Perforating Folliculitis 

  • Author: Suguru Imaeda, MD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Jan 17, 2012
 

Background

Perforating disorders are characterized by transepidermal elimination of altered keratin or dermal connective tissue material. These disorders include perforating folliculitis (as shown below), Kyrle disease, elastosis perforans serpiginosa, reactive perforating collagenosis, and acquired perforating dermatosis. Cases of overlap are described, and diagnostic criteria are not well-defined for all the entities.[1] Clinically, the lesions are hyperkeratotic to verrucous papules and nodules.

Typical appearance of lesions of perforating folliTypical appearance of lesions of perforating folliculitis consisting of keratotic follicular papules.

In perforating folliculitis, keratotic follicular papules develop, particularly over extensor surfaces. Microscopically, the disorder is characterized by disruption of the infundibular portion of the follicular wall, with transepidermal (transfollicular) elimination of connective-tissue elements and cellular debris.

Perforating folliculitis may present as an isolated finding, apparently unrelated to other disease states, but also can be associated with chronic renal failure and diabetes mellitus. Perforating folliculitis is closely related, if not identical, to the acquired perforating dermatosis that occurs with chronic renal disease. Kyrle disease (hyperkeratosis follicularis et parafollicularis in cutem penetrans) may simply represent an exaggerated form of perforating folliculitis. In addition, another disorder of transepidermal elimination, elastosis perforans serpiginosa, occasionally displays involvement of follicular units.

Next

Pathophysiology

As in Kyrle disease, the concept of an extrinsic keratin plug penetrating the epidermis generally has been discredited. Abnormally premature keratinization at the expense of proliferation is a possible explanation, as proposed by Carter and Constantine and Tappeiner et al in Kyrle disease.[2, 3] A role for fibronectin has been postulated. In addition, a primary alteration of connective tissue or deposition of foreign material within the superficial dermis, with subsequent engulfment and elimination by proliferative follicular epithelium, also is conceivable as a mechanism. Such a response to experimental implantation of foreign material has been described.

In addition, evidence suggests a pathologic role for excessively coiled hairs. Mehregan first proposed that curled hairs within follicular canals may act as springs, penetrating the lateral follicular wall, thereby initiating the process of transepidermal elimination.[4] Support for this concept has been provided by an ultrastructural study of acquired perforating dermatosis that showed hair shaft fragments within transepidermal channels, even in patients in whom follicular involvement was not demonstrable on routine light microscopy. Factors that may promote coiling of hairs include follicular hyperkeratosis (occasional perforated follicles can be identified in keratosis pilaris) or contact dermatitis (eg, resulting from formaldehyde in clothing). Finally, trauma, such as scratching of pruritic skin, may well play a significant role in lesional development, possibly by setting in motion one or more of the pathologic events described above.[5]

Previous
Next

Epidemiology

Frequency

United States

Incidence of perforating folliculitis in the United States is not known precisely, although the disorder is not uncommon. In Detroit, Michigan, 50 cases were reported during a 2-year period in the early 1970s, although this observation was followed by a declining incidence of new cases.

International

International incidence of perforating folliculitis is not known.

Mortality/Morbidity

Perforating folliculitis Morbidity is associated with the cosmetic appearance of lesions and the pruritus that occasionally accompanies them. Although cutaneous disease is insignificant, substantial morbidity or mortality rates can be seen in association with the primary underlying diseases, such as diabetes mellitus or chronic renal failure.

Race

Although generally no ethnic predilection has been identified, 1 study found a higher incidence of Kyrle disease in chronic renal failure among African American individuals.

Sex

Perforating folliculitis occurs equally in males and females; no sex predilection has been reported.

Age

Perforating folliculitis is more common in the second through fourth decades of life.

Previous
Proceed to Clinical Presentation
 
 
Contributor Information and Disclosures
Author

Suguru Imaeda, MD  Chief of Dermatology, Yale University Health Services; Chief of Dermatology, West Haven Veterans Affairs Medical Center; Assistant Professor, Department of Dermatology, Yale University School of Medicine

Suguru Imaeda, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, Connecticut State Medical Society, Sigma Xi, and Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Specialty Editor Board

Timothy McCalmont, MD  Director, UCSF Dermatopathology Service, Professor of Clinical Pathology and Dermatology, Departments of Pathology and Dermatology, University of California at San Francisco; Editor-in-Chief, Journal of Cutaneous Pathology

Timothy McCalmont, MD is a member of the following medical societies: Alpha Omega Alpha, American Medical Association, American Society of Dermatopathology, California Medical Association, College of American Pathologists, and United States and Canadian Academy of Pathology

Disclosure: Apsara Consulting fee Independent contractor

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

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.

Rosalie Elenitsas, MD  Herman Beerman Associate Professor of Dermatology, University of Pennsylvania School of Medicine; Director, Penn Cutaneous Pathology Services, Department of Dermatology, University of Pennsylvania Health System

Rosalie Elenitsas, MD is a member of the following medical societies: American Academy of Dermatology and American Society of Dermatopathology

Disclosure: Lippincott Williams Wilkins Royalty Textbook editor; DLA Piper Consulting fee Consulting

Joel M Gelfand, MD, MSCE  Medical Director, Clinical Studies Unit, Assistant Professor, Department of Dermatology, Associate Scholar, Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania

Joel M Gelfand, MD, MSCE is a member of the following medical societies: Society for Investigative Dermatology

Disclosure: AMGEN Consulting fee Consulting; AMGEN Grant/research funds Investigator; Genentech Grant/research funds investigator; Centocor Consulting fee Consulting; Abbott Grant/research funds investigator; Abbott Consulting fee Consulting; Novartis investigator; Pfizer Grant/research funds investigator; Celgene Consulting fee DMC Chair; NIAMS and NHLBI Grant/research funds investigator

Chief Editor

Dirk M Elston, MD  Director, Ackerman Academy of Dermatopathology, New York

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

Disclosure: Nothing to disclose.

Additional Contributors

The authors and editors of eMedicine gratefully acknowledge the significant contributions of previous author, James W. Patterson, MD, to the development and writing of this article which has been recently updated.

References

  1. Abe R, Murase S, Nomura Y, et al. Acquired perforating dermatosis appearing as elastosis perforans serpiginosa and perforating folliculitis. Clin Exp Dermatol. Aug 2008;33(5):653-4. [Medline].

  2. Carter VH, Constantine VS. Kyrle's disease. I. Clinical findings in five cases and review of literature. Arch Dermatol. Jun 1968;97(6):624-32. [Medline].

  3. Tappeiner J, Wolff K, Schreiner E. [Kyrle's disease]. Hautarzt. Jan 1969;20(1):296-310. [Medline].

  4. Mehregan AH, Coskey RJ. Perforating folliculitis. Arch Dermatol. Apr 1968;97(4):394-9. [Medline].

  5. Pavlovic MD, Zecevic RD, Stamenkovic M, Stojadinovic O, Zolotarevski L. Trauma-induced perforating folliculitis. Eur J Dermatol. Nov-Dec 2003;13(6):592. [Medline].

  6. Burkhart CG. Perforating folliculitis. A reappraisal of its pathogenesis. Int J Dermatol. Nov 1981;20(9):597-9. [Medline].

  7. Hurwitz RM. The evolution of perforating folliculitis in patients with chronic renal failure. Am J Dermatopathol. Jun 1985;7(3):231-9. [Medline].

  8. White CR Jr, Heskel NS, Pokorny DJ. Perforating folliculitis of hemodialysis. Am J Dermatopathol. Apr 1982;4(2):109-16. [Medline].

  9. Bilezikci B, Seckin D, Demirhan B. Acquired perforating dermatosis in patients with chronic renal failure: a possible pathogenetic role for fibronectin. J Eur Acad Dermatol Venereol. Mar 2003;17(2):230-2. [Medline].

  10. Headley CM, Wall B. ESRD-associated cutaneous manifestations in a hemodialysis population. Nephrol Nurs J. Dec 2002;29(6):525-7, 531-9; quiz 540-1. [Medline].

  11. Hong SB, Park JH, Ihm CG, Kim NI. Acquired perforating dermatosis in patients with chronic renal failure and diabetes mellitus. J Korean Med Sci. Apr 2004;19(2):283-8. [Medline].

  12. Hurwitz RM, Melton ME, Creech FT 3rd, Weiss J, Handt A. Perforating folliculitis in association with hemodialysis. Am J Dermatopathol. Apr 1982;4(2):101-8. [Medline].

  13. Kahana M, Trau H, Dolev E, Schewach-Millet M, Gilon E. Perforating folliculitis in association with primary sclerosing cholangitis. Am J Dermatopathol. Jun 1985;7(3):271-6. [Medline].

  14. Mahajan S, Koranne RV, Sardana K, Mendiratta V, Damani A. Perforating folliculitis with jaundice in an Indian male: a rare case with sclerosing cholangitis. Br J Dermatol. Mar 2004;150(3):614-6. [Medline].

  15. Patterson JW, Graff GE, Eubanks SW. Perforating folliculitis and psoriasis. J Am Acad Dermatol. Sep 1982;7(3):369-76. [Medline].

  16. Neill SM, Pembroke AC, du Vivier AW, Salisbury JR. Phrynoderma and perforating folliculitis due to vitamin A deficiency in a diabetic. J R Soc Med. Mar 1988;81(3):171-2. [Medline].

  17. Fistarol SK, Itin PH. Acquired perforating dermatosis in a patient with Poland syndrome. Dermatology. 2003;207(4):390-4. [Medline].

  18. Rubio FA, Herranz P, Robayna G, Pena JM, Contreras F, Casado M. Perforating folliculitis: report of a case in an HIV-infected man. J Am Acad Dermatol. Feb 1999;40(2 Pt 2):300-2. [Medline].

  19. Gilaberte Y, Coscojuela C, Vazquez C, Rosello R, Vera J. Perforating folliculitis associated with tumour necrosis factor-alpha inhibitors administered for rheumatoid arthritis. Br J Dermatol. Feb 2007;156(2):368-71. [Medline].

  20. Wolber C, Udvardi A, Tatzreiter G, Schneeberger A, Volc-Platzer B. Perforating folliculitis, angioedema, hand-foot syndrome--multiple cutaneous side effects in a patient treated with sorafenib. J Dtsch Dermatol Ges. May 2009;7(5):449-52. [Medline].

  21. Ohe S, Danno K, Sasaki H, Isei T, Okamoto H, Horio T. Treatment of acquired perforating dermatosis with narrowband ultraviolet B. J Am Acad Dermatol. Jun 2004;50(6):892-4. [Medline].

  22. Zachariae H, Sogaard H. Progressive generalized perforating folliculitis. Dermatologica. 1984;168(3):131-7. [Medline].

  23. Ashton RE, Montheith PG. Successful treatment of perforating folliculitis with 13-cis-retinoic acid. J Dermatol Treat. 1992;3:67-8.

  24. Chang P, Fernández V. Acquired perforating disease: report of nine cases. Int J Dermatol. Dec 1993;32(12):874-6. [Medline].

  25. Combemale P, Courtois D, Chouvet B. [Perforating folliculitis]. Ann Dermatol Venereol. 1990;117(8):515-20. [Medline].

  26. Patterson JW. The perforating disorders. J Am Acad Dermatol. Apr 1984;10(4):561-81. [Medline].

  27. Patterson JW, Brown PC. Ultrastructural changes in acquired perforating dermatosis. Int J Dermatol. Mar 1992;31(3):201-5. [Medline].

  28. Sehgal VN, Jain S, Thappa DM, Bhattacharya SN, Logani K. Perforating dermatoses: a review and report of four cases. J Dermatol. Jun 1993;20(6):329-40. [Medline].

  29. Tsunoda T, Horiuchi N, Deguchi M, Manoma H. Three cases of perforating folliculitis. Japanese Journal of Clinical Dermatology. Nov 1999;53:908-10.

 
Previous
Next
 
Typical appearance of lesions of perforating folliculitis consisting of keratotic follicular papules.
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2012 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.