Nevus comedonicus: A case report with the histological findings and brief review of the literature (2024)

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  • Int J Surg Case Rep
  • v.105; 2023 Apr
  • PMC10090243

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Nevus comedonicus: A case report with the histological findings and brief review of the literature (1)

Guide for AuthorsAbout this journalExplore this journalInternational Journal of Surgery Case Reports

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.

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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.

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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

Saif_shdefat@hotmail.com

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

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Articles from International Journal of Surgery Case Reports are provided here courtesy of Elsevier

Nevus comedonicus: A case report with the histological findings and brief review of the literature (2024)

FAQs

What is the histology of nevus comedonicus? ›

Histopathology of nevus comedonicus reveals cystically dilated hair follicles forming epidermal invaginations filled with lamellar keratin. EHK has been described in the keratinocytes of the follicular epithelial wall.

How do you get rid of nevus comedonicus? ›

As nevus comedonicus is an asymptomatic benign condition, no treatment is required. Surgical excision of small lesions can be curative and should be considered in consultation with a dermatologic/plastic surgeon. Incomplete excision may result in recurrence of the lesion. Laser surgery has been tried.

How many people have nevus comedonicus? ›

Nevus comedonicus is considered relatively rare. One dermatology department found 12 cases in 100,000 skin biopsy specimens. Another department reported an incidence of 1 case per 45,000 dermatology visits.

What are the histological features of nevus? ›

Distinguishing histologic features include (1) involvement by nevus cells of deep dermal appendages and neurovascular structures (including hair follicles, sebaceous glands, arrector pili muscles, and within walls of blood vessels), (2) extension of nevus cells to deep dermis and subcutaneous fat, (3) infiltration of ...

What is the histology of connective tissue nevus? ›

Histologic examination of connective tissue nevi shows an excess of collagen or elastic tissue in the dermis. This may not be apparent unless a specimen of normal adjacent skin is obtained for comparison. Thus biopsies of connective tissue nevi are often reported as 'normal skin.

What is the best treatment for nevus? ›

Melanocytic nevi can be surgically removed for cosmetic considerations or because of concern regarding the biological potential of a lesion. Melanocytic nevi removed for cosmesis are often removed by tangential or shave excision. Punch excision can be used for relatively small lesions.

How long does a nevus last? ›

A mole on your skin is also known as a nevus, or a beauty mark. It is very common to have moles and most are harmless. They're not contagious and they shouldn't hurt, itch, or bleed. A mole can last as long as 50 years.

Should I get nevus removed? ›

Congenital moles will need to be monitored for skin cancer. Some may also be removed for cosmetic or functional reasons, especially when the placement of a mole causes emotional distress for a child.

What is a birthmark that looks like a blackhead? ›

A comedo naevus (comedo nevus), also known as naevus comedonicus, is a rare, benign, cutaneous anomaly consisting of grouped, dilated follicular openings containing soft, dark keratin that resemble comedones.

Which type of nevus is most likely to become malignant? ›

However, dysplastic nevi are a risk factor for developing melanoma, and the more dysplastic nevi a person has, the greater their risk of developing melanoma (1, 3). Researchers estimate that the risk of melanoma is about 10 times greater for someone with more than five dysplastic nevi than for someone who has none.

Is nevus a disease? ›

Nevus (plural: nevi) is the medical term for a mole. Nevi are very common. Most people have between 10 and 40. Common nevi are harmless collections of colored cells.

What is inside nevus comedonicus? ›

Nevus comedonicus (NC), first described by Kofmann in 1895, is a rare condition considered to be a type of epidermal nevus. Nevus comedonicus presents as grouped, dilated follicular openings with dark keratin plugs.

What is the histology of acne Keloidalis? ›

Histology of folliculitis keloidalis nuchae

In the dermis are disrupted hair follicles with scattered naked hair shafts seen within a fibrotic dermis (Figures 2 and 3). There is a dense lymphoplasmacytic infiltrate with scattered neutrophils (Figure 4).

What is the histology of nevus of Ito? ›

Histologic findings for nevi of Ota and Ito have some similarities. Overlying epidermis is normal. In the papillary and upper reticular dermis, dendritic melanocytes are present and surrounded by fibrous sheaths (which are not present in other dermal melanocytosis, such as blue nevus or Mongolian spots).

What is the histology of histopathology darier disease? ›

Histology of Darier disease

Acantholysis can be seen at all levels within the epidermis. Dyskeratosis of the keratinocytes is seen, with two notable changes described. Corps ronds refer to cells with small pyknotic nuclei, a perinuclear clear halo and eosinophilic cytoplasm (Figures 4 and 5).

References

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