History

The lesions are typically present at birth or develop in early childhood. They are usually asymptomatic. However, one case report described itching as an accompanying symptom. Patients usually seek help for cosmetic reasons. The lesions grow as the patient does and often grow faster at puberty. Patients may be able to express keratinaceous material from the pores.

With the inflammatory form, repeated bacterial infections, drainage, cysts, fistula and abscess formation, and scarring may develop.^{[9, 10, 11]}Reports from 2015 and 2016 describe nevus comedonicus syndrome complicated by hidradenitis suppurativa‒like lesions.^{[12, 13]}More recent reports detail cases of nevus comedonicus on the scalp with late-onset presentation.^{[14, 15]}

Physical Examination

The lesion appears as a collection of discrete, dilated follicular ostia plugged with pigmented keratinaceous material.^{[16, 17]}Dermoscopy displays multiple, well-defined structureless brown homogenous circular pores with keratin plugs.^[18]They can be single or multiple, usually unilateral, and range in size from a few centimeters to involving half of the entire body.

Nevus comedonicus is typically found on the face, trunk, neck, and upper extremities.^{[19, 20, 21, 22]}Rarely, it has been described on the palms^[23]and soles, scalp,^{[24, 25]}or penis.^[26]When it occurs on the elbows and knees, it can appear as verrucous nodules. When it occurs in the intertriginous zones, the mechanical stress can produce hidradenitis suppurativa–like lesions.^[27]

Nevus comedonicus may be linear, interrupted, unilateral, bilateral, present in a dermatomal distribution, following the lines of Blaschko, or segmental.^{[11, 28, 29, 30, 31, 32, 33]}

Nevus comedonicus syndrome is the association of nevus comedonicus with abnormalities in the central nervous system, skeletal system, skin, and eyes as follows:

Central nervous system - Epilepsy, electroencephalogram abnormalities, transverse myelitis, microcephaly
Skeletal system - Scoliosis, hemivertebrae, spina bifida occulta, foot deformities, absent fifth finger, syndactyly, supernumerary digits
Skin - Ichthyosis, trichilemmal cysts, leukoderma, white hairs, Sturge-Weber syndrome, hemangiomas, linear basal cell nevus
Eye - Congenital cataracts (unilateral and bilateral)^[34]
Other - Bilateral oligodontia,^[35]multiple basal cell carcinomas, rare systemic malignancies

Complications

Persons with inflammatory nevus comedonicus can develop cysts, recurrent bacterial infections, fistulae, and abscesses, and these may subsequently heal with scarring. Treat these lesions with appropriate antibiotics or surgical drainage. Infections may be recurrent.

Differential Diagnoses

Kofmann S. A case of rare localization and spreading of comedones. Arch Dermatol Syphilol. 1895. 32:177-8.
Patrizi A, Neri I, Fiorentini C, Marzaduri S. Nevus comedonicus syndrome: a new pediatric case. Pediatr Dermatol. 1998 Jul-Aug. 15(4):304-6. [QxMD MEDLINE Link].
Engber PB. The nevus comedonicus syndrome: a case report with emphasis on associated internal manifestations. Int J Dermatol. 1978 Nov. 17(9):745-9. [QxMD MEDLINE Link].
Martinez M, Levrero P, Bazzano C, Larre Borges A, De Anda G. Nevus comedonicus syndrome in a woman with Paget bone disease and breast cancer: a mere coincidence?. Eur J Dermatol. 2006 Nov-Dec. 16(6):697-8. [QxMD MEDLINE Link].
Giam YC, Ong BH, Rajan VS. Naevus comedonicus in homozygous twins. Dermatologica. 1981. 162(4):249-53. [QxMD MEDLINE Link].
Levinsohn JL, Sugarman JL, Yale Center for Mendelian Genomics, McNiff JM, Antaya RJ, Choate KA. Somatic Mutations in NEK9 Cause Nevus Comedonicus. Am J Hum Genet. 2016 May 5. 98 (5):1030-7. [QxMD MEDLINE Link].
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Bettoli V, Toni G, Ricci M, Zauli S, Virgili A. Hidradenitis suppurativa-acne inversa-like lesions complicating naevus comedonicus: second case supporting the mechanical stress as a triggering factor. G Ital Dermatol Venereol. 2016 Jun. 151 (3):306-7. [QxMD MEDLINE Link].
Qian G, Liu T, Zhou C, Zhang Y. Naevus comedonicus syndrome complicated by hidradenitis suppurativa-like lesions responding to acitretin treatment. Acta Derm Venereol. 2015 Nov. 95 (8):992-3. [QxMD MEDLINE Link].
Mathachan SR, Arora P, Ahuja A, Gautam RK. Late onset nevus comedonicus of scalp: a rare site for an uncommon condition. Dermatol Online J. 2019 Mar 15. 25 (3):[QxMD MEDLINE Link].
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Media Gallery

Nevus comedonicus. Courtesy of DermNet New Zealand (http://www.dermnetnz.org/assets/Uploads/lesions/com-naev1.jpg).

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Author

Marjon Vatanchi, MD Board Certified Dermatologist, Board Certified Micrographic Dermatologic Surgeon

Marjon Vatanchi, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, American Society for Laser Medicine and Surgery, Dermatologic Society of Greater New York, Medical Dermatology Society, National Psoriasis Foundation, New England Dermatological Society, Society for Pediatric Dermatology, Women's Dermatologic Society

Disclosure: Nothing to disclose.

Coauthor(s)

Ally-Khan B Somani, MD, PhD Assistant Professor of Dermatology, Director of Dermatologic Surgery and Cutaneous Oncology Division, Director of Micrographic Surgery and Dermatologic Oncology (MSDO) Fellowship, Department of Dermatology, Adjunct Assistant Professor of Otolaryngology-Head and Neck Surgery, Indiana University Health, Indiana University School of Medicine

Ally-Khan B Somani, MD, PhD is a member of the following medical societies: American Academy of Cosmetic Surgery, American Academy of Dermatology, American College of Mohs Surgery, American Society for Dermatologic Surgery, American Society for Laser Medicine and Surgery, Association for the Advancement of Wound Care, Association of Professors of Dermatology, Dermatologic and Aesthetic Surgery International League, European Academy of Dermatology and Venereology, European Society for Micrographic Surgery, International Society for Dermatologic Surgery, International Society of Dermatology, International Transplant and Skin Cancer Collaborative, Society for Investigative Dermatology, Skin Cancer Foundation

Disclosure: Nothing to disclose.

Specialty Editor Board

Michael J Wells, MD, FAAD Dermatologic/Mohs Surgeon, The Surgery Center at Plano Dermatology

Michael J Wells, MD, FAAD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, Texas Medical Association

Disclosure: Nothing to disclose.

Lester F Libow, MD Dermatopathologist, South Texas Dermatopathology Laboratory

Lester F Libow, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, Texas Medical Association

Disclosure: Nothing to disclose.

Chief Editor

William D James, MD Paul R Gross Professor of Dermatology, Vice-Chairman, Residency Program Director, Department of Dermatology, University of Pennsylvania School of Medicine

William D James, MD is a member of the following medical societies: American Academy of Dermatology, Society for Investigative Dermatology

Disclosure: Received income in an amount equal to or greater than $250 from: Elsevier; WebMD<br/>Served as a speaker for various universities, dermatology societies, and dermatology departments.

Additional Contributors

Barbara R Reed, MD Clinical Professor, Department of Dermatology, Dermatology Service, Denver Veterans Affairs Medical Center, University of Colorado Health Sciences Center; Consulting Staff, Denver Skin Clinic

Disclosure: Nothing to disclose.

Rossitza Lazova, MD Associate Professor of Dermatology and Pathology, Director of Dermatopathology Residency and Fellowship Program, Yale University School of Medicine; Consulting Pathologist/Dermatopathologist, Veterans Affairs Medical Center, West Haven, Connecticut

Rossitza Lazova, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, International Society of Dermatopathology

Disclosure: Nothing to disclose.

Acknowledgements

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous authors Joseph J. Shaffer, MBBS, Vincent A. de Leo, MD, to the development and writing of this article.

Nevus Comedonicus Clinical Presentation

History

Physical Examination

Complications

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