Nevus Comedonicus


Article Author:
Feroze Kaliyadan


Article Editor:
Karalikkattil Ashique


Editors In Chief:
Amanda Oakley
Jules Lipoff
Shyam Verma


Managing Editors:
Avais Raja
Orawan Chaigasame
Carrie Smith
Abdul Waheed
Khalid Alsayouri
Trevor Nezwek
Radia Jamil
Patrick Le
Anoosh Zafar Gondal
Saad Nazir
William Gossman
Hassam Zulfiqar
Steve Bhimji
John Shell
Matthew Varacallo
Heba Mahdy
Ahmad Malik
Sarosh Vaqar
Mark Pellegrini
James Hughes
Beata Beatty
Nazia Sadiq
Hajira Basit
Phillip Hynes
Tehmina Warsi


Updated:
6/27/2019 12:15:44 PM

Introduction

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. Kofmann initially had suggested labeling the condition 'comedo nevus.' [1]

Etiology

The exact etiology has not been clarified. Nevus comedonicus is considered to be an epidermal nevus developing from the hair follicle. The other opinion is that it is a hamartoma derived from the mesodermal part of the pilosebaceous unit. Genetic mosaicism has been proposed to be part of etiology of nevus comedonicus.[2][3]

Epidemiology

The prevalence has been reported to be between 1 in 45,000 to 1 in 100,000. There is no specific gender predilection; males and females can be equally affected. No increased incidence within a specific ethnicity has been reported. Most of the cases develop before the age of ten years.[4][5][6]

Pathophysiology

A mutation of Fibroblast Growth Factor Receptor 2 (FGFR2) along with increased expression of interleukin-1-alpha is considered an important factor in the pathogenesis. Other possible factors include gamma-secretase and filaggrin. Recent studies have highlighted the importance of somatic mutations in NEK 9 in nevus comedonicus. NEK9 has been postulated to be important in the regulation of follicular homeostasis. The NEK 9 mutations are associated with increased phosphorylation at Thr210, indicating activation of NEK9 associated kinase. The formation of the comedones in nevus comedonicus also has been associated with other changes such as loss of markers of follicular differentiation and ectopic expression of keratin 10. A recent study has suggested a role for upregulation of ABCA 12 in nevus comedonicus.[2][1][3]

Histopathology

 skin biopsy shows the typical features of comedones -dilated follicular ostia filled with keratin.[4][1]

History and Physical

NC develops shortly after birth in about half of patients, and most patients develop the lesions before the age of ten years. The most common affected sites are the face, neck, upper arms, chest, and abdomen. Sites such as the scalp are affected quite rarely. Other rare but reported sites of involvement include the palms and the glans penis. The usual presentation is in the form of a single group of dilated and plugged follicular ostia in a honeycomb pattern. Other patterns seen include linear, segmental, or Blaschkoid. The lesions may rarely be bilateral. Two specific subtypes have been described: (1) a non-pyogenic type and (2) a type associated with the formation of cysts, pustules, and abscesses. Nevus comedonicus can occur in isolation or in combination with systemic abnormalities in the central nervous system, skeletal system, another skin manifestation, or ocular and dental abnormalities. Combination subtypes are known as nevus comedonicus syndrome. [1][7][4] [8] [9][10]

Evaluation

The diagnosis is usually clinical. Detailed evaluation of the central nervous system, skeletal system, and the eyes is required in suspected cases of NC syndrome. A skin biopsy shows the typical dilated follicular ostia filled with keratin. Immunohistochemistry studies have shown an increased expression of proliferating cell nuclear antigen, intercellular adhesion molecule-1 (ICAM-1), HLA-DR, and CD68. Electron microscopy has shown an increased number of Langerhans cells. Usually the clinical diagnosis is quite obvious with the history of early age of onset and the typical morphology, but in case of atypical presentations other conditions to consider as differentials include atypical acne (e.g., segmental acne and other mosaic acneiform conditions), other acneiform conditions such as chloracne, Favre- Racouchot syndrome (i.e., nodular elastosis with cysts and comedones), and familial dyskeratotic comedones. This last is a relatively rare, autosomal dominantly inherited condition characterized by hyperkeratotic, comedonal lesions. Histological examination shows comedones with associated dyskeratosis. [11]

Recently, dermoscopy has been reported to be useful in the diagnosis of nevus comedonicus. Dermoscopy highlights the typical comedonal lesions. The typical dermoscopy findings described include multiple light and dark brown, circular or barrel shaped homogenous areas with prominent keratin plugs.[12][13]

Treatment / Management

Spontaneous resolution of nevus comedonicus has not been described; however, aggressive treatment is not recommended as nevus comedonicus is essentially a benign condition. Treatment is mainly reserved for cosmetic reasons or when there are complications such as the formation of cysts or abscesses. Cosmetic treatment for the post-inflammatory scarring also might be required. Treatment options include topical and systemic retinoids. These sometimes are combined with topical steroids for their anti-inflammatory action, salicylic acid, or 12% ammonium lactate. Surgical excision and ablative lasers have been tried in NC. Newer treatment avenues being explored include FGFR inhibitors, interleukin-1-alpha inhibitors, and anti-gamma-secretase drugs. Successful treatment of the keratin plugs with a pore strip has been described. Lasers have been described to be effective in the treatment of nevus comedonicus. Ultrapulse carbondioxide lasers and erbium-YAG lasers have been reported to effectively treat lesions of nevus comedonicus. A recent report has mentioned the use of a 1450 nm diode laser in treating nevus comedonicus. It is postulated that sublesional collagen stimulation by the laser might lead to a reduction of the epidermal invaginations leading to an improvement in the skin texture.[14][15][16][1][17][18]

Pearls and Other Issues

Several associations have been reported along with nevus comedonicus and as part of the nevus comedonicus syndrome. Nevus comedonicus syndrome refers to nevus comedonicus presenting with extra cutaneous manifestations usually involving the central nervous system, skeletal system, teeth, and eyes.  Common central nervous system abnormalities reported include- seizure disorders, delayed mental development,  electroencephalogram abnormalities, microcephaly and transverse myelitis. Skeletal abnormalities include syndactyly, supernumerary digits, scoliosis, and spina bifida. The most common ocular manifestation described is a cataract. Oligodontia is the most reported dental abnormality.  Nevus comedonicus syndrome is considered to be a part of the epidermal nevus syndrome which covers extra cutaneous manifestations occurring in association with conditions like nevus comedonicus, verrucous epidermal nevus, and nevus sebaceous. The other syndromes considered to be part of the group of epidermal nevus syndromes include Schimmelpenning syndrome, phacomatosis pigmentokeratotica, angora hair nevus syndrome, and Becker nevus syndrome.[19] [1][20][21][22]

Hidradenitis suppurative can occur along with nevus comedonicus. Hidradenitis suppurative-like lesions complicating nevus comedonicus have been described and mechanical stress has been postulated as a trigger for their development. Other rare systemic associations reported with nevus comedonicus include Alagille syndrome, spinal dysraphism, hypothyroidism, and Paget bone disease. Primary dermatological conditions occurring in association with nevus comedonicus include basal cell carcinoma, squamous cell carcinoma, keratoacanthoma, linear morphea, lichen striatus, accessory breast tissue, epidermolytic hyperkeratosis, nevoid hyperkeratosis of areola, hidradenoma papilliform, syringocystadenoma papilliform, epidermoid cysts, cutaneous horns, hemangiomas, ichthyosis, leukoderma, pilar sheath tumor, dilated pore of Winer, trichoepithelioma, and other epidermal nevi.[23][24][25]

Enhancing Healthcare Team Outcomes

It is important to look for features of nevus comedonicus syndrome in any patient presenting with lesions suggestive of nevus comedonicus. Patients must be counseled regarding the possibility of intermittent flares. As of now there are no clear randomized control trials to clearly predict treatment success with any particular modality, but patients must be made aware of all available options so that an informed decision can be made.


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    Contributed by Feroze Kaliyadan, MD
Attributed To: Contributed by Feroze Kaliyadan, MD

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Nevus Comedonicus - Questions

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A 7 year old male child presents to the dermatology out-patient department with grouped, linear papules over the right scapular area. The lesions developed soon after birth and have been asymptomatic. On examination dilated follicular lesions with keratinous plugs are seen. Mutations in which of the following is considered to be involved in the pathogenesis of this condition?



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Nevus comedonicus has been considered to mosaic form of which of the following syndromes?



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A 9 year old male child presents to the dermatology out-patient department with grouped, linear papules over the right scapular area. The lesions developed soon after birth and have been asymptomatic. On examination dilated follicular lesions with keratinous plugs are seen. Which system is least likely expect?



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A 7-year-old male child presents to the clinic with asymptomatic raised skin lesions over the right upper arm, present since the age of 1 year. On examination, grouped, dilated and plugged follicular lesions are seen. A skin biopsy shows dilated follicular Ostia with keratin. Which of the following is most likely to be seen on the immunohistochemistry of the skin biopsy specimen?



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A 16-year-old male presents to the clinic with raised skin lesions over his left axilla and left pinna, present since birth. There is a history of occasional purulent discharge from the lesions. There is no history of any other skin or systemic disease. On examination, grouped comedones are seen on both the sites. Which of the following laser will be most effective in treating the skin lesions?



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A 17-year-old male patient presents to the clinic with recurrent purulent discharge localized to the left side of his neck, since birth. There is no history of any other skin or systemic disease. On examination, grouped pustules and comedones in a linear configuration are seen localized to the left side of the neck. A skin biopsy shows dilated follicular ostia filled with keratin and some mixed inflammatory infiltrate. Which of the following classes of medications has been found to be most useful in the treatment of such cases?



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A 13-year-old female child presents to the clinic with a history of asymptomatic, raised lesions over her thighs, present since birth. There is no history of any other significant skin or systemic disease. On examination, grouped comedones are seen over the site, and a skin biopsy shows dilated follicular ostia filled with keratin. Electron microscopy shows an increased number of Langerhans cells. Which of the following is the most likely pattern expected to be seen on dermoscopy of the skin lesions?



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Nevus Comedonicus - References

References

Nevus comedonicus of the scalp., Kaliyadan F,Nambiar A,Al Ameer A,Amri M,, Skinmed, 2014 Jan-Feb     [PubMed]
Nevus comedonicus: an updated review., Tchernev G,Ananiev J,Semkova K,Dourmishev LA,Schönlebe J,Wollina U,, Dermatology and therapy, 2013 Jun     [PubMed]
Ultrastructural features of nevus comedonicus., Cho SB,Oh SH,Lee JH,Bang D,Bang D,, International journal of dermatology, 2012 May     [PubMed]
Nevus comedonicus syndrome--nevus comedonicus associated with ipsilateral polysyndactyly and bilateral oligodontia., Kaliyadan F,Nampoothiri S,Sunitha V,Kuruvilla VE,, Pediatric dermatology, 2010 Jul-Aug     [PubMed]
Successful treatment to a tretinoin/clindamycin gel in a late onset of nevus comedonicus., Capusan TM,Chicharro P,Rodriguez-Jimenez P,Martinez-Mera C,Urquía A,Aragüés M,de Argila D,, Dermatologic therapy, 2017 Mar 14     [PubMed]
Ultrapulse carbon dioxide laser treatment for bilateral facial nevus comedonicus: A case report., Zhu C,Sun A,, Dermatologic therapy, 2017 Feb 15     [PubMed]
Bilateral nevus comedonicus of the eyelids associated with bladder cancer and successful treatment with topical tretinoin., Polat M,Altunay Tuman B,Sahin A,Dogan U,Boran C,, Dermatologic therapy, 2016 Nov     [PubMed]
Somatic Mutations in NEK9 Cause Nevus Comedonicus., Levinsohn JL,Sugarman JL,McNiff JM,Antaya RJ,Choate KA,, American journal of human genetics, 2016 May 5     [PubMed]
Dermoscopy on nevus comedonicus: a case report and review of the literature., Kamińska-Winciorek G,Spiewak R,, Postepy dermatologii i alergologii, 2013 Aug     [PubMed]
Bilateral nevus comedonicus syndrome., Ito T,Mitamura Y,Tsuji Y,Harada K,Urabe K,, Yonago acta medica, 2013 Jun     [PubMed]
Linear morhpea, nevus comedonicus, and lichen striatus in a 5-year-old girl., Sinha A,Natarajan S,, Pediatric dermatology, 2011 Jan-Feb     [PubMed]
Nevus comedonicus syndrome in a woman with Paget bone disease and breast cancer: a mere coincidence?, Martinez M,Levrero P,Bazzano C,Larre Borges A,De Anda G,, European journal of dermatology : EJD, 2006 Nov-Dec     [PubMed]
Nevus comedonicus with hidradenoma papilliferum and syringocystadenoma papilliferum in the female genital area., Lee HJ,Chun EY,Kim YC,Lee MG,, International journal of dermatology, 2002 Dec     [PubMed]
Naevus comedonicus as dermatologic hallmark of occult spinal dysraphism., Filosa G,Bugatti L,Ciattaglia G,Salaffi F,Carotti M,, Acta dermato-venereologica, 1997 May     [PubMed]
Extensive naevus comedonicus in a child with Alagille syndrome., Woods KA,Larcher VF,Harper JI,, Clinical and experimental dermatology, 1994 Mar     [PubMed]
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Vidaurri-de la Cruz H,Tamayo-Sánchez L,Durán-McKinster C,de la Luz Orozco-Covarrubias M,Ruiz-Maldonado R, Epidermal nevus syndromes: clinical findings in 35 patients. Pediatric dermatology. 2004 Jul-Aug;     [PubMed]
Zanniello R,Pilloni L,Conti B,Faa G,Rongioletti F, Late-Onset Nevus Comedonicus With Follicular Epidermolytic Hyperkeratosis-Case Report and Review of the Literature. The American Journal of dermatopathology. 2018 Nov 15;     [PubMed]
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