Close
New

Medscape is available in 5 Language Editions – Choose your Edition here.

 

Nevus Comedonicus Clinical Presentation

  • Author: Rossitza Lazova, MD; Chief Editor: William D James, MD  more...
 
Updated: Jun 27, 2016
 

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.[7, 8, 9] Reports from 2015 and 2016 describe nevus comedonicus syndrome complicated by hidradenitis suppurativa‒like lesions.[10, 11]

Next

Physical

The lesion appears as a collection of discrete, dilated follicular ostia plugged with pigmented keratinaceous material.[12, 13] 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.[14, 15, 16, 17] Rarely, it has been described on the palms[18] and soles, scalp,[19, 20] or penis.[21] When it occurs on the elbows and knees, it can appear as verrucous nodules.

Nevus comedonicus may be linear, interrupted, unilateral, bilateral, present in a dermatomal distribution, following the lines of Blaschko, or segmental.[9, 22, 23, 24, 25, 26, 27]

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)[28]
  • Other - Bilateral oligodontia,[29] multiple basal cell carcinomas, rare systemic malignancies
Previous
Next

Causes

The cause is unknown. However, whole-exome sequencing in nevus comedonicus identified somatic NEK9 mutations, each affecting highly conserved residues within its kinase or RCC1 domains.[30] All mutations were gain of function, resulting in increased phosphorylation at Thr210, a hallmark of NEK9 kinase activation. The authors found that comedo formation in nevus comedonicus is marked by loss of follicular differentiation markers, expansion of keratin-15‒positive cells from localization within the bulge to the entire sub-bulge follicle and cyst, and ectopic expression of keratin 10, a marker of interfollicular differentiation not present in normal follicles. These findings suggest that NEK9 mutations in nevus comedonicus disrupt normal follicular differentiation and identify NEK9 as a potential regulator of follicular homeostasis.[30]

Previous
Next

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.

Previous
 
 
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, International Society of Dermatopathology

Disclosure: Nothing to disclose.

Specialty Editor Board

Michael J Wells, MD, FAAD Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine

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: Nothing to disclose.

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.

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.

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. 1998 Jul-Aug. 15(4):304-6. [Medline].

  3. Engber PB. The nevus comedonicus syndrome: a case report with emphasis on associated internal manifestations. Int J Dermatol. 1978 Nov. 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. 2006 Nov-Dec. 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. 1993 Feb. 28(2 Pt 1):273-4. [Medline].

  7. Vasiloudes PE, Morelli JG, Weston WL. Inflammatory nevus comedonicus in children. J Am Acad Dermatol. 1998 May. 38(5 Pt 2):834-6. [Medline].

  8. Kirtak N, Inaloz HS, Karakok M, Erguven HG, Ozgoztasi O. Extensive inflammatory nevus comedonicus involving half of the body. Int J Dermatol. 2004 Jun. 43(6):434-6. [Medline].

  9. Asokan N, Sridevi PK. Inflammatory nevus comedonicus in an infant. Nov-Dec/2008.

  10. 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. [Medline].

  11. 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. [Medline].

  12. Sikorski D, Parker J, Shwayder T. A boy with an unusual scalp birthmark. Nevus Comedonicus. Int J Dermatol. 2011 Jun. 50(6):670-2. [Medline].

  13. Arias-Santiago S, Aneiros-Fernandez J, Buendia-Eisman A, Giron-Prieto MS, Aneiros-Cachaza J. Unknown: A 5-year-old boy with comedo-like lesions on the right buttock. Dermatology Online Journal. 16:11. [Medline]. [Full Text].

  14. Ghaninezhad H, Ehsani AH, Mansoori P, Taheri A. Naevus comedonicus of the scalp. J Eur Acad Dermatol Venereol. 2006 Feb. 20(2):184-5. [Medline].

  15. Guldbakke KK, Khachemoune A, Deng A, Sina B. Naevus comedonicus: a spectrum of body involvement. Clin Exp Dermatol. 2007 Sep. 32(5):488-92. [Medline].

  16. Lefkowitz A, Schwartz RA, Lambert WC. Nevus comedonicus. Dermatology. 1999. 199(3):204-7. [Medline].

  17. 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].

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

  19. Kaliyadan F, Nambiar A, Al Ameer A, Amri M. Nevus comedonicus of the scalp. Skinmed. 2014 Jan-Feb. 12(1):59-60. [Medline].

  20. Kikkeri NN, Priyanka R, Parshawanath H. Nevus comedonicus on scalp: a rare site. Indian J Dermatol. 2015 Jan-Feb. 60 (1):105. [Medline].

  21. 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].

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

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

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

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

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

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

  28. Pavithra S, Pai H, Mallya H, Pai GS. Nevus comedonicus syndrome. Indian J Dermatol. 2011 Nov. 56(6):771-2. [Medline]. [Full Text].

  29. Kaliyadan F, Nampoothiri S, Sunitha V, Kuruvilla VE. Nevus comedonicus syndrome--nevus comedonicus associated with ipsilateral polysyndactyly and bilateral oligodontia. Pediatr Dermatol. 2010 Jul-Aug. 27(4):377-9. [Medline].

  30. 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. [Medline].

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

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

  33. 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. 2008 Mar. 22(3):375-7. [Medline].

  34. Sardana K, Garg V. Successful treatment of nevus comedonicus with ultrapulse CO2 laser. 2009; 75(5):534-5.

Previous
Next
 
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2016 by WebMD LLC. This website also contains material copyrighted by 3rd parties.