Nevus Comedonicus 

  • Author: Rossitza Lazova, MD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Jul 7, 2010
 

Background

In 1895, Kofmann[1] described the first case of nevus comedonicus. It manifests as groups of closely set, dilated follicular openings with dark keratin plugs resembling comedones. The majority of cases are isolated. However, nevus comedonicus may be part of nevus comedonicus syndrome in association with skeletal or central nervous system anomalies, ocular abnormalities, and cutaneous defects.[2, 3, 4]

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Pathophysiology

Many consider nevus comedonicus to be a hamartoma deriving from a failure of the mesodermal part of the folliculosebaceous unit to develop properly, with subsequent abnormal differentiation of the epithelial portion. The follicular structures that result are unable to form terminal hair or sebaceous glands and are capable only of producing soft keratin, which accumulates in the adnexal orifices and produces the comedonelike lesions observed in persons with this condition. Another view is that nevus comedonicus is an epidermal nevus involving hair follicles or an appendageal nevus of sweat ducts. Lesions that extend onto a palm or sole typically demonstrate sweat duct dilatation with keratin in the volar portion of the lesion. See Epidermal Nevus Syndrome for more information.

The etiology of nevus comedonicus is unclear. Why some nevus comedonicus patients present late in life is not known, although a genetic mosaicism has been proposed. While the majority of cases are sporadic, several families with this condition have been documented. Only one report has described nevus comedonicus occurring in homozygous twins.[5]

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Epidemiology

Frequency

United States

Exact figures are lacking. 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. The incidence of nevus comedonicus syndrome is even more difficult to estimate; it is considered less common than nonsyndromal nevus comedonicus.

Mortality/Morbidity

Most patients are asymptomatic. Uncommonly, the lesions become repeatedly inflamed and infected, leading to painful cysts, abscesses, fistula formation, and scarring. Additionally, patients may be distressed over the cosmetic appearance of the lesions.

Race

No racial predilection is recognized.

Sex

Males and females are equally affected.

Age

Approximately 50% of cases of nevus comedonicus are evident at birth, with the other 50% developing during childhood, usually before age 10 years. A few case reports describe onset later in life, including in the seventh decade. These cases usually occur after some form of trauma[6] or a rash.

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Contributor Information and Disclosures
Author

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, and International Society of Dermatopathology

Disclosure: Nothing to disclose.

Specialty Editor Board

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.

Michael J Wells, MD  Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center

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

Disclosure: Nothing to disclose.

Catherine M Quirk, MD  Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania

Catherine M Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD  Director, Department of Dermatology, Geisinger Medical Center

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

References
  1. Kofmann S. A case of rare localization and spreading of comedones. Arch Dermatol Syphilol. 1895;32:177-8.

  2. Patrizi A, Neri I, Fiorentini C, Marzaduri S. Nevus comedonicus syndrome: a new pediatric case. Pediatr Dermatol. Jul-Aug 1998;15(4):304-6. [Medline].

  3. Engber PB. The nevus comedonicus syndrome: a case report with emphasis on associated internal manifestations. Int J Dermatol. Nov 1978;17(9):745-9. [Medline].

  4. 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. Nov-Dec 2006;16(6):697-8. [Medline].

  5. Giam YC, Ong BH, Rajan VS. Naevus comedonicus in homozygous twins. Dermatologica. 1981;162(4):249-53. [Medline].

  6. Grimalt R, Caputo R. Posttraumatic nevus comedonicus. J Am Acad Dermatol. Feb 1993;28(2 Pt 1):273-4. [Medline].

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  13. Nabai H, Mehregan AH. Nevus comedonicus. A review of the literature and report of twelve cases. Acta Derm Venereol. 1973;53(1):71-4. [Medline].

  14. Wood MG, Thew MA. Nevus comedonicus. A case with palmar involvement and review of the literature. Arch Dermatol. Aug 1968;98(2):111-6. [Medline].

  15. Abdel-Aal H, Abdel-Aziz AM. Nevus comedonicus. Report of three cases localized on glans penis. Acta Derm Venereol. 1975;55(1):78-80. [Medline].

  16. Beck MH, Dave VK. Extensive nevus comedonicus. Arch Dermatol. Sep 1980;116(9):1048-50. [Medline].

  17. Paige TN, Mendelson CG. Bilateral nevus comedonicus. Arch Dermatol. Aug 1967;96(2):172-5. [Medline].

  18. Schecter AK, Lester B, Pan TD, Robinson-Bostom L. Linear nevus comedonicus with epidermolytic hyperkeratosis. J Cutan Pathol. Aug 2004;31(7):502-5. [Medline].

  19. Sharma RP, Singh SP. Extensive unilateral nevus comedonicus with bilateral involvement of face. Indian J Dermatol Venereol Leprol. Jul-Aug 2001;67(4):195-6. [Medline].

  20. Woods KA, Larcher VF, Harper JI. Extensive naevus comedonicus in a child with Alagille syndrome. Clin Exp Dermatol. Mar 1994;19(2):163-4. [Medline].

  21. Bhagwat PV, Tophakhane RS, Rathod RM, Tonita NM, Naidu V. Nevus comedonicus along Blaschko's lines. Indian J Dermatol Venereol Leprol. May-Jun 2009;75(3):330. [Medline].

  22. Decherd JW, Mills O, Leyden JJ. Naevus comedonicus--treatment with retinoic acid. Br J Dermatol. May 1972;86(5):528-9. [Medline].

  23. Milton GP, DiGiovanna JJ, Peck GL. Treatment of nevus comedonicus with ammonium lactate lotion. J Am Acad Dermatol. Feb 1989;20(2 Pt 2):324-8. [Medline].

  24. Caers SJ, Van der Geer S, Beverdam EG, Krekels GA, Ostertag JU. Successful treatment of nevus comedonicus with the use of the Erbium Yag laser. J Eur Acad Dermatol Venereol. Mar 2008;22(3):375-7. [Medline].

  25. Sardana K, Garg V. Successful treatment of nevus comedonicus with ultrapulse CO2 laser. Indian J Dermatol Venereol Leprol: 2009; 75(5):534-5. [Medline].

  26. Deliduka SB, Kwong PC. Treatment of Nevus comedonicus with topical tazarotene and calcipotriene. J Drugs Dermatol. Nov-Dec 2004;3(6):674-6. [Medline].

  27. Peck GL, Yoder FW. Treatment of lamellar ichthyosis and other keratinising dermatoses with an oral synthetic retinoid. Lancet. Nov 27 1976;2(7996):1172-4. [Medline].

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