Abstract

Becker nevus syndrome is a rare disease characterized by a Becker nevus associated with developmental abnormalities. Development, such as ipsilateral breast hypoplasia or other cutaneous, muscular or skeletal abnormalities. cutaneous, muscular or skeletal. We present a rare case of Becker nevus syndrome with spinal cord involvement.

Keywords: Becker’s neavus syndrome; Spina bifida; Hamartoma.

Citation: Hamraoui H, Baybay H, Soughi M, Elloudi S, Douhi Z, et al. Becker nevus associated with spina bifida. J Clin Images Med Case Rep. 2024; 5(5): 3022.

Introduction

Becker’s Nevus (BN) is an isolated benign cutaneous hamartoma, first described by William Becker in 1949 [1]. BN is characterized by unilateral hyperpigmented plaques with or without hypertrichosis [2]. The prevalence of BN was 0.52% with a male-to-female ratio of 4:1 in the peripubertal period or early adulthood [3]. In 1997, Happle proposed the term “Becker nevus syndrome” to describe the association of BN with other cutaneous (mainly ipsilateral breast hypoplasia), muscular and skeletal abnormalities [4]. We report the case of a girl who presents with Becker neavus associated with spinal cord injury.

Observation

This is a 10-year-old child operated on for spina bifida at birth from a non-consanguineous marriage who consulted for a lesion dating back to birth, the dermatological examination revealed the presence of hypertrichosis localized at the lumbosacral level. Sacral surmounted on the right side by an atrophic scar related to the previous operation the action to be taken was to request a lumbosacral MRI which revealed Diastematomyelia type I associating a division of the cord and a fibrous septum Hydromyelia at the level of L1-L2. Lower marrow attached with sacral dysraphism and sacral lumbo dural mega-sac. The file was discussed with the pediatricians who eliminated a contraindication to hair removal laser then the patient was put on Nd yag Spot laser 9 mm 15 d/cm2 1.6 ms 6 Hz having received two sessions with slight improvement.

Figure 1: Hypertrichosis in the sacral region.

Figure 2: Clinical photo after one laser session.

Discussion

Becker’s nevus is also known as Becker’s melanosis. It is a benign lesion which can present itself as congenital or acquired with glabrous or hypertrichotic lesions [5]. Some authors consider it to be exclusively congenital but androgen dependent [6]. Panizzon et al. proposed three varieties of BN, according to clinical presentation: melanotic (39%), hypertrichotic (19%) and mixed types (42%) [7] called Becker nevus syndrome when associated with ipsilateral breast hypoplasia or muscle defects of the skin or skeleton. Considered a rare syndrome belonging to epidermal nevus syndromes [8]. It presents clinically with multiple macules associated with hypertrichosis and hyperpigmentation of the trunk or shoulder with unilateral distribution. It can also present in the form of non-hairy BN [9]. Histologically it is characterized by acanthosis, epidermal hyperpigmentation, papillomatosis, hyperplasia of the arrector pili muscle and melanophages in the dermis [10] generally the Diagnosis of Becker neavus syndrome is clinical, it associates the presence of Becker neavus and other abnormalities which may include ipsilateral hypoplasia of the shoulder or arm, ipsilateral hypoplasia of the breast, supernumerary nipple, facial asymmetry, cutaneous hypoplasia of the temporal region, spina bifida like the case of our patient [11].

The treatment is not specific and can be done by dermabrasion [12], cryotherapy, also various laser treatments have been used alone or jointly. The number of BN treatments ranged from 1 to 12 and the laser wavelengths used ranged from 504 to 10,600 nm. Although a combination of lasers of different wavelengths seems more effective [13] a reduction in hyperpigmentation by the use of topical flutamide which is a topical anti-androgen [14] some authors report the effectiveness of oral spironolactone in Becker nevus associated with breast hypoplasia [15].

Conclusion

We report this case to raise awareness among clinicians of this syndrome which is often associated with a negative socio-psychological impact and aesthetically unpleasant for patients.

References

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