eMedicine Specialties > Dermatology > Diseases of the Dermis

Reactive Perforating Collagenosis: Differential Diagnoses & Workup

Author: Susan Cooper, MB, ChB, MD, MRCGP, MRCP, Consultant Dermatologist and Honorary Senior Clinical Lecturer, Department of Dermatology, Churchill Hospital, Oxford, United Kingdom
Coauthor(s): Ruth G Asher, MBChB, FRCPath, Locum Consultant Dermatopathologist, John Radcliffe Hospital, Oxford
Contributor Information and Disclosures

Updated: Oct 30, 2009

Differential Diagnoses

Elastosis Perforans Serpiginosum
Kyrle Disease
Perforating Folliculitis
Prurigo Nodularis

Workup

Laboratory Studies

  • The purpose of any investigation is to search for underlying causes, particularly causes of pruritus.
  • Urea and creatinine determinations are needed to detect any underlying renal impairment. A creatinine clearance test and other more complex renal investigations may be required.
  • Ferritin levels and a full blood cell count can help detect anemia. Low iron stores can cause pruritus.
  • Random glucose testing can help detect diabetes.
  • Skin biopsy is essential to make a diagnosis.

Histologic Findings

The histology varies with the stage of the reactive perforating collagenosis. Early lesions show epidermal hyperplasia associated with underlying degenerate basophilic collagen fibers. In established lesions, a cup-shaped depression of the epidermis associated with a keratin plug containing parakeratosis, inflammatory debris and collagen fibers can be seen.
 

Cup-shaped invagination of the epidermis associat...

Cup-shaped invagination of the epidermis associated with a keratin plug containing inflammatory debris and collagen fibers.

Cup-shaped invagination of the epidermis associat...

Cup-shaped invagination of the epidermis associated with a keratin plug containing inflammatory debris and collagen fibers.


 
Vertically orientated basophilic collagen fibers are seen in the underlying dermis, with focal extrusion through the epidermis.
 

Vertically orientated collagen fibers are extrude...

Vertically orientated collagen fibers are extruded into the overlying keratin plug.

Vertically orientated collagen fibers are extrude...

Vertically orientated collagen fibers are extruded into the overlying keratin plug.


 
The epidermis is atrophic and may show ulceration. However, at the edges of the cup-shaped invagination, the epidermis is hyperplastic. Additionally, a mild perivascular lymphohistiocytic infiltrate can be seen.

Extruded collagen fibers may be demonstrated with elastic van Gieson (EVG) staining, which stains the fibers red. No extrusion of elastic fibers should be seen (staining black with EVG).

An elastic van Gieson stain demonstrating the exp...

An elastic van Gieson stain demonstrating the expulsion of collagen fibers (red) into the overlying keratin plug.

An elastic van Gieson stain demonstrating the exp...

An elastic van Gieson stain demonstrating the expulsion of collagen fibers (red) into the overlying keratin plug.


More on Reactive Perforating Collagenosis

Overview: Reactive Perforating Collagenosis
Differential Diagnoses & Workup: Reactive Perforating Collagenosis
Treatment & Medication: Reactive Perforating Collagenosis
Follow-up: Reactive Perforating Collagenosis
Multimedia: Reactive Perforating Collagenosis
References

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. 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].

  8. 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].

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

  10. 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].

  11. 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].

  12. 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].

  13. 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].

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

  15. 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].

  16. 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].

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

Further Reading

Keywords

reactive perforating collagenosis, acquired perforating dermatosis, perforating disorders, Kyrle's disease, elastosis perforans serpiginosa, perforating folliculitis

Contributor Information and Disclosures

Author

Susan Cooper, MB, ChB, MD, MRCGP, MRCP, Consultant Dermatologist and Honorary Senior Clinical Lecturer, Department of Dermatology, Churchill Hospital, Oxford, United Kingdom
Susan Cooper, MB, ChB, MD, MRCGP, MRCP 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.

Medical Editor

James W Patterson, MD, Director of Dermatopathology, Professor of Pathology and Dermatology, Departments of Pathology and Dermatology, 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 Medical Association, American Society of Dermatopathology, Medical Society of Virginia, Royal Society of Medicine, Society for Investigative Dermatology, and United States and Canadian Academy of Pathology
Disclosure: Nothing to disclose.

Pharmacy Editor

Richard P Vinson, MD, Assistant Clinical Professor, Department of Dermatology, Texas Tech University 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.

Managing Editor

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: Nothing to disclose.

CME Editor

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, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

 
 
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