Reactive Perforating Collagenosis 

  • Author: Susan Cooper, MB, ChB, MD, MRCGP, FRCP; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Aug 2, 2011
 

Background

Reactive perforating collagenosis is a rare skin disorder characterized by the transepidermal elimination of altered collagen through the epidermis. The 2 distinct forms of are an inherited form that manifests in childhood and an acquired sporadic form that occurs in adulthood.

The 4 primary perforating diseases are reactive perforating collagenosis, elastosis perforans serpiginosum, Kyrle disease, and perforating folliculitis, although whether perforating folliculitis is a primary perforating disease has been questioned, given that ruptured follicles are a feature of many infective conditions.

Some authors reserve the term reactive perforating collagenosis for the rare inherited form of the disease, preferring the term acquired perforating dermatosis for the acquired form. This article describes both inherited reactive perforating collagenosis and acquired reactive perforating collagenosis.

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Pathophysiology

The major abnormality in reactive perforating collagenosis is focal damage to collagen and the elimination of the disrupted collagen through the epidermis.[1] A frequent association with pruritus, the tendency to the Koebner phenomenon and the distribution of lesions on trauma-prone areas provides evidence that superficial trauma (eg, scratching) may play a part in the etiology of reactive perforating collagenosis. Cold may precipitate the lesions, especially in the inherited form.

The acquired form usually occurs in patients with diabetes or chronic renal failure, especially those receiving dialysis. Other associations with systemic diseases, such as malignancy, have been reported.

Faver et al has proposed diagnostic criteria for the adult (acquired) form of reactive perforating collagenosis, as follows[2] :

  • Elimination of necrotic basophilic collagen bundles into a cup-shaped epidermal depression as seen in biopsy specimens
  • Umbilicated papules or nodules with a central, adherent keratotic plug
  • Onset of lesions after age 18 years
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Epidemiology

Frequency

International

  • Inherited reactive perforating collagenosis is a rare disorder. Fewer than 50 cases of inherited reactive perforating collagenosis have been reported.
  • The acquired form of reactive perforating collagenosis is more common, occurring in as many as 10% of patients receiving maintenance hemodialysis.[3, 4]

Mortality/Morbidity

  • The lesions of reactive perforating collagenosis are intensely itchy, leaving some scarring after resolution. The lesions of reactive perforating collagenosis often occur on a background of very itchy skin.

Race

  • No racial variations in the incidence of reactive perforating collagenosis are reported.

Sex

  • The sexual incidence of reactive perforating collagenosis is equal.

Age

  • The inherited form of reactive perforating collagenosis usually presents in infancy or early childhood.
  • The acquired form of reactive perforating collagenosis occurs in adults. In a series of 22 patients with acquired reactive perforating collagenosis, the mean patient age at presentation was 56 years.[2]
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Contributor Information and Disclosures
Author

Susan Cooper, MB, ChB, MD, MRCGP, FRCP  Consultant Dermatologist and Honorary Senior Clinical Lecturer, Department of Dermatology, Churchill Hospital, UK

Susan Cooper, MB, ChB, MD, MRCGP, FRCP is a member of the following medical societies: Royal College of Physicians

Disclosure: Nothing to disclose.

Coauthor(s)

Ruth G Asher, MBChB, FRCPath  Locum Consultant Dermatopathologist, John Radcliffe Hospital, Oxford

Ruth G Asher, MBChB, FRCPath is a member of the following medical societies: Association of Clinical Pathologists, British Society of Dermatopathology, International Academy of Pathology, International Society of Dermatopathology, and Royal Society of Medicine

Disclosure: Nothing to disclose.

Specialty Editor Board

James W Patterson, MD  Professor of Pathology and Dermatology, Director of Dermatopathology, University of Virginia Medical Center

James W Patterson, MD is a member of the following medical societies: American Academy of Dermatology, American College of Physicians, American Society of Dermatopathology, Royal Society of Medicine, Society for Investigative Dermatology, and United States and Canadian Academy of Pathology

Disclosure: Nothing to disclose.

Richard P Vinson, MD  Assistant Clinical Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine; Consulting Staff, Mountain View Dermatology, PA

Richard P Vinson, MD is a member of the following medical societies: American Academy of Dermatology, Association of Military Dermatologists, Texas Dermatological Society, 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

Catherine M Quirk, MD  Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania

Catherine M Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology

Disclosure: Nothing to disclose.

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.

Acknowledgments

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous author, Kristiana Gray, MD, to the development and writing of this article.

References

  1. Millard PR, Young E, Harrison DE, Wojnarowska F. Reactive perforating collagenosis: light, ultrastructural and immunohistological studies. Histopathology. Oct 1986;10(10):1047-56. [Medline].

  2. Faver IR, Daoud MS, Su WP. Acquired reactive perforating collagenosis. Report of six cases and review of the literature. J Am Acad Dermatol. Apr 1994;30(4):575-80. [Medline].

  3. Morton CA, Henderson IS, Jones MC, Lowe JG. Acquired perforating dermatosis in a British dialysis population. Br J Dermatol. Nov 1996;135(5):671-7. [Medline].

  4. Poliak SC, Lebwohl MG, Parris A, Prioleau PG. Reactive perforating collagenosis associated with diabetes mellitus. N Engl J Med. Jan 14 1982;306(2):81-4. [Medline].

  5. Hoque SR, Ameen M, Holden CA. Acquired reactive perforating collagenosis: four patients with a giant variant treated with allopurinol. Br J Dermatol. Apr 2006;154(4):759-62. [Medline].

  6. Ramesh V, Sood N, Kubba A, Singh B, Makkar R. Familial reactive perforating collagenosis: a clinical, histopathological study of 10 cases. J Eur Acad Dermatol Venereol. Jul 2007;21(6):766-70. [Medline].

  7. Hafiji J, Hook CE, Burrows NP. Hyperkeratotic papules in a child with Down syndrome. Diagnosis: acquired reactive perforating collagenosis in Down syndrome. Pediatr Dermatol. Jan-Feb 2011;28(1):53-4. [Medline].

  8. Kim EJ, Kim MY, Kim HO, Park YM. Acquired reactive perforating collagenosis triggered by insect bite. J Dermatol. Sep 2007;34(9):677-9. [Medline].

  9. Ikezaki E, Sugita K, Kabashima K, Tokura Y. Scabies-induced acquired reactive perforating collagenosis. J Eur Acad Dermatol Venereol. Jan 2008;22(1):120-1. [Medline].

  10. Bovenmyer DA. Reactive perforating collagenosis. Experimental production of the lesion. Arch Dermatol. Sep 1970;102(3):313-7. [Medline].

  11. Amano H, Nagai Y, Kishi C, Ishikawa O. Acquired reactive perforating collagenosis in dermatomyositis. J Dermatol. May 4 2011;[Medline].

  12. Kiliç A, Gönül M, Cakmak SK, Gül U, Demiriz M. Acquired reactive perforating collagenosis as a presenting sign of hepatocellular carcinoma. Eur J Dermatol. Jul-Aug 2006;16(4):447. [Medline].

  13. Chae KS, Park YM, Cho SH, Cho BK. Reactive perforating collagenosis associated with periampullary carcinoma. Br J Dermatol. Sep 1998;139(3):548-50. [Medline].

  14. Yazdi S, Saadat P, Young S, Hamidi R, Vadmal MS. Acquired reactive perforating collagenosis associated with papillary thyroid carcinoma: a paraneoplastic phenomenon?. Clin Exp Dermatol. May 5 2009;[Medline].

  15. Calista D, Morri M. Acquired reactive perforating collagenosis induced by indinavir in 2 patients with HIV disease. Eur J Dermatol. Jan-Feb 2008;18(1):84-5. [Medline].

  16. Berger RS. Reactive perforating collagenosis of renal failure/diabetes responsive to topical retinoic acid. Cutis. Jun 1989;43(6):540-2. [Medline].

  17. Brinkmeier T, Schaller J, Herbst RA, Frosch PJ. Successful treatment of acquired reactive perforating collagenosis with doxycycline. Acta Derm Venereol. 2002;82(5):393-5. [Medline].

  18. Vion B, Frenk E. [Acquired reactive collagen disease in the adult: successful treatment with UV-B light]. Hautarzt. Jul 1989;40(7):448-50. [Medline].

  19. Serrano G, Aliaga A, Lorente M. Reactive perforating collagenosis responsive to PUVA. Int J Dermatol. Mar 1988;27(2):118-9. [Medline].

 
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Typical keratotic papules.
Cup-shaped invagination of the epidermis associated with a keratin plug containing inflammatory debris and collagen fibers.
Vertically orientated collagen fibers are extruded into the overlying keratin plug.
An elastic van Gieson stain demonstrating the expulsion of collagen fibers (red) into the overlying keratin plug.
 
 
 
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