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- Int J Surg Case Rep
- v.105; 2023 Apr
- PMC10090243
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Int J Surg Case Rep. 2023 Apr; 105: 108021.
Published online 2023 Mar 27. doi:10.1016/j.ijscr.2023.108021
PMCID: PMC10090243
PMID: 37001367
Mahmoud Al-Balas,a Hamzeh Al-Balas,a Saif Alshdifat,b,⁎ and Rand Kokashb
Author information Article notes Copyright and License information PMC Disclaimer
Abstract
Introduction
Nevus comedonicus (NC) is a rare cutaneous disorder thought to be caused by hamartomatous pilosebaceous tissue proliferation that was first described in 1895. Clinically NC appears as a group of elevated follicular openings often linearly arrayed, giving the appearance of comedones. NC usually manifests at birth but can also present later during adolescence and rarely in adulthood.
Case presentation
A 21-year-old medically healthy single male presented with right-sided chest black papules in comedo distribution with areas of superficial ulcerations and suppurations in periareolar distribution since the age of 16. Histopathological examination showed dilated follicular ostia filled with keratin plug, devoid of the hair shaft, and lined by stratified squamous epithelium with compact hyperkeratosis, focal parakeratosis, and patchy atrophy and acanthosis.
Clinical discussion
Nevus comedonicus often responds effectively to conservative treatment, however some cases need surgery intervention. The patient failed conservative medical and topical treatment, and he was treated by surgical-wide local excision and primary closure of the affected skin with free nipple grafting.
Conclusion
Nevus comedonicus (NC) is a rare cutaneous pathology secondary to pilosebaceous apparatus developmental defect that usually manifests at birth and can affect any area of skin; they typically manifest as black papules in comedo distribution. They can present as an isolated cutaneous pathology or as a component of nevus comedonicus syndrome. Different therapeutic approaches were described, including topical retinoids, keratolytic agents, oral retinoids, antibiotic therapy, manual extraction of comedos, dermabrasion, and surgical resection of the lesion.
Keywords: Nevus comedonicus, Pilosebaceous apparatus, Hyperkeratosis, Nevus comedonicus syndrome
Highlights
•
Nevus comedonicus is rare cutaneous disorders secondary to hamartomatous pilosebaceous tissue proliferation.
•
Ocular, skeletal, and neural congenital abnormalities can be associated with nevus comedonicus.
•
Nevus comedonicus is associated with cosmetic, psychologic and pathological complications.
•
Different treatment modalities are available for management of nevus comedonicus and depends on the extent of the pathology.
1. Introduction
Nevus comedonicus (NC) is a rare cutaneous disorder thought to be caused by hamartomatous pilosebaceous tissue proliferation. This uncommon developmental abnormality clinically manifests as grouped, often linearly arrayed, elevated follicular openings. The follicular openings eventually fill with dark keratin plugs, giving the appearance of open comedones [1]. Kofmann, who suggested using the term “comedo nevus” first described the condition in 1895 [2]. It is usually present at birth but can begin in adolescence and, in rare cases, adulthood [3].
The most common sites for these lesions are the face, neck, upper arms, chest, and abdomen [4]. Other uncommon locations that might be involved include breast tissue, palm, glans, penis, ear, and scalp [5].This case report has been reported in accordance with SCARE 2020 standards [6].
2. Presentation of case
A 21-year-old male with unremarkable medical history presented to the breast clinic with a complaint of right chest wall pain, dark skin pigmentations, and purulent discharge from superficial ulcerations. Patient complaints started at the age of 16 with dark pigmentations over the right-sided chest wall in a periareolar distribution. Since that, he has had recurrent attacks of chest wall abscesses and discharges that were treated conservatively using antibiotics, intralesional steroids, and topical retinoid cream with unsatisfactory outcomes. The patient has no previous medical illness and is not on any medication with no history of allergy.
The physical examination showed an area with extensive dark papules distributed in a honeycomb appearance and superficial skin ulcerations with pus discharge over the right-sided chest wall in a periareolar distribution [Fig. 1a]. Based on patient evaluation and after a discussion of his uncommon clinical condition, the decision was to proceed with the excision of the affected skin with primary closure.
Fig. 1
A superficial ulcerations and numerous follicular openings filled with dark keratin plugs, giving the appearance of open comedones in right chest wall with periareolar distribution. B) An excised ellipse of skin and subcutaneous tissue after nipple graft excision.
An elliptical wide local excision of the affected right-sided chest skin, subcutaneous tissue with primary closure, and free nipple grafting was performed [Fig. 1b]. The histopathologic report showed an epidermis with hyperkeratosis and focal acanthosis, cystically dilated hair follicles plugged with keratinaceous debris, and one hair follicle in the sections present with surrounding acute inflammation and granulation tissue formation. The dermis showed mild interstitial and perivascular lymphohistiocytic inflammation [Fig. 2]. The patient had an uneventful postoperative course, the nipple graft was completely taken, and no recurrence of symptoms over one year of follow-up has been documented.
Fig. 2
Microscopic appearance of the excised skin. Figures (a, b, c) shows dilated follicular ostia filled with keratin plug, devoid of hair shaft and lined by stratified squamous epithelium with compact hyperkeratosis, focal parakeratosis, and patchy atrophy and acanthosis. Adjacent epidermis shows acanthosis with focal ulceration associated with granulation tissue. The dermis shows moderate mixed inflammatory infiltrates.
3. Discussion
Nevus comedonicus (NC) is a pilosebaceous apparatus developmental defect that is clinically identified by clusters of slightly raised papules that include a comedo-like with a dark, firm hyperkeratotic plug in the core [7]. The precise cause has not been determined; one theory describes NC as an epidermal nevus that develops from the hair follicle. Another theory is that it is a hamartoma derived from the pilosebaceous unit's mesodermal component. It has been proposed that genetic mosaicism plays a role in the etiology of nevus comedonicus [8]. The true incidence of NC ranges from 1 in 45,000 to 100,000, with conflicting predominance based on gender, but a recent study showed that it has a higher incidence in males with a male-female ratio of 1.5:1 [2], [4]. They typically exist from birth but can also appear later, typically before the age of 10. It is uncommon for NC to arise in adults, and if it occurs, it is typically accompanied by irritation or trauma [3].
The interaction of fibroblast growth factor (FGF) and FGF receptor-2 (FGFR2) is a critical pathway for the development of pilosebaceous units [9]. Recent studies have shown that somatic mutations in NEK 9 have an essential role in nevus comedonicus, and another study shows upregulation of ABCA12 expression levels in the sebaceous glands of NC patients [8], [10].
Clinically, NC can be classified into two groups, one characterized by the predominance of comedones production without suppuration and another less common group that has acneiform features, inflammatory lesions, and suppuration [11]. The latter is typically challenging to treat since patients frequently experience mild to severe infections and leave behind visible scars (i.e., our case falls into this group).
NC usually manifests as dilated follicular ostia arranged in a linear pattern along the lines of Blaschko. The ostia contain blackhead comedone-like lamellate keratinocytic material [12]. Most lesions are unilateral, but bilateral involvement has also been reported [13]. Lesions are usually restricted to a specific skin region and do not cross the midline [14].
Differential diagnosis consists of numerous types of epidermal nevi (particularly the linear epidermal nevus), familial dyskeratotic comedones, and linear comedonic formations typically associated with acne vulgaris or chronically sun-damaged skin. Atrophoderma vermiculata and keratosis pilaris atrophicans may be mistaken for nevus comedonicus, but their symmetry distinguishes them [15].
Under microscope, large clusters of enlarged follicular ostia devoid of hair shafts filled with keratin layers are the hallmark histopathologic findings. It also can reveal a variety of cystic formations, including small cysts, cystic invaginations, and rarely massive cysts, all of which are lined with keratinizing squamous epithelium. The epidermis can exhibit hyperkeratosis and acanthosis but not para- or dyskeratosis [16].
Diagnosis of NC is usually clinically based. However, dermoscopy or video dermoscopy may also be beneficial in most cases [17]. Biopsy is only recommended in rare cases, particularly in the second group of clinical presentation [3]; in our patient, a chest wall ultrasound was performed and showed subcutaneous edematous tissue with the involvement of breast tissue. The relationship between NC and extracutaneous manifestations is known as nevus comedonicus syndrome (NCS) [12]. Although most cases have no systemic manifestations, it is essential to rule out NCS, which is associated with ocular, skeletal, and neural abnormalities, most commonly ipsilateral congenital cataracts and finger/toe malformations [17].
Since NC is a benign condition, it does not require intensive treatment unless it is for cosmetic reasons or in complicated cases in which inflammatory changes develop [16],that may cause scarring and persistent harm to the skin.Surgery can be performed to ensure complete removal and nonrecurrence [18].A study using pore strips revealed an excellent prognosis [19], we can also use systemic antibiotics, intralesional corticosteroid injections, oral isotretinoin,demabrasion, cryotherapy, coagulation, extraction of comedones, and topical treatments such retinoic acid, urea, tretinoin, and ammonium lactate lotion can be used [20]; all have shown varying degree of success.
4. Conclusion
Nevus comedonicus (NC) is a rare cutaneous pathology secondary to a pilosebaceous apparatus developmental defect that usually manifests at birth and can affect any skin area. Regardless of their typical comedo-like black papules, they might manifest similarly to other cutaneous lesions. Identification of NC is vital to avoid related complications such as suppurations and ulceration. Medical local or systemic treatment can be tried prior to surgical excision.
Informed consent
Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.
Ethics approval
Ethical Approval was waived by the authors' institution.
Funding
The author(s) received no financial support for the research, authorship and/or publication of this article.
Author contribution
Mahmoud Al-Balas: study concept, data collection, patient management, literature review, review manuscript
Hamzeh Al-Balas: study concept, data collection, patient management, literature review, review manuscript
Saif Alshdifat: literature review, writing manuscript, preparing draft paper and supplementary material
Rand Kokash: literature review, writing manuscript, preparing draft paper and supplementary material
Guarantor
Saif Alshdifat
Faculty of Medicine, Hashemite University, Zarqa 13133, Jordan
Research registration number
Not applicable.
Conflict of interest statement
The author(s) declared no potential conflicts of interest with respect to research, authorship and/or publication of the article.
References
1. Givan J., Hurley M.Y., Glaser D.A. Nevus comedonicus: a novel approach to treatment. Dermatol. Surg. 2010;36:721–725. doi:10.1111/J.1524-4725.2010.01537.X. [PubMed] [CrossRef] [Google Scholar]
2. Torchia D. Nevus comedonicus syndrome: a systematic review of the literature. Pediatr. Dermatol. 2021;38:359–363. doi:10.1111/PDE.14508. [PubMed] [CrossRef] [Google Scholar]
3. Timbó R.P.F., Gomes M.K., Falcão E.M.M., Ramos-E-Silva M. Nevus comedonicus: case report. Case Rep. Dermatol. 2017;9:184–189. doi:10.1159/000480722. [PMC free article] [PubMed] [CrossRef] [Google Scholar]
4. Patrizi A., Neri I., Fiorentini C., Marzaduri S. Nevus comedonicus syndrome: a new pediatric case. Pediatr. Dermatol. 1998;15:304–306. doi:10.1046/j.1525-1470.1998.1998015304.x. [PubMed] [CrossRef] [Google Scholar]
5. Lefkowitz A., Schwartz R.A., Lambert W.C. Nevus comedonicus. Dermatology. 1999;199:204–207. doi:10.1159/000018275. [PubMed] [CrossRef] [Google Scholar]
6. Agha R.A., Franchi T., Sohrabi C., Mathew G., Kerwan A., Thoma A., et al. The SCARE 2020 guideline: updating consensus surgical CAseREport (SCARE) guidelines. Int. J. Surg. 2020;84:226–230. doi:10.1016/J.IJSU.2020.10.034. [PubMed] [CrossRef] [Google Scholar]
7. Kim S.C., Kang W.H. Nevus comedonicus associated with epidermal nevus. J. Am. Acad. Dermatol. 1989;21:1085–1088. doi:10.1016/S0190-9622(89)70301-7. [PubMed] [CrossRef] [Google Scholar]
8. Liu F., Yang Y., Zheng Y., Liang Y.H., Zeng K. Mutation and expression of ABCA12 in keratosis pilaris and nevus comedonicus. Mol. Med. Rep. 2018;18:3153–3158. doi:10.3892/MMR.2018.9342. [PMC free article] [PubMed] [CrossRef] [Google Scholar]
9. Hafner C., van Oers J.M.M., Vogt T., Landthaler M., Stoehr R., Blaszyk H., et al. Mosaicism of activating FGFR3 mutations in human skin causes epidermal nevi. J. Clin. Invest. 2006;116:2201–2207. doi:10.1172/JCI28163. [PMC free article] [PubMed] [CrossRef] [Google Scholar]
10. Levinsohn J.L., Sugarman J.L., McNiff J.M., Antaya R.J., Choate K.A. Somatic mutations in NEK9 cause nevus comedonicus. Am. J. Hum. Genet. 2016;98:1030–1037. doi:10.1016/J.AJHG.2016.03.019. [PMC free article] [PubMed] [CrossRef] [Google Scholar]
11. Harper K.E., Spielvogel R.L. Nevus comedonicus of the palm and wrist. Case report with review of five previously reported cases. J. Am. Acad. Dermatol. 1985;12:185–188. doi:10.1016/S0190-9622(85)80014-1. [PubMed] [CrossRef] [Google Scholar]
12. Ferrari B., Taliercio V., Restrepo P., Luna P., Abad M.E., Larralde M. Nevus comedonicus: a case series. Pediatr. Dermatol. 2015;32:216–219. doi:10.1111/PDE.12466. [PubMed] [CrossRef] [Google Scholar]
13. Paige T.N., Mendelson C.G. Bilateral nevus comedonicus. Arch. Dermatol. 1967;96:172–175. doi:10.1001/ARCHDERM.1967.01610020064019. [PubMed] [CrossRef] [Google Scholar]
14. Cestari T.F., Rubim M., Valentini B.C. Nevus comedonicus: case report and brief review of the literature. Pediatr. Dermatol. 1991;8:300–305. doi:10.1111/J.1525-1470.1991.TB00938.X. [PubMed] [CrossRef] [Google Scholar]
15. Manola I., Ljubojević S., Lipozencić J., Pustisek N. Nevus comedonicus–case report and review of therapeutical approach. Acta Dermatovenerol. Croat. 2003;11:221–224. [PubMed] [Google Scholar]
16. Tchernev G., Ananiev J., Semkova K., Dourmishev L.A., Scho J., Wollina U. Nevus comedonicus: an updated review. Dermatol. Ther. (Heidelb.) 2013;3(1):33–40. doi:10.1007/S13555-013-0027-9. [PMC free article] [PubMed] [CrossRef] [Google Scholar]
17. Kamińska-Winciorek G., Śpiewak R. Dermoscopy on nevus comedonicus: a case report and review of the literature. Postepy Dermatol. Alergol. 2013;30:252–254. doi:10.5114/PDIA.2013.37036. [PMC free article] [PubMed] [CrossRef] [Google Scholar]
18. Manikavachakan N., Siddaraju M.N., Rajendran S.C., Venkataraman A. Extensive nevus comedonicus, complicated with recurrent abscesses, successfully treated with surgical resurfacing. J. Cutan. Aesthet. Surg. 2018;11:33–37. doi:10.4103/JCAS.JCAS_122_17. [PMC free article] [PubMed] [CrossRef] [Google Scholar]
19. Inoue Y., Miyamoto Y., Ono T. Two cases of nevus comedonicus: successful treatment of keratin plugs with a pore strip. J. Am. Acad. Dermatol. 2000;43:927–929. doi:10.1067/MJD.2000.103992. [PubMed] [CrossRef] [Google Scholar]
20. Chhabra N., Pandhi D., Verma P., Sharma S. Inflammatory nevus comedonicus with epidermoid cyst. Indian J. Dermatol. 2014;59:422. doi:10.4103/0019-5154.135533. [PMC free article] [PubMed] [CrossRef] [Google Scholar]
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