Rate of Benign and Malignant Secondary Tumors Associated With Nevus Sebaceous: A Systematic Review and Meta-Analysis.

BACKGROUND: Nevus sebaceous (NS) is a rare congenital skin lesion affecting approximately 0.3% of all newborns. Although benign, NS lesions can harbor malignant secondary tumors. The published rate of development of these malignant tumors varies. This meta-analysis aimed to identify the rate of malignant and benign secondary neoplasms occurring in NS. METHODS: A literature search was conducted using PubMed, Embase, and Web of Science from inception to April 2023. Eligible studies reported incidence or risk of secondary neoplasms in patients with NS. Two independent reviewers screened studies, extracted data, and assessed the quality of included studies. The primary outcome was the pooled incidence of secondary neoplasms. Studies with sample sizes greater than 50 patients were eligible for meta-analysis using the random-effects model. RESULTS: Twenty-eight studies were identified, 22 of which were eligible for meta-analysis. The overall rate of secondary neoplasms was 12.8% (95% confidence interval [Cl], 9.2%-17.6%). The rates of development of malignant and benign tumors were 2.4% (95% CI, 1.4%-4.1%) and 10.3% (95% CI, 7.5%-13.9%), respectively. The rate of development of basal cell carcinoma was 1.7% (95% CI, 0.9%-3.2%), whereas the rate of the development of syringocystadenoma papilliferum was 3.6% (95% CI, 2.5%-5.3%) and that if trichoblastoma was 2.6% (95% CI, 1.7%-3.8%). CONCLUSIONS: Although the rate of development of malignant tumors within a primary NS lesion is low, it is not negligible. Prophylactic early excision remains a viable approach to prevent secondary malignant neoplasms, address cosmetic and functional complications, and preempt the need for complex reconstruction in the future. We propose that resection of NS lesions in childhood remains a reasonable first-line option in the appropriate patient keeping in mind that it may leave an undesirable scar.

Sebaceous Nevus of the Scalp.

Excisions of scalp nevus sebaceous (NS) presents a unique challenge due to limited soft tissue laxity, hair-bearing skin, and convex surfaces which often leave the surgeon and patient underwhelmed with the reconstructive outcome. In this study, the authors conducted an institutional review board-approved retrospective review of patients who underwent excision of pathologically proven scalp primary NS from 2003 to 2017 at our institution to better define the reconstructive outcomes and options for treatment of pediatric scalp NS. 92 patients were included in the study, 54 males (58.7%) and 38 females (41.3%). The average age at surgery was 7.24 years (0.5-16.0; SD 4.7). Local tissue undermining/galeal scoring with primary closure (LTUGS) was used for lesions with average surface area of 3.6 cm, rotational or transposition flaps (RF/TF) for lesions averaging 4.3 cm, completed serial excision for lesions averaging 13.9 cm, and tissue expansion (TE) for lesions averaging 21 cm (P <0.001). One or more poor outcomes were experienced by 35 patients (38%), with a significant difference between the surgical groups; LTUGS 37.2% (29/78), RF/TF 60% (3/5), serial excision 100% (3/3), TE 0% (0/6) (P = 0.022). Univariant binary regression analysis within the LTUGS and RF/TF groups showed that lesion size was a significant predictor of poor outcomes (P = 0.012). All specimens in this study were negative for carcinoma. Therefore, most pediatric nevus sebaceous of the scalp can be managed by a single-phase procedure though risk of poor outcomes increase with nevus size with high rates of poor outcomes even with small lesions.

Management of naevus sebaceous: a national survey of UK dermatologists and plastic surgeons.

Naevus sebaceous (NS) is a congenital cutaneous hamartoma, which typically occurs on the head and neck. Historically, the treatment of choice was excision in infancy because of the potential for malignant transformation; however, recent studies suggest that this risk is < 1% and unlikely in childhood. We sent a questionnaire to UK dermatologists and plastic surgeons to investigate current management practice of NS. We found that almost a third of dermatologists still recommend excision for malignancy prevention, while over 90% of plastic surgeons consider excision, with 64% citing malignancy prevention as the reason. Plastic surgeons most commonly recommended excision in childhood, whereas dermatologists waited until adulthood. We have shown there is significant variation in practice across the UK in the management of naevus NS. It is important that patients across the UK receive the same standard of care, and therefore we advocate the development of evidence-based guidance for treatment of naevus NS.

Picosecond 532-nm neodymium-doped yttrium aluminum garnet laser-a promising modality for the management of verrucous epidermal nevi.

The verrucous epidermal nevus (VEN) is the most common type of epidermal nevi. As lesions can be disfiguring, treatment is often sought. Many therapeutic approaches have been reported, with variable efficacy and safety. Picosecond (PS) lasers are novel laser devices designated to target small chromophores. A side effect of these lasers is blistering due to epidermal-dermal separation. We aimed to harness this side effect of the PS lasers to treat patients with VEN. The purpose of this study was to report our experience treating VEN using a PS 532-nm laser. We present a retrospective case series of six patients with large VEN who were treated using a PS 532-nm laser (2-6 treatments, 8-10 weeks apart). Response in clinical photographs was assessed by two independent dermatologists and graded on a scale of 0 (exacerbation) to 4 (76-100% improvement). Patient satisfaction was recorded on a scale of 1-5. All patients demonstrated significant improvement. Average improvement was 3.7 on the quartile scale of improvement. Patient satisfaction rate averaged 4.7. The PS 532-nm laser is a promising novel modality for the treatment of large VEN.

Syringocystadenocarcinoma Papilliferum In Situ, a Variant of Cutaneous Adenocarcinoma In Situ: A Case Report With Literature Review.

Syringocystadenocarcinoma papilliferum in situ, a variant of cutaneous adenocarcinoma in situ, is extremely rare. Only 9 cases have been published to date with 2 cases demonstrating pagetoid epidermal involvement. In this study, we report a case of syringocystadenocarcinoma papilliferum in situ with pagetoid epidermal involvement arising from a long-standing nevus sebaceus on the scalp of a 60-year-old woman.

Double-Opposing Unilobar Rotation Flaps in the Reconstruction of Moderate-to-Large Defects of the Scalp.

Closure of medium-to-large-size defects of the scalp are often associated with unacceptable aesthetic results, wound break down, alopecia, and excessive scarring. The authors present 2 cases of double-opposing unilobar rotation flaps for the reconstruction of large, that is, at least 7 cm diameter, full thickness defects of the scalp. Unlike previously described double flap closures of scalp defects, the double-opposing unilobar rotation flap design are true rotation flaps, which require a Burow triangle excisions and which have a versatility in both width and length of design to accommodate closure of large defects of the scalp. Some of the advantages of this technique are the retention of hair-bearing skin without distortion of the follicle position, an aesthetically pleasing scar, minimal tension at the wound closure site, and the versatility as well as simplicity of the technique.

Syringocystadenocarcinoma papilliferum: a systematic review of clinical characteristics, reappraisal of associations, diagnostic pitfalls and management challenges.

Syringocystadenocarcinoma papilliferum (SCACP) is a rare and aggressive malignant adnexal tumor originating from apocrine or pluripotent appendageal glands, often associated with a preceding syringocystadenoma papilliferum (SCAP) or nevus sebaceus (NS). This systematic review rigorously examines SCACP through an analysis of 78 cases documented between 1980 and 2024. The study aims to provide a comprehensive review of the clinical manifestations, diagnosis, treatment modalities, and outcomes associated with SCACP, while also reappraising its associations, particularly with NS. SCACP predominantly affects older adults, with an average age of 66.3 years and a slight male predominance, commonly presenting as ulcerated nodules or plaques on the scalp. This review highlights the aggressive nature of SCACP, evidenced by significant rates of metastasis and recurrence. Treatment is primarily surgical, with Mohs micrographic surgery offering potential benefits in terms of margin control and cosmetic outcomes. The association of SCACP with NS is critically evaluated, suggesting a complex etiopathogenesis and underscoring the importance of recognizing this association for timely diagnosis and management. Our review also briefly discusses potential pitfalls faced by clinicians in the diagnosis of SCACP. Our findings emphasize the need for standardized treatment protocols and further research into targeted therapies to improve patient outcomes in SCACP.

Carbon dioxide laser: first-line therapy in vulvar inflammatory linear verrucous epidermal nevus.

Inflammatory linear verrucous epidermal nevus (ILVEN) is normally associated with the failure of topical and systemic treatments and with recurrences on interruption of therapy. Many physical approaches have been used, but they generally resulted in varying rates of recurrence and unacceptable scarring. We reported a case of ILVEN treated with a single session CO(2) laser treatment. In our experience, CO(2) laser was quick, easy, effective, and safe; we therefore believe that this approach should be considered as a first-line surgical option in the treatment of genital ILVEN, particularly in cases of mucosal involvement.

Ileostomy-associated chronic papillomatous dermatitis showing nevus sebaceous-like hyperplasia, HPV 16 infection, and lymphedema: a case report and literature review of ostomy-associated reactive epidermal hyperplasias.

Chronic papillomatous dermatitis (CPD) is a stoma site complication due to chronic irritant contact dermatitis. Papillomatosis can also arise in the setting of human papillomavirus (HPV) infection or chronic lymphedema (elephantiasis). Herein, we report the case of a 57-year-old female who presented with a papillomatous growth surrounding a loop ileostomy suspected to be recurrent ovarian serous carcinoma. Excisional biopsy demonstrated nevus sebaceous (NS)-like organoid hyperplasia with koilocytes overlying a dermal scar that exhibited lymphangiectases. Polymerase chain reaction and sequencing for HPV DNA detected HPV 16. In situ hybridization for high-risk HPV DNA showed punctate nuclear pattern in the keratinocytes populating the NS-like hyperplasia indicating integrated HPV 16 DNA. No recurrence has been observed 11 months postexcision. Reports of CPD have documented a spectrum of reactive epidermal hyperplasias including pseudoepitheliomatous, verrucous, papillomatous, syringofibroadenomatous, and rudimentary follicular hyperplasias. HPV DNA has been detected in 3 of 4 CDP cases tested to date and in authentic NS. We postulate that localized lymphedema secondary to scarring coupled with chronic epidermal irritation and inflammation allowed for latent HPV infection to manifest as CPD with NS-like cutaneous hyperplasia.

Electroplaning of non-inflammatory linear verrucous epidermal nevi (LVEN).

BACKGROUND: The non-inflammatory linear verrucous subtype of epidermal nevi (LVEN) although generally benign, can be aesthetically displeasing and functionally disfiguring to patients. OBJECTIVE: To provide a permanent improvement in the clinical appearance of LVEN with minimal scarring. METHOD: Electroplaning with Surgitron® FFPF EMC was the chosen method of treatment. RESULTS: Electroplaning is a simple, safe, and effective method for improving the appearance of non-inflammatory linear epidermal nevi.

Nevus sebaceous revisited.

Nevus sebaceous of Jadassohn is a hamartoma with a combination of abnormalities of the epidermis, hair follicles, and sebaceous and apocrine glands. Herein, we discuss the results of an extensive literature review on the topic of nevus sebaceous with a particular focus on the debate about the necessity for prophylactic excision. We also focus on the documentation of associated malignant tumors that were reported to develop within NS. In addition to reporting the number and types of neoplasms, we documented the recommendations of all authors for therapeutic handling of these nevi.

Isolated unilateral linear epidermal nevus of the upper eyelid.

A 75-year-old man presented with a recurrent, unilateral, solitary, linear, corrugated lesion of the right upper eyelid of prolonged duration together with bilateral dermatochalasis. A re-excision with blepharoplasty was performed. Histopathologic analysis of the tissue revealed parallel linear arrays of papillomatosis and acanthosis with overlying basket-weave hyperkeratosis consistent with a linear epidermal nevus. Immunohistochemical studies disclosed normal numbers of intraepidermal melanocytes and Langerhans cells without Merkel cells or an increase in cycling keratinocytes. Although the term "nevus" is mostly used in conjunction with the common nevomelanocytic nevus, in fact nevi of other cutaneous cellular elements can occur on a malformational basis (such as sebaceous, eccrine, apocrine, pilar, and elastic fiber nevi). Ophthalmologists should be aware of epidermal nevi because they are rarely associated with cataracts, malignant cutaneous neoplasms, neurologic abnormalities, and musculoskeletal disorders. For focal lesions like the present one, local excision is appropriate. A select differential diagnosis of histopathologically related conditions is provided.

Letter: Transient peripheral facial nerve paralysis after local anesthetic procedure.

Complications may arise after laser therapy of the face. The most common ones are bleeding and infections; facial nerve paresis or paralysis is rarely reported. We describe a case of a transient peripheral facial nerve paralysis after laser therapy of an epidermal verrucous nevus localized at the left preauricular area.

Carbon dioxide laser treatment of epidermal nevi: response and long-term follow-up.

BACKGROUND AND OBJECTIVES: Epidermal nevi, which are benign skin growths, have been treated using a range of approaches, with varying results. Topical treatments are ineffective and, while surgical excision is a more definitive treatment, it causes scar formation. In recent decades, epidermal nevi have been treated with various types of laser therapy. We describe our experience with the use of carbon dioxide (CO(2)) laser therapy to treat epidermal nevi and inflammatory linear verrucous epidermal nevi (ILVEN). PATIENTS AND METHODS: Twenty patients (15 with epidermal nevi and 5 with ILVEN) underwent CO(2) laser treatment at our hospital between 2002 and 2010. RESULTS: Response was good (>50% reduction in lesion size) in 50% of cases and excellent (>75% reduction) in 30%. A greater resistance to treatment was observed in patients with ILVEN (only 40% had a good response). Long-term follow-up (at least 18 months) showed a recurrence rate of 30%. The side effects were hypopigmentation (25% of patients) and scarring (20%). CONCLUSIONS: We consider CO(2) laser therapy to be the treatment of choice for epidermal nevi as it is well tolerated and has proven to be safe and effective in the long term. While the response in patients with ILVEN was limited, CO(2) laser therapy might be a good option for selected cases or for palliative treatment since no other treatments have yet proven effective in this setting.