Skip to main content

Keratinous Cysts and Hamartomas

  • Chapter
  • First Online:
Pediatric Dermatopathology

Abstract

There are many cysts and hamartomatous growths that present during childhood. The clinical and histologic features of these lesions are described in this chapter, along with discussions of pathogenesis and any associated syndromes.

With special contributions from Haider Mejbel, MD, Baylor College of Medicine.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 109.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 139.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 219.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Jiang J, Petronic-Rosic V, Hoag J, Shea CR. Eccrine mucinous metaplasia associated with an apocrine cystadenoma. J Cutan Pathol. 2005;32(4):307–9.

    Article  CAS  PubMed  Google Scholar 

  2. Sugiyama A, Sugiura M, Piris A, Tomita Y, Mihm MC. Apocrine cystadenoma and apocrine hidrocystoma: examination of 21 cases with emphasis on nomenclature according to proliferative features. J Cutan Pathol. 2007;34(12):912–7.

    Article  PubMed  Google Scholar 

  3. Hassan MO, Khan MA, Kruse TV. Apocrine cystadenoma. An ultrastructural study. Arch Dermatol. 1979;115(2):194–200.

    Article  CAS  PubMed  Google Scholar 

  4. Glusac EJ, Hendrickson MS, Smoller BR. Apocrine cystadenoma of the vulva. J Am Acad Dermatol. 1994;31(3 Pt 1):498–9.

    Article  CAS  PubMed  Google Scholar 

  5. Poppe H, Kneitz H, Weyandt GH, Brocker EB, Hamm H. Multiple translucent papules on the nose of a 5-year-old boy. Diagnosis: multiple eccrine hidrocystomas. Pediatr Dermatol. 2011;28(1):53–4.

    Article  PubMed  Google Scholar 

  6. Housholder AL, Plotner AN, Sheth AP. Multiple axillary papules in an infant. Diagnosis: multiple congenital apocrine hidrocystomas. Pediatr Dermatol. 2013;30(4):491–2.

    Article  PubMed  Google Scholar 

  7. Shimokawa M, Matsushita S, Kanekura T. Successful treatment of multiple eccrine hidrocystoma with topical atropine sulfate ointment. J Dermatol. 2009;36(2):114–5.

    Article  PubMed  Google Scholar 

  8. Kontochristopoulos G, Markantoni V, Stefanaki C, Kanelleas A, Rigopoulos D, Gregoriou S. Multiple eccrine hidrocystomas treated with botulinum toxin A. Clin Exp Dermatol. 2010;36(1):95–6.

    Article  Google Scholar 

  9. Howard R. Congenital midline lesions: pits and protuberances. Pediatr Ann. 1998;27(3):150–60.

    Article  CAS  PubMed  Google Scholar 

  10. Doi O, Hutson JM, Myers NA, McKelvie PA. Branchial remnants: a review of 58 cases. J Pediatr Surg. 1988;23(9):789–92.

    Article  CAS  PubMed  Google Scholar 

  11. Takimoto T, Itoh M, Furukawa M, Sakano K, Sakashita H, Iwawaki J, Uie T. Branchial cleft (pouch) anomalies: a review of 42 cases. Auris Nasus Larynx. 1991;18(1):87–92.

    Article  CAS  PubMed  Google Scholar 

  12. Rickles NH, Little JW. The histogenesis of the branchial cyst. II. A study of the lining epithelium. Am J Pathol. 1967;50(5):765–77.

    CAS  PubMed  PubMed Central  Google Scholar 

  13. Zvulunov A, Amichai B, Grunwald MH, Avinoach I, Halevy S. Cutaneous bronchogenic cyst: delineation of a poorly recognized lesion. Pediatr Dermatol. 1998;15(4):277–81.

    Article  CAS  PubMed  Google Scholar 

  14. Zvulunov A, Avinoach I. Branchial cleft anomalies and bronchogenic cysts are two unrelated disorders of embryogenesis. Pediatr Dermatol. 2000;17(4):332–3.

    Article  CAS  PubMed  Google Scholar 

  15. Nakamura Y, Fujishima F, Ito S, Ishida K, Sato S, Watanabe M, Tanaka H, Nio M, Sasano H. A case of cutaneous bronchogenic cyst in the scapular area. Pol J Pathol. 2011;62(2):120–1.

    PubMed  Google Scholar 

  16. Saha D, Sinha R, Pai RR, Kumar A, Chakraborti S. Laryngeal cysts in infants and children—a pathologist’s perspective (with review of literature). Int J Pediatr Otorhinolaryngol. 2013;77(7):1112–7.

    Article  PubMed  Google Scholar 

  17. Lacruz G, Gonzalez-Ensenat MA, Sunol M, Vicente A. Unknown: a congenital nodule on the scapula. Dermatol Online J. 2012;18(10):12.

    PubMed  Google Scholar 

  18. Miller 3rd OF, Tyler W. Cutaneous bronchogenic cyst with papilloma and sinus presentation. J Am Acad Dermatol. 1984;11(2 Pt 2):367–71.

    Article  PubMed  Google Scholar 

  19. Nobuhara KK, Gorski YC, La Quaglia MP, Shamberger RC. Bronchogenic cysts and esophageal duplications: common origins and treatment. J Pediatr Surg. 1997;32(10):1408–13.

    Article  CAS  PubMed  Google Scholar 

  20. Rodgers BM, Harman PK, Johnson AM. Bronchopulmonary foregut malformations. The spectrum of anomalies. Ann Surg. 1986;203(5):517–24.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  21. Ozel SK, Kazez A, Koseogullari AA, Akpolat N. Scapular bronchogenic cysts in children: case report and review of the literature. Pediatr Surg Int. 2005;21(10):843–5.

    Article  PubMed  Google Scholar 

  22. Ali AA, Al-Jandan B, Suresh CS, Subaei A. The relationship between the location of thyroglossal duct cysts and the epithelial lining. Head Neck Pathol. 2013;7(1):50–3.

    Article  PubMed  Google Scholar 

  23. Shahin A, Burroughs FH, Kirby JP, Ali SZ. Thyroglossal duct cyst: a cytopathologic study of 26 cases. Diagn Cytopathol. 2005;33(6):365–9.

    Article  PubMed  Google Scholar 

  24. Lee DH, Yoon TM, Lee JK, Lim SC. Is fine needle aspiration cytology appropriate for preoperatively diagnosing thyroglossal duct cysts in children under the age of 10 years? Int J Pediatr Otorhinolaryngol. 2012;76(4):480–2.

    Article  PubMed  Google Scholar 

  25. Siklar Z, Berberoglu M, Yagmurlu A, Hacihamdioglu B, Savas Erdeve S, Fitoz S, Kir M, Ocal G. Synchronous occurrence of papillary carcinoma in the thyroid gland and thyroglossal duct in an adolescent with congenital hypothyroidism. J Clin Res Pediatr Endocrinol. 2012;4(1):30–3.

    Article  PubMed  PubMed Central  Google Scholar 

  26. Vassilatou E, Proikas K, Margari N, Papadimitriou N, Hadjidakis D, Dimitriadis G. An adolescent with a rare midline neck tumor: thyroid carcinoma in a thyroglossal duct cyst. J Pediatr Hematol Oncol. 2014;36(5):407–9.

    Article  PubMed  Google Scholar 

  27. Foley DS, Fallat ME. Thyroglossal duct and other congenital midline cervical anomalies. Semin Pediatr Surg. 2006;15(2):70–5.

    Article  PubMed  Google Scholar 

  28. Allard RH. The thyroglossal cyst. Head Neck Surg. 1982;5(2):134–46.

    Article  CAS  PubMed  Google Scholar 

  29. Bellet JS. Developmental anomalies of the skin. Semin Perinatol. 2013;37(1):20–5.

    Article  PubMed  Google Scholar 

  30. McCollough ML, Glover AT, Grabski WJ, Berger TG. Orbital dermoid cysts showing conjunctival epithelium. Am J Dermatopathol. 1991;13(6):611–5.

    Article  CAS  PubMed  Google Scholar 

  31. Orozco-Covarrubias L, Lara-Carpio R, Saez-De-Ocariz M, Duran-McKinster C, Palacios-Lopez C, Ruiz-Maldonado R. Dermoid cysts: a report of 75 pediatric patients. Pediatr Dermatol. 2013;30(6):706–11.

    Article  PubMed  Google Scholar 

  32. Sinclair RD, Darley C, Dawber RP. Congenital inclusion dermoid cysts of the scalp. Australas J Dermatol. 1992;33(3):135–40.

    Article  CAS  PubMed  Google Scholar 

  33. Hills SE, Maddalozzo J. Congenital lesions of epithelial origin. Otolaryngol Clin North Am. 2014;48(1):209–23.

    Article  Google Scholar 

  34. Al-Khateeb TH, Al-Masri NM, Al-Zoubi F. Cutaneous cysts of the head and neck. J Oral Maxillofac Surg. 2009;67(1):52–7.

    Article  PubMed  Google Scholar 

  35. Andersen WK, Rao BK, Bhawan J. The hybrid epidermoid and apocrine cyst. A combination of apocrine hidrocystoma and epidermal inclusion cyst. Am J Dermatopathol. 1996;18(4):364–6.

    Article  CAS  PubMed  Google Scholar 

  36. Kim MS, Lee JH, Son SJ. Hybrid cysts: a clinicopathological study of seven cases. Australas J Dermatol. 2012;53(1):49–51.

    Article  PubMed  Google Scholar 

  37. Miyake H, Hara H, Shimojima H, Suzuki H. Follicular hybrid cyst (trichilemmal cyst and pilomatricoma) arising within a nevus sebaceus. Am J Dermatopathol. 2004;26(5):390–3.

    Article  PubMed  Google Scholar 

  38. Park HS, Kim WS, Lee JH, Yang JM, Lee ES, Jang KT, Kim DS, Lee DY. Association of human papillomavirus infection with palmoplantar epidermal cysts in Korean patients. Acta Derm Venereol. 2005;85(5):404–8.

    Article  PubMed  Google Scholar 

  39. Egawa K, Egawa N, Honda Y. Human papillomavirus-associated plantar epidermoid cyst related to epidermoid metaplasia of the eccrine duct epithelium: a combined histological, immunohistochemical, DNA-DNA in situ hybridization and three-dimensional reconstruction analysis. Br J Dermatol. 2005;152(5):961–7.

    Article  CAS  PubMed  Google Scholar 

  40. Aloi F, Tomasini C, Pippione M. HPV-related follicular cysts. Am J Dermatopathol. 1992;14(1):37–41.

    Article  CAS  PubMed  Google Scholar 

  41. Honda A, Iwasaki T, Sata T, Kawashima M, Morishima T, Matsukura T. Human papillomavirus type 60-associated plantar wart. Ridged wart. Arch Dermatol. 1994;130(11):1413–7.

    Article  CAS  PubMed  Google Scholar 

  42. Lee S, Lee W, Chung S, Kim D, Sohn M, Kim M, Kim J, Bae H, Kam S. Detection of human papillomavirus 60 in epidermal cysts of nonpalmoplantar location. Am J Dermatopathol. 2003;25(3):243–7.

    Article  PubMed  Google Scholar 

  43. Kashima M, Takahama H, Baba T, Egawa K, Kitasato H, Murakami Y, Mizoguchi M. Detection of human papillomavirus type 57 in the tissue of a plantar epidermoid cyst. Dermatology. 2003;207(2):185–7.

    Article  PubMed  Google Scholar 

  44. Kitasato H, Egawa K, Honda Y, Ono T, Mizushima Y, Kawai S. A putative human papillomavirus type 57 new subtype isolated from plantar epidermoid cysts without intracytoplasmic inclusion bodies. J Gen Virol. 1998;79(Pt 8):1977–81.

    Article  CAS  PubMed  Google Scholar 

  45. Matsukura T, Iwasaki T, Kawashima M. Molecular cloning of a novel human papillomavirus (type 60) from a plantar cyst with characteristic pathological changes. Virology. 1992;190(1):561–4.

    Article  CAS  PubMed  Google Scholar 

  46. Ramagosa R, de Villiers EM, Fitzpatrick JE, Dellavalle RP. Human papillomavirus infection and ultraviolet light exposure as epidermoid inclusion cyst risk factors in a patient with epidermodysplasia verruciformis? J Am Acad Dermatol. 2008;58(5 Suppl 1):S68.e1–6.

    Google Scholar 

  47. Nagore E, Sanchez-Motilla JM, Febrer MI, Aliaga A. Median raphe cysts of the penis: a report of five cases. Pediatr Dermatol. 1998;15(3):191–3.

    Article  CAS  PubMed  Google Scholar 

  48. Cardoso R, Freitas JD, Reis JP, Tellechea O. Median raphe cyst of the penis. Dermatol Online J. 2005;11(3):37.

    PubMed  Google Scholar 

  49. Romani J, Barnadas MA, Miralles J, Curell R, de Moragas JM. Median raphe cyst of the penis with ciliated cells. J Cutan Pathol. 1995;22(4):378–81.

    Article  CAS  PubMed  Google Scholar 

  50. Acenero MJ, Garcia-Gonzalez J. Median raphe cyst with ciliated cells: report of a case. Am J Dermatopathol. 2003;25(2):175–6.

    Article  PubMed  Google Scholar 

  51. Koga K, Yoshida Y, Koga M, Takeshita M, Nakayama J. Median raphe cyst with ciliated cells of the penis. Acta Derm Venereol. 2007;87(6):542–3.

    Article  PubMed  Google Scholar 

  52. Unal B, Bassorgun CI, Eren Karanis MI, Elpek GO. Perianal median raphe cyst: a rare lesion with unusual histology and localization. Case Rep Dermatol Med. 2015;2015:487814.

    PubMed  PubMed Central  Google Scholar 

  53. Nishida H, Kashima K, Daa T, Yada N, Arakane M, Matsuda K, Kato A, Shimizu F, Yokoyama S. Pigmented median raphe cyst of the penis. J Cutan Pathol. 2012;39(8):808–10.

    Article  PubMed  Google Scholar 

  54. Urahashi J, Hara H, Yamaguchi Z, Morishima T. Pigmented median raphe cysts of the penis. Acta Derm Venereol. 2000;80(4):297–8.

    Article  CAS  PubMed  Google Scholar 

  55. Asarch RG, Golitz LE, Sausker WF, Kreye GM. Median raphe cysts of the penis. Arch Dermatol. 1979;115(9):1084–6.

    Article  CAS  PubMed  Google Scholar 

  56. Krauel L, Tarrado X, Garcia-Aparicio L, Lerena J, Sunol M, Rodo J, Ribo JM. Median raphe cysts of the perineum in children. Urology. 2008;71(5):830–1.

    Article  PubMed  Google Scholar 

  57. Chong SJ, Kim SY, Kim HS, Kim GM, Kim SY, Jung JH. Cutaneous ciliated cyst in a 16-year-old girl. J Am Acad Dermatol. 2007;56(1):159–60.

    Article  PubMed  Google Scholar 

  58. Ashton MA. Cutaneous ciliated cyst of the lower limb in a male. Histopathology. 1995;26(5):467–9.

    Article  CAS  PubMed  Google Scholar 

  59. Hung T, Yang A, Binder SW, Barnhill RL. Cutaneous ciliated cyst on the finger: a cutaneous mullerian cyst. Am J Dermatopathol. 2012;34(3):335–8.

    Article  PubMed  Google Scholar 

  60. Lee MT, Heller DS, Lambert WC, Bethel C. Cutaneous ciliated cyst with interspersed apocrine features presenting as a pilonidal cyst in a child. Pediatr Dev Pathol. 2001;4(3):310–2.

    Article  CAS  PubMed  Google Scholar 

  61. Tachibana T, Sakamoto F, Ito M, Ito K, Kaneko Y, Takenouchi T. Cutaneous ciliated cyst: a case report and histochemical, immunohistochemical, and ultrastructural study. J Cutan Pathol. 1995;22(1):33–7.

    Article  CAS  PubMed  Google Scholar 

  62. Bivin Jr WW, Heath JE, Drachenberg CB, Strauch ED, Papadimitriou JC. Cutaneous ciliated cyst: a case report with focus on mullerian heterotopia and comparison with eccrine sweat glands. Am J Dermatopathol. 2010;32(7):731–4.

    Article  PubMed  Google Scholar 

  63. Fontaine DG, Lau H, Murray SK, Fraser RB, Wright Jr JR. Cutaneous ciliated cyst of the abdominal wall: a case report with a review of the literature and discussion of pathogenesis. Am J Dermatopathol. 2002;24(1):63–6.

    Article  PubMed  Google Scholar 

  64. Joehlin-Price AS, Huang JH, Brooks JS, Scharschmidt TJ, Iwenofu OH. PAX-8 expression in cutaneous ciliated cysts: evidence for Mullerian origin. Am J Dermatopathol. 2014;36(2):167–70.

    Article  PubMed  Google Scholar 

  65. Farmer ER, Helwig EB. Cutaneous ciliated cysts. Arch Dermatol. 1978;114(1):70–3.

    Article  CAS  PubMed  Google Scholar 

  66. al-Nafussi AI, Carder P. Cutaneous ciliated cyst: a case report and immunohistochemical comparison with fallopian tube. Histopathology. 1990;16(6):595–8.

    Article  CAS  PubMed  Google Scholar 

  67. Joehlin-Price AS, Huang JH, Brooks JS, Scharschmidt TJ, Iwenofu OH. PAX-8 expression in cutaneous ciliated cysts: evidence for Mullerian origin. Am J Dermatopathol. 2013;36(2):167–70.

    Article  Google Scholar 

  68. Sickel JZ. Cutaneous ciliated cyst of the scalp. A case report with immunohistochemical evidence for estrogen and progesterone receptors. Am J Dermatopathol. 1994;16(1):76–9.

    Article  CAS  PubMed  Google Scholar 

  69. Sunohara M, Ozawa T, Morimoto K, Tateishi C, Ishii M. Two cases of steatocystoma simplex in infants. Dermatol Online J. 2012;18(7):2.

    PubMed  Google Scholar 

  70. Kaur T, Kanwar AJ. Steatocystoma multiplex in four successive generations. J Dermatol. 2003;30(7):559–61.

    Article  PubMed  Google Scholar 

  71. Cho S, Chang SE, Choi JH, Sung KJ, Moon KC, Koh JK. Clinical and histologic features of 64 cases of steatocystoma multiplex. J Dermatol. 2002;29(3):152–6.

    Article  PubMed  Google Scholar 

  72. Plewig G, Wolff HH, Braun-Falco O. Steatocystoma multiplex: anatomic reevaluation, electron microscopy, and autoradiography. Arch Dermatol Res. 1982;272(3–4):363–80.

    Article  CAS  PubMed  Google Scholar 

  73. Hurlimann AF, Panizzon RG, Burg G. Eruptive vellus hair cyst and steatocystoma multiplex: hybrid cysts. Dermatology. 1996;192(1):64–6.

    Article  CAS  PubMed  Google Scholar 

  74. Nogita T, Chi HI, Nakagawa H, Ishibashi Y. Eruptive vellus hair cysts with sebaceous glands. Br J Dermatol. 1991;125(5):475–6.

    Article  CAS  PubMed  Google Scholar 

  75. Patel U, Terushkin V, Fischer M, Kamino H, Patel R. Eruptive vellus hair cysts. Dermatol Online J. 2012;18(12):7.

    PubMed  Google Scholar 

  76. Sanchez-Yus E, Aguilar-Martinez A, Cristobal-Gil MC, Urbina-Gonzalez F, Guerra-Rodriguez P. Eruptive vellus hair cyst and steatocystoma multiplex: two related conditions? J Cutan Pathol. 1988;15(1):40–2.

    Article  CAS  PubMed  Google Scholar 

  77. Oertel YC, Scott DM. Cytologic-pathologic correlations: fine needle aspiration of three cases of steatocystoma multiplex. Ann Diagn Pathol. 1998;2(5):318–20.

    Article  CAS  PubMed  Google Scholar 

  78. Hohl D. Steatocystoma multiplex and oligosymptomatic pachyonychia congenita of the Jackson-Sertoli type. Dermatology. 1997;195(1):86–8.

    Article  CAS  PubMed  Google Scholar 

  79. Lim TW, Paik JH, Kim NI. A case of pachyonychia congenita with oral leukoplakia and steatocystoma multiplex. J Dermatol. 1999;26(10):677–81.

    Article  CAS  PubMed  Google Scholar 

  80. Antal AS, Kulichova D, Redler S, Betz RC, Ruzicka T. Steatocystoma multiplex: keratin 17 – the key player? Br J Dermatol. 2012;167(6):1395–7.

    Article  CAS  PubMed  Google Scholar 

  81. Covello SP, Smith FJ, Sillevis Smitt JH, Paller AS, Munro CS, Jonkman MF, Uitto J, McLean WH. Keratin 17 mutations cause either steatocystoma multiplex or pachyonychia congenita type 2. Br J Dermatol. 1998;139(3):475–80.

    Article  CAS  PubMed  Google Scholar 

  82. Lee PJ, Raniga S, Biyani DK, Watson AJ, Faragher IG, Frizelle FA. Sacrococcygeal pilonidal disease. Colorectal Dis. 2008;10(7):639–50. Discussion 651–2.

    Article  CAS  PubMed  Google Scholar 

  83. da Silva JH. Pilonidal cyst: cause and treatment. Dis Colon Rectum. 2000;43(8):1146–56.

    Article  PubMed  Google Scholar 

  84. von Laffert M, Stadie V, Ulrich J, Marsch WC, Wohlrab J. Morphology of pilonidal sinus disease: some evidence of its being a unilocalized type of hidradenitis suppurativa. Dermatology. 2011;223(4):349–55.

    Article  Google Scholar 

  85. Cordero SC, Royer MC, Rush WL, Hallman JR, Lupton GP. Pure apocrine nevus: a report of 4 cases. Am J Dermatopathol. 2012;34(3):305–9.

    Article  PubMed  Google Scholar 

  86. Herrmann JJ, Eramo LR. Congenital apocrine hamartoma: an unusual clinical variant of organoid nevus with apocrine differentiation. Pediatr Dermatol. 1995;12(3):248–51.

    Article  CAS  PubMed  Google Scholar 

  87. Dua J, Grabczynska S. Eccrine nevus affecting the forearm of an 11-year-old girl successfully controlled with topical glycopyrrolate. Pediatr Dermatol. 2014;31(5):611–2.

    Article  PubMed  Google Scholar 

  88. Kawaoka JC, Gray J, Schappell D, Robinson-Bostom L. Eccrine nevus. J Am Acad Dermatol. 2004;51(2):301–4.

    Article  PubMed  Google Scholar 

  89. Mahdavy M, Smoller BR. Eccrine nevus presenting as a perianal skin tag: a case report and review of the literature. Am J Dermatopathol. 2002;24(4):361–3.

    Article  PubMed  Google Scholar 

  90. Masferrer E, Vicente MA, Bassas-Vila J, Rovira C, Gonzalez-Ensenat MA. Porokeratotic eccrine ostial and dermal duct naevus: report of 10 cases. J Eur Acad Dermatol Venereol. 2010;24(7):847–51.

    Article  CAS  PubMed  Google Scholar 

  91. Moreno A, Pujol RM, Salvatella N, Alomar A, de Moragas JM. Porokeratotic eccrine ostial and dermal duct nevus. J Cutan Pathol. 1988;15(1):43–8.

    Article  CAS  PubMed  Google Scholar 

  92. Warren RB, Verbov JL, Kokai GK. Porokeratotic eccrine ostial and dermal duct nevus. Pediatr Dermatol. 2006;23(5):465–6.

    Article  CAS  PubMed  Google Scholar 

  93. Pelle MT, Pride HB, Tyler WB. Eccrine angiomatous hamartoma. J Am Acad Dermatol. 2002;47(3):429–35.

    Article  PubMed  Google Scholar 

  94. Nghiem BT, Kheterpal M, Dandekar M, Chan M, Lowe L, Wang F. Adult-onset eccrine angiomatous hamartoma: report of a rare entity with unusual histological features. J Eur Acad Dermatol Venereol. 2014;28(7):981–3.

    Article  CAS  PubMed  Google Scholar 

  95. Lopez V, Pinazo I, Santonja N, Jorda E. Eccrine angiomatous hamartoma in a child. Pediatr Dermatol. 2010;27(5):548–9.

    Article  PubMed  Google Scholar 

  96. Galan A, McNiff JM. Eccrine angiomatous hamartoma with features resembling verrucous hemangioma. J Cutan Pathol. 2007;34 Suppl 1:68–70.

    Article  PubMed  Google Scholar 

  97. Patterson AT, Kumar MG, Bayliss SJ, Witman PM, Dehner LP, Gru AA. Eccrine angiomatous hamartoma: a clinicopathologic review of 18 cases. Am J Dermatopathol. 2016;38(6):413–7.

    Article  PubMed  Google Scholar 

  98. Shin J, Jang YH, Kim SC, Kim YC. Eccrine angiomatous hamartoma: a review of ten cases. Ann Dermatol. 2013;25(2):208–12.

    Article  PubMed  PubMed Central  Google Scholar 

  99. Wang L, Wang S, Gao T, Wang G. Eccrine angiomatous hamartoma is a lymphatic proliferation. Eur J Dermatol. 2013;23(5):614–7.

    PubMed  Google Scholar 

  100. Challa VR, Jona J. Eccrine angiomatous hamartoma: a rare skin lesion with diverse histological features. Dermatologica. 1977;155(4):206–9.

    Article  CAS  PubMed  Google Scholar 

  101. Requena L, Sangueza OP. Cutaneous vascular anomalies. Part I. Hamartomas, malformations, and dilation of preexisting vessels. J Am Acad Dermatol. 1997;37(4):523–49. Quiz 549–52.

    Article  CAS  PubMed  Google Scholar 

  102. Larralde M, Bazzolo E, Boggio P, Abad ME, Santos Munoz A. Eccrine angiomatous hamartoma: report of five congenital cases. Pediatr Dermatol. 2009;26(3):316–9.

    Article  PubMed  Google Scholar 

  103. Happle R. The group of epidermal nevus syndromes Part I. Well defined phenotypes. J Am Acad Dermatol. 2010;63(1):1–22. Quiz 23–4.

    Article  PubMed  Google Scholar 

  104. Fedda F, Khattab R, Ibrahim A, Hayek S, Khalifeh I. Verruciform xanthoma: a special epidermal nevus. Cutis. 2011;88(6):269–72.

    PubMed  Google Scholar 

  105. Happle R, Mittag H, Kuster W. The CHILD nevus: a distinct skin disorder. Dermatology. 1995;191(3):210–6.

    Article  CAS  PubMed  Google Scholar 

  106. Micali G, Nasca MR, De Pasquale R. Linear acantholytic dyskeratotic epidermal nevus of the sole. Pediatr Dermatol. 1999;16(2):166–8.

    CAS  PubMed  Google Scholar 

  107. Shaffer HC, Schosser R, Phillips C. Acantholytic dyskeratotic epidermal nevus with eccrine differentiation: a case report and review of literature. J Cutan Pathol. 2009;36(9):1001–4.

    Article  PubMed  Google Scholar 

  108. Ersoy-Evans S, Sahin S, Mancini AJ, Paller AS, Guitart J. The acanthosis nigricans form of epidermal nevus. J Am Acad Dermatol. 2006;55(4):696–8.

    Article  PubMed  Google Scholar 

  109. Arora B, Singh Khinda VI, Bajaj N, Singh Brar G. Congenital epidermal nevus. Int J Clin Pediatr Dent. 2014;7(1):43–6.

    PubMed  PubMed Central  Google Scholar 

  110. Hafner C, Lopez-Knowles E, Luis NM, Toll A, Baselga E, Fernandez-Casado A, Hernandez S, Ribe A, Mentzel T, Stoehr R, Hofstaedter F, Landthaler M, Vogt T, Pujol RM, Hartmann A, Real FX. Oncogenic PIK3CA mutations occur in epidermal nevi and seborrheic keratoses with a characteristic mutation pattern. Proc Natl Acad Sci U S A. 2007;104(33):13450–4.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  111. Hafner C, Toll A, Gantner S, Mauerer A, Lurkin I, Acquadro F, Fernandez-Casado A, Zwarthoff EC, Dietmaier W, Baselga E, Parera E, Vicente A, Casanova A, Cigudosa J, Mentzel T, Pujol RM, Landthaler M, Real FX. Keratinocytic epidermal nevi are associated with mosaic RAS mutations. J Med Genet. 2012;49(4):249–53.

    Article  CAS  PubMed  Google Scholar 

  112. Hafner C, van Oers JM, Vogt T, Landthaler M, Stoehr R, Blaszyk H, Hofstaedter F, Zwarthoff EC, Hartmann A. Mosaicism of activating FGFR3 mutations in human skin causes epidermal nevi. J Clin Invest. 2006;116(8):2201–7.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  113. Miranda LQ, Fracaroli TS, Fonseca JC, Fontenelle E, Curvo RP, Porto LC, Souto R. Analysis of mutations in the PIK3CA and FGFR3 genes in verrucous epidermal nevus. An Bras Dermatol. 2013;88(6 Suppl 1):36–8.

    Article  PubMed  PubMed Central  Google Scholar 

  114. Toll A, Real FX. Somatic oncogenic mutations, benign skin lesions and cancer progression: where to look next? Cell Cycle. 2008;7(17):2674–81.

    Article  CAS  PubMed  Google Scholar 

  115. Bourdeaut F, Herault A, Gentien D, Pierron G, Ballet S, Reynaud S, Paris R, Schleiermacher G, Baumann C, Philippe-Chomette P, Gauthier-Villars M, Peuchmaur M, Radvanyi F, Delattre O. Mosaicism for oncogenic G12D KRAS mutation associated with epidermal nevus, polycystic kidneys and rhabdomyosarcoma. J Med Genet. 2010;47(12):859–62.

    Article  PubMed  Google Scholar 

  116. Loffeld A, McLellan NJ, Cole T, Payne SJ, Fricker D, Moss C. Epidermal naevus in Proteus syndrome showing loss of heterozygosity for an inherited PTEN mutation. Br J Dermatol. 2006;154(6):1194–8.

    Article  CAS  PubMed  Google Scholar 

  117. Vidaurri-de la Cruz H, Tamayo-Sanchez L, Duran-McKinster C, de la Luz Orozco-Covarrubias M, Ruiz-Maldonado R. Epidermal nevus syndromes: clinical findings in 35 patients. Pediatr Dermatol. 2004;21(4):432–9.

    Article  PubMed  Google Scholar 

  118. Lindhurst MJ, Sapp JC, Teer JK, Johnston JJ, Finn EM, Peters K, Turner J, Cannons JL, Bick D, Blakemore L, Blumhorst C, Brockmann K, Calder P, Cherman N, Deardorff MA, Everman DB, Golas G, Greenstein RM, Kato BM, Keppler-Noreuil KM, Kuznetsov SA, Miyamoto RT, Newman K, Ng D, O'Brien K, Rothenberg S, Schwartzentruber DJ, Singhal V, Tirabosco R, Upton J, Wientroub S, Zackai EH, Hoag K, Whitewood-Neal T, Robey PG, Schwartzberg PL, Darling TN, Tosi LL, Mullikin JC, Biesecker LG. A mosaic activating mutation in AKT1 associated with the Proteus syndrome. N Engl J Med. 2011;365(7):611–9.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  119. Lindhurst MJ, Wang JA, Bloomhardt HM, Witkowski AM, Singh LN, Bick DP, Gambello MJ, Powell CM, Lee CC, Darling TN, Biesecker LG. AKT1 gene mutation levels are correlated with the type of dermatologic lesions in patients with Proteus syndrome. J Invest Dermatol. 2013;134(2):543–6.

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  120. Niihori T, Aoki Y, Narumi Y, Neri G, Cave H, Verloes A, Okamoto N, Hennekam RC, Gillessen-Kaesbach G, Wieczorek D, Kavamura MI, Kurosawa K, Ohashi H, Wilson L, Heron D, Bonneau D, Corona G, Kaname T, Naritomi K, Baumann C, Matsumoto N, Kato K, Kure S, Matsubara Y. Germline KRAS and BRAF mutations in cardio-facio-cutaneous syndrome. Nat Genet. 2006;38(3):294–6.

    Article  CAS  PubMed  Google Scholar 

  121. Schubbert S, Zenker M, Rowe SL, Boll S, Klein C, Bollag G, van der Burgt I, Musante L, Kalscheuer V, Wehner LE, Nguyen H, West B, Zhang KY, Sistermans E, Rauch A, Niemeyer CM, Shannon K, Kratz CP. Germline KRAS mutations cause Noonan syndrome. Nat Genet. 2006;38(3):331–6.

    Article  CAS  PubMed  Google Scholar 

  122. Baykal C, Buyukbabani N, Kavak A, Alper M. Nevoid hyperkeratosis of the nipple and areola: a distinct entity. J Am Acad Dermatol. 2002;46(3):414–8.

    Article  PubMed  Google Scholar 

  123. Shastry V, Betkerur J, Kushalappa PA. Unilateral nevoid hyperkeratosis of the nipple: a report of two cases. Indian J Dermatol Venereol Leprol. 2006;72(4):303–5.

    Article  PubMed  Google Scholar 

  124. Rosman IS, Hepper DM, Lind AC, Anadkat MJ. Nevoid hyperkeratosis of the areola misinterpreted as mycosis fungoides. J Cutan Pathol. 2012;39(5):545–8.

    Article  PubMed  Google Scholar 

  125. Templeton SF. Folliculosebaceous cystic hamartoma: a clinical pathologic study. J Am Acad Dermatol. 1996;34(1):77–81.

    Article  CAS  PubMed  Google Scholar 

  126. Tanimura S, Arita K, Iwao F, Kasai M, Fujita Y, Kawasaki H, Abe R, Sawamura D, Kimura T, Shimizu H. Two cases of folliculosebaceous cystic hamartoma. Clin Exp Dermatol. 2006;31(1):68–70.

    Article  CAS  PubMed  Google Scholar 

  127. Badr A, Lakshmiah GR. Folliculosebaceous cystic hamartoma of the nipple: a case report. J Cutan Pathol. 2009;36(5):597–600.

    Article  PubMed  Google Scholar 

  128. Kimura T, Miyazawa H, Aoyagi T, Ackerman AB. Folliculosebaceous cystic hamartoma. A distinctive malformation of the skin. Am J Dermatopathol. 1991;13(3):213–20.

    Article  CAS  PubMed  Google Scholar 

  129. Wu YH. Folliculosebaceous cystic hamartoma or trichofolliculoma? A spectrum of hamartomatous changes inducted by perifollicular stroma in the follicular epithelium. J Cutan Pathol. 2008;35(9):843–8.

    Article  PubMed  Google Scholar 

  130. Merklen-Djafri C, Batard ML, Guillaume JC, Kleinclauss I, Cribier B. Folliculosebaceous cystic hamartoma: anatomo-clinical study. Ann Dermatol Venereol. 2012;139(1):23–30.

    Article  CAS  PubMed  Google Scholar 

  131. Yamamoto O, Suenaga Y, Bhawan J. Giant folliculosebaceous cystic hamartoma. J Cutan Pathol. 1994;21(2):170–2.

    Article  CAS  PubMed  Google Scholar 

  132. Misago N, Kimura T, Toda S, Mori T, Narisawa Y. A revaluation of folliculosebaceous cystic hamartoma: the histopathological and immunohistochemical features. Am J Dermatopathol. 2010;32(2):154–61.

    Article  PubMed  Google Scholar 

  133. Ansai S, Kimura T, Kawana S. A clinicopathologic study of folliculosebaceous cystic hamartoma. Am J Dermatopathol. 2010;32(8):815–20.

    Article  PubMed  Google Scholar 

  134. Cole P, Kaufman Y, Dishop M, Hatef DA, Hollier L. Giant, congenital folliculosebaceous cystic hamartoma: a case against a pathogenetic relationship with trichofolliculoma. Am J Dermatopathol. 2008;30(5):500–3.

    Article  PubMed  Google Scholar 

  135. Noh S, Kwon JE, Lee KG, Roh MR. Folliculosebaceous cystic hamartoma in a patient with neurofibromatosis type I. Ann Dermatol. 2011;23 Suppl 2:S185–7.

    Article  PubMed  PubMed Central  Google Scholar 

  136. Schulz T, Hartschuh W. Folliculo-sebaceous cystic hamartoma is a trichofolliculoma at its very late stage. J Cutan Pathol. 1998;25(7):354–64.

    Article  CAS  PubMed  Google Scholar 

  137. Yang XC, Yan H, Hao F, Yie QY, Zhong BY. Congenital localized basaloid follicular hamartoma: a case report and review of the literature. Int J Dermatol. 2010;49(4):443–7.

    Article  PubMed  Google Scholar 

  138. Grachtchouk V, Grachtchouk M, Lowe L, Johnson T, Wei L, Wang A, de Sauvage F, Dlugosz AA. The magnitude of hedgehog signaling activity defines skin tumor phenotype. EMBO J. 2003;22(11):2741–51.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  139. Wheeler Jr CE, Carroll MA, Groben PA, Briggaman RA, Prose NS, Davis DA. Autosomal dominantly inherited generalized basaloid follicular hamartoma syndrome: report of a new disease in a North Carolina family. J Am Acad Dermatol. 2000;43(2 Pt 1):189–206.

    Article  PubMed  Google Scholar 

  140. Akasaka T, Kon S, Mihm Jr MC. Multiple basaloid cell hamartoma with alopecia and autoimmune disease (systemic lupus erythematosus). J Dermatol. 1996;23(11):821–4.

    Article  CAS  PubMed  Google Scholar 

  141. Smith KJ, Skelton H. Basaloid follicular hamartomas associated with autoimmune disease: a possible role for retinoids in therapy. J Am Acad Dermatol. 2003;49(6):1067–70.

    Article  PubMed  Google Scholar 

  142. Starink TM, Lane EB, Meijer CJ. Generalized trichoepitheliomas with alopecia and myasthenia gravis: clinicopathologic and immunohistochemical study and comparison with classic and desmoplastic trichoepithelioma. J Am Acad Dermatol. 1986;15(5 Pt 2):1104–12.

    Article  CAS  PubMed  Google Scholar 

  143. Gon Ados S, Minelli L, Franzon PG. Case for diagnosis. Inflammatory linear verrucous epidermal nevus. An Bras Dermatol. 2010;85(5):729–31.

    Article  PubMed  Google Scholar 

  144. Lee SH, Rogers M. Inflammatory linear verrucous epidermal naevi: a review of 23 cases. Australas J Dermatol. 2001;42(4):252–6.

    Article  CAS  PubMed  Google Scholar 

  145. Toribio JT, Quicres PA. Inflammatory linear verrucose epidermal nevus. Dermatologica. 1975;150(2):65–9.

    Article  CAS  PubMed  Google Scholar 

  146. Welch ML, Smith KJ, Skelton HG, Frisman DM, Yeager J, Angritt P, Wagner KF. Immunohistochemical features in inflammatory linear verrucous epidermal nevi suggest a distinctive pattern of clonal dysregulation of growth. Military Medical Consortium for the Advancement of Retroviral Research. J Am Acad Dermatol. 1993;29(2 Pt 1):242–8.

    Article  CAS  PubMed  Google Scholar 

  147. Ito M, Shimizu N, Fujiwara H, Maruyama T, Tezuka M. Histopathogenesis of inflammatory linear verrucose epidermal naevus: histochemistry, immunohistochemistry and ultrastructure. Arch Dermatol Res. 1991;283(8):491–9.

    Article  CAS  PubMed  Google Scholar 

  148. Dupre A, Christol B. Inflammatory linear verrucose epidermal nevus. A pathologic study. Arch Dermatol. 1977;113(6):767–9.

    Article  CAS  PubMed  Google Scholar 

  149. Skoven I. Inflammatory linear verrucous epidermal nevus (ILVEN). Acta Derm Venereol. 1979;59(4):364–6.

    CAS  PubMed  Google Scholar 

  150. Abbasi N, Fangman WL, Rosenman KS, Schaffer JV. ILVEN-like persistent psoriasiform dermatitis confined to a congenital Becker nevus. Pediatr Dermatol. 2008;25(3):390–1.

    Article  PubMed  Google Scholar 

  151. Chien Jr P, Rosenman K, Cheung W, Wang N, Sanchez M. Linear psoriasis. Dermatol Online J. 2009;15(8):4.

    PubMed  Google Scholar 

  152. Vissers WH, Muys L, Erp PE, de Jong EM, van de Kerkhof PC. Immunohistochemical differentiation between inflammatory linear verrucous epidermal nevus (ILVEN) and psoriasis. Eur J Dermatol. 2004;14(4):216–20.

    CAS  PubMed  Google Scholar 

  153. Hofer T. Does inflammatory linear verrucous epidermal nevus represent a segmental type 1/type 2 mosaic of psoriasis? Dermatology. 2006;212(2):103–7.

    Article  PubMed  Google Scholar 

  154. Renner R, Colsman A, Sticherling M. ILVEN: is it psoriasis? Debate based on successful treatment with etanercept. Acta Derm Venereol. 2008;88(6):631–2.

    PubMed  Google Scholar 

  155. Patrizi A, Neri I, Fiorentini C, Marzaduri S. Nevus comedonicus syndrome: a new pediatric case. Pediatr Dermatol. 1998;15(4):304–6.

    Article  CAS  PubMed  Google Scholar 

  156. Arias-Santiago S, Aneiros-Fernandez J, Buendia-Eisman A, Giron-Prieto MS, Aneiros-Cachaza J. A 5-year-old boy with comedo-like lesions on the right buttock. Dermatol Online J. 2010;16(10):11.

    CAS  PubMed  Google Scholar 

  157. Ghaninezhad H, Ehsani AH, Mansoori P, Taheri A. Naevus comedonicus of the scalp. J Eur Acad Dermatol Venereol. 2006;20(2):184–5.

    Article  CAS  PubMed  Google Scholar 

  158. Kaliyadan F, Nambiar A, Al Ameer A, Amri M. Nevus comedonicus of the scalp. Skinmed. 2014;12(1):59–60.

    PubMed  Google Scholar 

  159. Vasiloudes PE, Morelli JG, Weston WL. Inflammatory nevus comedonicus in children. J Am Acad Dermatol. 1998;38(5 Pt 2):834–6.

    Article  CAS  PubMed  Google Scholar 

  160. Kim SC, Kang WH. Nevus comedonicus associated with epidermal nevus. J Am Acad Dermatol. 1989;21(5 Pt 2):1085–8.

    Article  CAS  PubMed  Google Scholar 

  161. Giam YC, Ong BH, Rajan VS. Naevus comedonicus in homozygous twins. Dermatologica. 1981;162(4):249–53.

    Article  CAS  PubMed  Google Scholar 

  162. 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.

    Article  Google Scholar 

  163. Brandling-Bennett HA, Morel KD. Epidermal nevi. Pediatr Clin North Am. 2010;57(5):1177–98.

    Article  PubMed  Google Scholar 

  164. Cribier B, Scrivener Y, Grosshans E. Tumors arising in nevus sebaceus: a study of 596 cases. J Am Acad Dermatol. 2000;42(2 Pt 1):263–8.

    Article  CAS  PubMed  Google Scholar 

  165. Levinsohn JL, Tian LC, Boyden LM, McNiff JM, Narayan D, Loring ES, Yun D, Sugarman JL, Overton JD, Mane SM, Lifton RP, Paller AS, Wagner AM, Antaya RJ, Choate KA. Whole-exome sequencing reveals somatic mutations in HRAS and KRAS, which cause nevus sebaceus. J Invest Dermatol. 2012;133(3):827–30.

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  166. Aslam A, Salam A, Griffiths CE, McGrath JA. Naevus sebaceus: a mosaic RASopathy. Clin Exp Dermatol. 2013;39(1):1–6.

    Article  Google Scholar 

  167. Groesser L, Herschberger E, Ruetten A, Ruivenkamp C, Lopriore E, Zutt M, Langmann T, Singer S, Klingseisen L, Schneider-Brachert W, Toll A, Real FX, Landthaler M, Hafner C. Postzygotic HRAS and KRAS mutations cause nevus sebaceous and Schimmelpenning syndrome. Nat Genet. 2012;44(7):783–7.

    Article  CAS  PubMed  Google Scholar 

  168. Sun BK, Saggini A, Sarin KY, Kim J, Benjamin L, LeBoit PE, Khavari PA. Mosaic activating RAS mutations in nevus sebaceus and nevus sebaceus syndrome. J Invest Dermatol. 2012;133(3):824–7.

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  169. Songu M, Adibelli H, Diniz G. White sponge nevus: clinical suspicion and diagnosis. Pediatr Dermatol. 2012;29(4):495–7.

    Article  PubMed  Google Scholar 

  170. Hernandez-Martin A, Fernandez-Lopez E, de Unamuno P, Armijo M. Diffuse whitening of the oral mucosa in a child. Pediatr Dermatol. 1997;14(4):316–20.

    Article  CAS  PubMed  Google Scholar 

  171. Lucchese A, Favia G. White sponge naevus with minimal clinical and histological changes: report of three cases. J Oral Pathol Med. 2006;35(5):317–9.

    Article  PubMed  Google Scholar 

  172. Frithiof L, Banoczy J. White sponge nevus (leukoedema exfoliativum mucosae oris): ultrastructural observations. Oral Surg Oral Med Oral Pathol. 1976;41(5):607–22.

    Article  CAS  PubMed  Google Scholar 

  173. Buchholz F, Schubert C, Lehmann-Willenbrock E. White sponge nevus of the vulva. Int J Gynaecol Obstet. 1985;23(6):505–7.

    Article  CAS  PubMed  Google Scholar 

  174. Jorgenson RJ, Levin S. White sponge nevus. Arch Dermatol. 1981;117(2):73–6.

    Article  CAS  PubMed  Google Scholar 

  175. Terrinoni A, Candi E, Oddi S, Gobello T, Camaione DB, Mazzanti C, Zambruno G, Knight R, Melino G. A glutamine insertion in the 1A alpha helical domain of the keratin 4 gene in a familial case of white sponge nevus. J Invest Dermatol. 2000;114(2):388–91.

    Article  CAS  PubMed  Google Scholar 

  176. Richard G, De Laurenzi V, Didona B, Bale SJ, Compton JG. Keratin 13 point mutation underlies the hereditary mucosal epithelial disorder white sponge nevus. Nat Genet. 1995;11(4):453–5.

    Article  CAS  PubMed  Google Scholar 

  177. Rugg EL, McLean WH, Allison WE, Lunny DP, Macleod RI, Felix DH, Lane EB, Munro CS. A mutation in the mucosal keratin K4 is associated with oral white sponge nevus. Nat Genet. 1995;11(4):450–2.

    Article  CAS  PubMed  Google Scholar 

  178. McGowan KA, Fuchs H, Hrabe de Angelis M, Barsh GS. Identification of a Keratin 4 mutation in a chemically induced mouse mutant that models white sponge nevus. J Invest Dermatol. 2007;127(1):60–4.

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Rights and permissions

Reprints and permissions

Copyright information

© 2017 Springer International Publishing Switzerland

About this chapter

Cite this chapter

Phung, T.L., Wright, T.S., Pourciau, C.Y., Smoller, B.R. (2017). Keratinous Cysts and Hamartomas. In: Pediatric Dermatopathology. Springer, Cham. https://doi.org/10.1007/978-3-319-44824-4_23

Download citation

  • DOI: https://doi.org/10.1007/978-3-319-44824-4_23

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-44822-0

  • Online ISBN: 978-3-319-44824-4

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics