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Reactive Perforating Collagenosis Clinical Presentation

  • Author: Susan Cooper, MB, ChB, MD, FRCP; Chief Editor: Dirk M Elston, MD  more...
 
Updated: Aug 18, 2015
 

History

Small, keratotic papules develop after minor trauma. The lesions start as pin-sized lesions, and they grow into larger papules/nodules over a few weeks. They heal with minor scarring.

Intense pruritus is a feature of the acquired form of reactive perforating collagenosis. Many patients have diabetes or renal failure, and many will be on hemodialysis.

A positive family history may be elicited in the childhood form of reactive perforating collagenosis.

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Physical

Skin lesions of reactive perforating collagenosis

Flesh-colored, umbilicated, dome-shaped papules or nodules as large as 10 mm in diameter are typical. They have an adherent, keratinous plug. A giant variant of reactive perforating collagenosis has been described.[5]

Reactive perforating collagenosis lesions may occur in a linear configuration, exhibiting the Koebner phenomenon.

Residual scarring may be seen from previously healed lesions.

Skin distribution of reactive perforating collagenosis

Lesions are most commonly found on the extensor surfaces of the limbs and the dorsa of the hands. Reactive perforating collagenosis lesions may also occur on the trunk and the face. See the image below.

Typical keratotic papules. Typical keratotic papules.
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Causes

See the list below:

  • The childhood form of reactive perforating collagenosis is inherited. [6] A number of affected families have been reported. The mode of inheritance is not clear. Reports of affected families reveal autosomal dominant inheritance, autosomal recessive inheritance, and sporadic cases.
  • A case has been described arising in a child with Down syndrome. [7]
  • The underlying cause of reactive perforating collagenosis is unknown, but an abnormal response to superficial trauma (eg, scratching) may be involved. Papules have been reported following scratches, acne spots, insect bites, [8] and scabies. [9] Lesions of reactive perforating collagenosis have been experimentally induced in susceptible skin by scratching. [10]
  • The acquired form of reactive perforating collagenosis may occur in association with chronic renal failure, often in a patient with underlying diabetes. In both the United Kingdom and the United States, the prevalence of this disorder in patients on dialysis is approximately 10%. [3, 4]
  • The acquired form of reactive perforating collagenosis also can occur in association with other nephropathies without diabetes.
  • Acquired reactive perforating collagenosis has been reported in association with hypothyroidism, hyperparathyroidism, and liver dysfunction, [2] and dermatomyositis. [11]
  • Malignancies, including lymphoma, hepatocellular carcinoma, [12] periampullary carcinoma, [13] and thyroid cancer, [14] have been associated with the acquired form of reactive perforating collagenosis.
  • Indinavir has induced reactive perforating dermatosis in 2 patients with HIV disease. [15]
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Contributor Information and Disclosures
Author

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

Susan Cooper, MB, ChB, MD, 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 is a member of the following medical societies: International Academy of Pathology, Royal Society of Medicine, International Society of Dermatopathology, British Society for Dermatopathology, Association of Clinical Pathologists

Disclosure: Nothing to disclose.

Specialty Editor Board

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, Texas Medical Association, Association of Military Dermatologists, Texas Dermatological Society

Disclosure: Nothing to disclose.

Rosalie Elenitsas, MD Herman Beerman 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, American Medical Association, American Society of Dermatopathology, Pennsylvania Academy of Dermatology

Disclosure: Received royalty from Lippincott Williams Wilkins for textbook editor.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

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

Disclosure: Nothing to disclose.

Additional Contributors

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, United States and Canadian Academy of Pathology

Disclosure: Nothing to disclose.

Acknowledgements

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. 1986 Oct. 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. 1994 Apr. 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. 1996 Nov. 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. 1982 Jan 14. 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. 2006 Apr. 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. 2007 Jul. 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. 2011 Jan-Feb. 28(1):53-4. [Medline].

  8. Kim EJ, Kim MY, Kim HO, Park YM. Acquired reactive perforating collagenosis triggered by insect bite. J Dermatol. 2007 Sep. 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. 2008 Jan. 22(1):120-1. [Medline].

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

  11. Kikuchi N, Ohtsuka M, Yamamoto T. Acquired Reactive Perforating Collagenosis: A Rare Association with Dermatomyositis. Acta Derm Venereol. 2013 Mar 5. [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. 2006 Jul-Aug. 16(4):447. [Medline].

  13. Chae KS, Park YM, Cho SH, Cho BK. Reactive perforating collagenosis associated with periampullary carcinoma. Br J Dermatol. 1998 Sep. 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. 2009 May 5. [Medline].

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

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

  17. Tilz H, Becker JC, Legat F, Schettini AP, Inzinger M, Massone C. Allopurinol in the treatment of acquired reactive perforating collagenosis*. An Bras Dermatol. 2013 Jan-Feb. 88(1):94-7. [Medline].

  18. 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].

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

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

  21. Sehgal VN, Verma P, Bhattacharya SN, Sharma S. Familial Reactive Perforating Collagenosis in a Child: Response to Narrow-Band UVB. Pediatr Dermatol. 2012 Apr 4. [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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