Document Type : Case Reports


1 Department of Dermatology, Imam Reza Hospital, Mashhad University of Medical Sciences, Mashhad, Iran

2 Department of Pathology, Imam Reza Hospital, Mashhad University of Medical Sciences, Mashhad, Iran


Nevus comedonicus (NC) is a rare developmental anomaly of the folliculosebaceous apparatus, which appears as numerous dilated papules containing firm, darkly pigmented, horny plugs. It appears shortly after birth and mostly before the age of 10; however, late-onset cases have been reported. There is no gender or racial predilection. Moreover, NC can be a component of nevus comedonicus syndrome, a neurocutaneous disorder with skeletal, ocular, and central nervous system abnormalities. EHK properties in NC are not a common finding and are rarely seen in association with each other. This paper reports a healthy, 27-year-old young woman who has been developing numbers of asymptomatic unilateral linear skin lesions on her chest, waist, right thigh, and popliteal fossa in a unilateral linear pattern over ten years. Skin biopsy revealed dilated follicular ostia with orthokeratotic hyperkeratosis, columns of parakeratosis, cornoid flagellation, epidermolytic hyperkeratosis, and mild acanthosis on its wall.


Main Subjects

  1. Tchernev G, Ananiev J, Semkova K, Dourmishev LA, Schonlebe J, Wollina U. Nevus comedonicus: an updated review. Dermatol Ther (Heidelb). 2013;3(1):33-40. [DOI:10.1007/s13555-013-0027-9] [PMID] [PMCID]
  2. Nabai H, Mehregan AH. Nevus comedonicus. A review of the literature and report of twelve cases. Acta Derm Venereol. 1973;53(1):71-4.
  3. Patrizi A, Neri I, Fiorentini C, Marzaduri S. Nevus comedonicus syndrome: a new pediatric case. Pediatr Dermatol. 1998;15(4):304-6. [DOI:10.1046/j.1525-1470.1998.1998015304.x] [PMID]
  4. Happle R. The group of epidermal nevus syndromes Part I. Well defined phenotypes. J Am Acad Dermatol. 2010;63(1):1-22; quiz 3-4. [DOI:10.1016/j.jaad.2010.01.017] [PMID]
  5. Melnik B, Schmitz G. FGFR2 signaling and the pathogenesis of acne. J Dtsch Dermatol Ges. 2008;6(9):721-8. [DOI:10.1111/j.1610-0387.2008.06822.x] [PMID]
  6. Pan Y, Lin MH, Tian X, Cheng HT, Gridley T, Shen J, et al. gamma-secretase functions through Notch signaling to maintain skin appendages but is not required for their patterning or initial morphogenesis. Dev Cell. 2004;7(5):731-43. [DOI:10.1016/j.devcel.2004.09.014] [PMID]
  7. Schecter AK, Lester B, Pan TD, Robinson-Bostom L. Linear nevus comedonicus with epidermolytic hyperkeratosis. J Cutan Pathol. 2004;31(7):502-5. [DOI:10.1111/j.0303-6987.2004.00206.x] [PMID]
  8. DiGiovanna JJ, Bale SJ. Epidermolytic hyperkeratosis: applied molecular genetics. J Invest Dermatol. 1994;102(3):390-4. [DOI:10.1111/1523-1747.ep12371801] [PMID]
  9. Aloi FG, Molinero A. Nevus comedonicus with epidermolytic hyperkeratosis. Dermatologica. 1987;174(3):140-3. [DOI:10.1159/000249006] [PMID]
  10. Lee S, Nasemann T, Neufahrt A. [Histogenesis of nevus comedonicus. Histochemical, light and electron microscopy studies]. Hautarzt. 1972;23(12):534-40.
  11. Kurokawa I, Nakai Y, Nishimura K, Hakamada A, Isoda K, Yamanaka K, et al. Cytokeratin and filaggrin expression in nevus comedonicus. J Cutan Pathol. 2007;34(4):338-41. [DOI:10.1111/j.1600-0560.2006.00619.x] [PMID]
  12. Wollina U. Histochemistry of the human hair follicle. Exs. 1997;78:31-58. [DOI:10.1007/978-3-0348-9223-0_2] [PMID]
  13. Barsky S, Doyle JA, Winkelmann RK. Nevus comedonicus with epidermolytic hyperkeratosis. A report of four cases. Arch Dermatol. 1981;117(2):86-8. [DOI:10.1001/archderm.1981.01650020028019] [PMID]
  14. Lookingbill DP, Ladda RL, Cohen C. Generalized epidermolytic hyperkeratosis in the child of a parent with nevus comedonicus. Arch Dermatol. 1984;120(2):223-6. [DOI:10.1001/archderm.1984.01650380083017] [PMID]
  15. 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. Am J Dermatopathol. 2019;41(6):453-5. [DOI:10.1097/DAD.0000000000001317] [PMID]