Follicular Becker's nevus (FBN) is a rare variant of Becker's nevus defined by perifollicular pigmentation and folliculocentric distribution. We report two new cases in Caucasian women, aged 22 and 37, with lesions on the breast and lumbar regions. Both presented progressive, asymptomatic hyperpigmented macules since adolescence. Dermoscopy revealed donut-shaped perifollicular structures with hypopigmented halos surrounded by a reticulated brown network, while histopathology confirmed orthohyperkeratosis, basal pigmentation, elongated rete ridges, and hypertrophic arrector pili muscle bundles. In addition, we reviewed all published FBN cases, highlighting its distinct folliculocentric pattern, predilection for non-scapular sites, and occurrence beyond Asian cohorts. Both of our patients showed favorable outcomes with picosecond laser therapy, suggesting this approach as a promising treatment option.

Becker nevus syndrome is a rare cutaneous disorder characterized by the presence of a hyperpigmented patch of skin typically occurring on the upper trunk, often associated with hypertrichosis, and sometimes associated with musculoskeletal abnormalities ipsilateral to the nevus. Becker nevus syndrome can lead to breast hypoplasia. Unlike other forms of breast hypoplasia, treatment should begin before the completion of breast development. Due to the rarity of the disease, breast reconstruction in these patients has no gold-standard approach, and treatment is often delayed. Therefore, our case report highlighted the need for early treatment and provided options for reconstruction in this population. Our case reported a single surgeon approach to breast hypoplasia in Becker nevus syndrome. Consent was obtained for medical photography and research purposes. A 37-year-old woman with hypertension presented with concerns regarding drastic breast asymmetry. On examination, she was noted to have significant breast asymmetry with right-sided hypoplasia, left macromastia, and a patchy hyperpigmented lesion along the right chest wall. After a discussion of surgical options, a right breast augmentation and left breast reduction was performed. The patient had no postoperative complications. She was satisfied with her breast size and symmetry at her 6-month postoperative clinic follow-up. Becker nevus syndrome in women may be more frequent than previously described in the literature, as it often goes undiagnosed. Little literature exists on providing surgical options. This article demonstrated a single-stage reconstruction that provided satisfactory results and improvement in symmetry.

The follicular variant of Becker's nevus is an under-reported entity. We present the rare occurrence of follicular Becker's nevus in 7 patients, confirmed through dermoscopy and histopathological examination. Dermoscopy shows perifollicular hypopigmentation surrounded by a well-defined net-like pigmentation corresponding clinically to the presence of folliculocentric macules. Histology shows prominent basal and suprabasal melanization surrounding the follicle, corresponding to well-defined net-like pigmentation seen on dermoscopy. However, the melanization does not extend along the entire length of the follicular epithelium leading to perifollicular hypopigmentation on dermoscopy. Though biopsy is confirmatory, it is not usually necessary.

Becker's nevus syndrome is defined by the association of a Becker nevus with homolateral breast hypoplasia or more rarely skeletal cutaneous or muscle deformities. Early diagnosis is important, especially in female patients to prevent and treat breast hypoplasia. We report two cases of Becker nevus syndrome with serious functional impairment and discuss possible treatment options. The term epidermal nevus syndrome (ENS) was originally used as an all-encompassing term used to describe epidermal nevi in association with other syndromic features. In more recent years, this term was expanded to include several more well-defined neurocutaneous syndromes and their association with an epidermal nevus. The genetic basis of many of these syndromes has been elucidated in recent years. However, much is still to be learned about the genotype-phenotype correlation. Common epidermal nevi include non-epidermolytic keratinocyte nevus, nevus sebaceus, nevus comedonicus, Becker’s nevus, and CHILD (congenital hemidysplasia with ichthyosiform nevus and limb defects) nevus. These are hamartomas of the skin, which are grouped as either organoid or keratinocytic. The syndromes included within this umbrella term are somewhat controversial. Therefore, the most frequently included syndromes will be discussed further. Schimmelpenning syndrome, phacomatosis pigmentokeratotica, nevus comedonicus syndrome, and Becker nevus syndrome are among the syndromes associated with organoid epidermal nevi. The syndromes associated with keratinocytic nevi include proteus syndrome and CHILD syndrome.