Official reprint from UpToDate®
www.uptodate.com ©2016 UpToDate®

Perforating dermatoses

Mary Maiberger, MD
Julia R Nunley, MD
Section Editor
Jeffrey Callen, MD, FACP, FAAD
Deputy Editor
Abena O Ofori, MD


The perforating dermatoses represent a group of skin disorders characterized by the "perforation," or elimination, of dermal connective tissue through the epidermis. Although several classifications exist, there are four major "primary" perforating disorders grouped according to the types of epidermal disruption, nature of eliminated material, and clinical features [1]:

Reactive perforating collagenosis (RPC)

Elastosis perforans serpiginosa (EPS) (picture 1A-B)

Perforating folliculitis (PF) (picture 2)

Acquired perforating dermatosis (APD, which encompasses Kyrle disease, acquired RPC, acquired PF, and acquired EPS and is often associated with underlying chronic renal failure or diabetes mellitus (picture 3A-B and table 1)


Subscribers log in here

To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information or to purchase a personal subscription, click below on the option that best describes you:
Literature review current through: Sep 2016. | This topic last updated: Sep 29, 2016.
The content on the UpToDate website is not intended nor recommended as a substitute for medical advice, diagnosis, or treatment. Always seek the advice of your own physician or other qualified health care professional regarding any medical questions or conditions. The use of this website is governed by the UpToDate Terms of Use ©2016 UpToDate, Inc.
  1. Rapini RP, Herbert AA, Drucker CR. Acquired perforating dermatosis. Evidence for combined transepidermal elimination of both collagen and elastic fibers. Arch Dermatol 1989; 125:1074.
  2. Millard PR, Young E, Harrison DE, Wojnarowska F. Reactive perforating collagenosis: light, ultrastructural and immunohistological studies. Histopathology 1986; 10:1047.
  3. Mehregan AH, Schwartz OD, Livingood CS. Reactive perforating collagenosis. Arch Dermatol 1967; 96:277.
  4. Bovenmyer DA. Reactive perforating collagenosis. Experimental production of the lesion. Arch Dermatol 1970; 102:313.
  5. Trattner A, Ingber A, Sandbank M. Mucosal involvement in reactive perforating collagenosis. J Am Acad Dermatol 1991; 25:1079.
  6. Weiner AL. Reactive perforating collagenosis. Arch Dermatol 1970; 102:540.
  7. Patterson JW. The perforating disorders. J Am Acad Dermatol 1984; 10:561.
  8. Cullen SI. Successful treatment of reactive perforating collagenosis with tretinoin. Cutis 1979; 23:187.
  9. Ramesh V, Sood N, Kubba A, et al. Familial reactive perforating collagenosis: a clinical, histopathological study of 10 cases. J Eur Acad Dermatol Venereol 2007; 21:766.
  10. LUTZ W. [Keratosis follicularis serpiginosa]. Dermatologica 1953; 106:318.
  11. Langeveld-Wildschut EG, Toonstra J, van Vloten WA, Beemer FA. Familial elastosis perforans serpiginosa. Arch Dermatol 1993; 129:205.
  12. Mehregan AH. Elastosis perforans serpiginosa: a review of the literature and report of 11 cases. Arch Dermatol 1968; 97:381.
  13. Lewis KG, Bercovitch L, Dill SW, Robinson-Bostom L. Acquired disorders of elastic tissue: part I. Increased elastic tissue and solar elastotic syndromes. J Am Acad Dermatol 2004; 51:1.
  14. Atzori L, Pinna AL, Pau M, Aste N. D-penicillamine elastosis perforans serpiginosa: description of two cases and review of the literature. Dermatol Online J 2011; 17:3.
  15. Bergman R, Friedman-Birnbaum R, Ludatscher R, Lichtig C. An ultrastructural study of the reactive type of elastosis perforans serpiginosa. Arch Dermatol 1987; 123:1127.
  16. Outland JD, Brown TS, Callen JP. Tazarotene is an effective therapy for elastosis perforans serpiginosa. Arch Dermatol 2002; 138:169.
  17. Tuyp EJ, McLeod WA. Elastosis perforans serpiginosa: treatment with liquid nitrogen. Int J Dermatol 1990; 29:655.
  18. Mehta RK, Burrows NP, Payne CM, et al. Elastosis perforans serpiginosa and associated disorders. Clin Exp Dermatol 2001; 26:521.
  19. Mehregan AH, Coskey RJ. Perforating folliculitis. Arch Dermatol 1968; 97:394.
  20. Patterson JW, Graff GE, Eubanks SW. Perforating folliculitis and psoriasis. J Am Acad Dermatol 1982; 7:369.
  21. Farrell AM. Acquired perforating dermatosis in renal and diabetic patients. Lancet 1997; 349:895.
  22. Kyrle J. Hyperkeratosis follicularis et parafollicularis in cutem penetrans. Arch Derm Syph 1916; 123:466.
  23. Carter VH, Constantine VS. Kyrle's disease. I. Clinical findings in five cases and review of literature. Arch Dermatol 1968; 97:624.
  24. Zelger B, Hintner H, Auböck J, Fritsch PO. Acquired perforating dermatosis. Transepidermal elimination of DNA material and possible role of leukocytes in pathogenesis. Arch Dermatol 1991; 127:695.
  25. Thiele-Ochel S, Schneider LA, Reinhold K, et al. Acquired perforating collagenosis: is it due to damage by scratching? Br J Dermatol 2001; 145:173.
  26. Tappeiner J, Wolff K, Schreiner E. [Kyrle's disease]. Hautarzt 1969; 20:296.
  27. Kasiakou SK, Peppas G, Kapaskelis AM, Falagas ME. Regression of skin lesions of Kyrle's disease with clindamycin: implications for an infectious component in the etiology of the disease. J Infect 2005; 50:412.
  28. Kawakami T, Soma Y, Mizoguchi M, Saito R. Immunohistochemical analysis of transforming growth factor-beta3 expression in acquired reactive perforating collagenosis. Br J Dermatol 2001; 144:197.
  29. Gambichler T, Birkner L, Stücker M, et al. Up-regulation of transforming growth factor-beta3 and extracellular matrix proteins in acquired reactive perforating collagenosis. J Am Acad Dermatol 2009; 60:463.
  30. Detmar M, Ruszczak Z, Imcke E, et al. [Kyrle disease in juvenile diabetes mellitus and chronic renal failure]. Z Hautkr 1990; 65:53.
  31. Morgan MB, Truitt CA, Taira J, et al. Fibronectin and the extracellular matrix in the perforating disorders of the skin. Am J Dermatopathol 1998; 20:147.
  32. Chang P, Fernández V. Acquired perforating disease: report of nine cases. Int J Dermatol 1993; 32:874.
  33. Hoque SR, Ameen M, Holden CA. Acquired reactive perforating collagenosis: four patients with a giant variant treated with allopurinol. Br J Dermatol 2006; 154:759.
  34. Alyahya GA, Heegaard S, Prause JU. Ocular changes in a case of Kyrle's disease. 20-year follow-up. Acta Ophthalmol Scand 2000; 78:585.
  35. Saray Y, Seçkin D, Bilezikçi B. Acquired perforating dermatosis: clinicopathological features in twenty-two cases. J Eur Acad Dermatol Venereol 2006; 20:679.
  36. Kim SW, Kim MS, Lee JH, et al. A clinicopathologic study of thirty cases of acquired perforating dermatosis in Korea. Ann Dermatol 2014; 26:162.
  37. Wagner G, Sachse MM. Acquired reactive perforating dermatosis. J Dtsch Dermatol Ges 2013; 11:723.
  38. Ohe S, Danno K, Sasaki H, et al. Treatment of acquired perforating dermatosis with narrowband ultraviolet B. J Am Acad Dermatol 2004; 50:892.
  39. Saleh HA, Lloyd KM, Fatteh S. Kyrle's disease. Effectively treated with isotretinoin. J Fla Med Assoc 1993; 80:395.
  40. Munch M, Balslev E, Jemec GB. Treatment of perforating collagenosis of diabetes and renal failure with allopurinol. Clin Exp Dermatol 2000; 25:615.
  41. Iyoda M, Hayashi F, Kuroki A, et al. Acquired reactive perforating collagenosis in a nondiabetic hemodialysis patient: successful treatment with allopurinol. Am J Kidney Dis 2003; 42:E11.
  42. Lübbe J, Sorg O, Malé PJ, et al. Sirolimus-induced inflammatory papules with acquired reactive perforating collagenosis. Dermatology 2008; 216:239.
  43. Brinkmeier T, Schaller J, Herbst RA, Frosch PJ. Successful treatment of acquired reactive perforating collagenosis with doxycycline. Acta Derm Venereol 2002; 82:393.
  44. Chan LY, Tang WY, Lo KK. Treatment of pruritus of reactive perforating collagenosis using transcutaneous electrical nerve stimulation. Eur J Dermatol 2000; 10:59.