of anteromedial surfaces of both the thighs, including vulva, were observed. The lesion was hyperkeratotic, hyperpigmented, and slightly indurated. It was grayish black and covered with slightly coarse hair except for the pubic, suprapubic, and upper thigh region, which was smooth and less pigmented. Vulva was hypertrophic [Figure 1]. Another hyperpigmented indurated plaque was observed over the thyroid region [Figure 2].
Figure 1: Hyperpigmented, hyperkeratotic, indurated plaque over groins, vulva, and anteromedial aspect of the thighs

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Figure 2: Hyperpigmented plaque over the anterior aspect of the neck

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On a general examination, the patient was found to be mentally retarded and deaf with a short stature. She had depressed nasal bridge, muddy conjunctiva with hypertrophic and hyperpigmented caruncle of both eyes, ichthyosis, brownish hair, absence of axillary and pubic hair, mitral valve prolapse, skeletal abnormalities namely long slender digits of both limbs (markedly seen in great toes and middle fingers), crowding of lateral toes, and halux valgus deformity of left great toe and syndactyly of left 4 th and 5 th toes [Figure 3].
Figure 3: Syndactyly of left 4th and 5th toes with left great toe halux valgus deformity

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Tissue sample was obtained in a punch biopsy from the hyperpigmented patch over the groin area. Histopathological examination revealed hyperkeratosis, acanthosis, elongated rete ridges, and increased pigmentation of basal keratinocytes: these observations were suggestive of Becker's nevus [Figure 4]. Dermal picture showed proliferation of dense collagen upto the subcutaneous tissue suggesting the picture of the collagen tissue hamartoma underneath the Becker's nevus. Van Gieson stain confirmed the dermal collagen proliferation [Figure 5].
Figure 4: Hyperkeratosis, acanthosis, elongated rete ridges, increased pigmentation of basal keratinocytes from a hyperpigmented, and hyperkeratotic plaque over groin area (H and E, x100)

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Figure 5: Increased dermal collagen proliferation observed on special stain (Van Gieson, × 100)

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   Discussion Top


Becker's nevus, also called Becker's melanosis and Becker's pigmentary hamartoma, is a cutaneous hamartoma characterized by circumscribed hyperpigmentation with hypertrichosis. The sporadic occurrence and asymmetrical distribution of most Becker's nevi suggest cutaneous mosaicism. [4] Few cases of familial presentations have been reported: It was found in 0.25% of the study population in a study and in 0.52% of them in another study. Hypothesis of hormonal hyperresponsiveness (i.e., androgen sensitivity) is suggested in this cutaneous hamartoma as Becker's nevus is more prone to acne and hypertrichosis and is predominant in males. [5] It is rarely congenital and may present in childhood, but it is usually first noticed during adolescence, initially pale in color and becoming more conspicuous later. It usually presents unilaterally, though few cases of bilateral presentation have been reported, the usual site is shoulder, anterior chest or scapular region, but lesions on face, neck, and distal limbs have been reported. [6]

Cutaneous anomalies reported to co-localize with Becker's nevus include granuloma annulare, basal cell carcinoma, malignant melanoma, lymphangioma, osteoma cutis, and hypohidrosis. Spina bifida, scoliosis, pectus carinatum, congenital adrenal hyperplasia, quadriparesis, and an accessory scrotum have also been reported. [6]

Several ipsilateral developmental abnormalities have been described, particularly breast hypoplasia, [7] supernumerary nipples, [8] aplasia of the pectoralis major muscle, [9] limb reduction, [10] segmental odontomaxillary dysplasia, [11] and lipoatrophy. [12]

On histopathology, an increased level of melanin in the basal epidermal layer was observed. Epidermis is acanthotic with variable hyperkeratosis and elongation of rete ridges. [13] Histopathological association that is common with Becker's nevus is smooth muscle hamartoma. [14] Only three cases of collagen hamartoma associated with Becker's nevus have been reported in the literature till date. [15],[16],[17]


   Conclusion Top


The peculiar findings in our case included early age of onset of Becker's nevus, bilateral presentation, uncommon site of presentation (i.e., groins, thighs, vulva, neck), association with 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, absence of axillary and pubic hair, and histopathological finding of collagen hamartoma underneath the Becker's nevus.

 
   References Top

1.Loose JE, Serletti JM, Pennino RP. Epidermal nevus syndrome: A review and case report. Ann Plast Surg 1999;43:211-4.  Back to cited text no. 1
    
2.Happle R. Epidermal nevus syndromes. Semin Dermatol 1995;14:111-21.  Back to cited text no. 2
    
3.Cossendy FE, Martinez NS, Bernhard GA, Dias MFRG, Azulay DR. Sindrome do nevo de Becker. An Bras Dermatol 2010;85:379-83.  Back to cited text no. 3
    
4.Lambert JR, Willems P, Abs R, Van Roy B. Becker's nevus associated with chromosomal mosaicism and congenital adrenal hyperplasia. J Am Acad Dermatol 1994;30:655-7.  Back to cited text no. 4
    
5.Person JR, Longscope C. Becker's naevus: An androgen-mediated hyperplasia with increased receptors. J Am Acad Dermatol 1984;10:235-8.  Back to cited text no. 5
    
6.Moss C, Shahidullah H. Naevi and other Developmental Defects. In: Burns T, Breathnach S, Cox N, Griffiths C, editors. Rook's Textbook of Dermatology. Vol. 1. 8 th ed. Wiley-Blackwell; 2010.P S18.17-18.19.  Back to cited text no. 6
    
7.Hoon Jung J, Chan Kim Y, Joon Park H, Woo Cinn Y. Becker's nevus with ipsilateral breast hypoplasia: Improvement with spironolactone. J Dermatol 2003;30:154-6.  Back to cited text no. 7
    
8.Urbani CE. Paradominant inheritance, supernumeray nipples and Becker's nevus: Once again. Eur J Dermatol 2001;11:597.  Back to cited text no. 8
    
9.Moore JA, Schosser RH. Becker's melanosis and hypoplasia of the breast and pectoralis major muscle. Pediatr Dermatol 1985;3:34-7.  Back to cited text no. 9
    
10.Copeman PWM, Wilson-Jones EWJ. Pigmented hairy epidermal nevus (Becker). Arch Dermatol 1965;92:249-51.  Back to cited text no. 10
    
11.Jones AC, Ford MJ. Simultaneous occurrence of segmental odontomaxillary dysplasia and Becker's nevus. J Oral Maxillofac Surg 1999;57:1251-4.  Back to cited text no. 11
    
12.Van Gerwen HJ, Koopman RJ, Steijlen PM, Happle R. Becker's nevus with localized lipoatrophy and ipsilateral breast hypoplasia. Br J Dermatol 1993;129:213.  Back to cited text no. 12
    
13.Lapeere H, Boone B, Schepper SD, Vergaeghe E, Ongenae E, Geel NV, et al. Hypomelanoses and Hypermelanoses. In: Wolff K, Goldsmith LA, Katz SI, Gilchrist BA, Paller AS, Leffell DJ, editors. Fitzpatrick's Dermatology in General Medicine. Vol. 1., 7 th ed. Philadelphia: Mc Graw Hill; 2003.p. 639.  Back to cited text no. 13
    
14.Thappa DM, Sirka CS, Srikanth S. Smooth muscle hamartoma associated with bilateral becker's nevus. Indian J Dermatol Venerol Leprol 1997;63:387-9.  Back to cited text no. 14
    
15.Kim DH, Kim CW, Kim TY. Becker's naevus associated with connective tissue naevus. Acta Derm Venereol 1999;79:393-4.  Back to cited text no. 15
    
16.Fenske NA, Donelan PA. Becker's nevus coexistent with connective-tissue nevus. Arch Dermatol 1984;120:1347-50.  Back to cited text no. 16
    
17.Panizzon R, Brungger H, Vogel A. Becker nevus. A clinic-histologic-electron microscopy study of 39 patients. Hautarzt 1984;35:578-84.  Back to cited text no. 17
    

What is new?
1. Becker's nevus syndrome occurring in female at early age with bilateral involvement of lower extremities.
2. Unusual skeletal abnormalities (depressed nasal bridge, long slender digits, crowding of lateral toes, valgus deformity of first metatarsophalangeal joint), mental retardation, and cardiac defects (mitral valve prolapse).
3. Occurrence of collagen hamartoma.


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]



 

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    Abstract
   Introduction
   Case Report
   Discussion
   Conclusion
    References
    Article Figures

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