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Warty Dyskeratoma Clinical Presentation

  • Author: Molly Ann Allen Hinshaw, MD; Chief Editor: Dirk M Elston, MD  more...
 
Updated: Aug 17, 2015
 

History

Warty dyskeratoma (WD) commonly presents as an asymptomatic keratotic nodule. Scalp, face, and neck are common sites of involvement. Mucosal lesions have been reported.[2, 3, 4] Multiple lesions in the same patient have been reported.[5, 6] Patients may report an insidious onset or a slight recent enlargement of the lesion.

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Physical

Warty dyskeratoma (WD) presents as a whitish or grayish hyperkeratotic papule with an umbilicated center, often involving the face, scalp, or back. Lesions are usually smaller than 1-2 cm.

Rarely, lesions with a similar appearance can be found involving the mucosal surfaces.[2, 3, 4] Infrequently, subungual warty dyskeratoma occurs and when it does it produces longitudinal erythronychia.[11, 12] Multiple lesions may occur in exceptional cases.[5, 6]

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Causes

James Fitzpatrick, MD, of Dermatopathology Consultants of Colorado, has demonstrated using immunihistochemistry (unpublished work) that warty dyskeratoma demonstrates absence of SERCA2. This finding suggests that acquired genetic mutations in ATP2A2, as demonstrated by the absence of its protein product, SERCA2, play a role in the development of WD. See the image below.

Absence of sarco/endoplasmic reticulum Ca2+ ATPase Absence of sarco/endoplasmic reticulum Ca2+ ATPase 2 (SERCA2) staining by immunohistochemistry within a warty dyskeratoma (right side of image), in contrast to the unaffected epidermis (left side of image). Photomicrograph courtesy of James E. Fitzpatrick, MD.

To date, genetic evaluations of WD for mutations in ATP2A2 (gene encoding SERCA2) have not been reported in the literature.

Two reports have demonstrated immunohistochemical staining of WD with antikeratin antibodies HKN-6 and HKN-7, and this reaction was interpreted as suggesting a follicular origin for WD.

Ultrastructural findings have been interpreted to suggest that acantholysis is due to a defect in desmosome-tonofilament complexes.

Epidermal viral infection with secondary hyperproliferation and acantholysis has also been proposed as a causative mechanism. However, a 2002 study showed that 13 of 13 cases did not show human papillomavirus DNA after polymerase chain reaction testing, and no data support a viral etiology at this time.[10]

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Contributor Information and Disclosures
Author

Molly Ann Allen Hinshaw, MD Clinical Associate Professor, Department of Dermatology, University of Wisconsin School of Medicine and Public Health

Molly Ann Allen Hinshaw, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, Women's Dermatologic Society, Wisconsin Dermatological Society, International Society of Dermatopathology, Dermatology Foundation

Disclosure: Have a 5% or greater equity interest in: Accure Medical, I founded this company with two partners so have 33% interest in the company. We have spent our personal funds to start the company, have no products, and no income yet.

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

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, United States and Canadian Academy of Pathology

Disclosure: Received consulting fee from Apsara for independent contractor.

References
  1. Szymanski FJ. Warty dyskeratoma; a benign cutaneous tumor resembling Darier's disease microscopically. Arch Derm. 1957 Apr. 75(4):567-72. [Medline].

  2. Allon I, Buchner A. Warty dyskeratoma/focal acantholytic dyskeratosis--an update on a rare oral lesion. J Oral Pathol Med. Mar 2012. 41(3):261-7. [Medline].

  3. Azuma Y, Matsukawa A. Warty dyskeratoma with multiple lesions. J Dermatol. 1993 Jun. 20(6):374-7. [Medline].

  4. Duray PH, Merino MJ, Axiotis C. Warty dyskeratoma of the vulva. Int J Gynecol Pathol. 1983. 2(3):286-93. [Medline].

  5. Koç M, Kavala M, Kocatürk E, Mete O, Can B, Zindanci I, et al. Multiple warty dyskeratomas on the scalp. Dermatol Online J. 2009 Feb. 15(2):8. [Medline].

  6. Heymann WR. Warty dyskeratoma appearing in a patient with Darier's disease. Int J Dermatol. 1988 Sep. 27(7):521-2. [Medline].

  7. Allon I, Buchner A. Warty dyskeratoma/focal acantholytic dyskeratosis - an update on a rare oral lesion. J Oral Pathol Med. 2011 Sep 21. [Medline].

  8. Jang EJ, Lee JY, Kim MK, Yoon TY. Warty dyskeratoma involving two adjoining follicles. Ann Dermatol. 2011 Feb. 23(1):98-100. [Medline]. [Full Text].

  9. Savignac M, Edir A, Simon M, Hovnanian A. Darier disease : a disease model of impaired calcium homeostasis in the skin. Biochim Biophys Acta. 2011 May. 1813(5):1111-7. [Medline].

  10. Kaddu S, Dong H, Mayer G, Kerl H, Cerroni L. Warty dyskeratoma--"follicular dyskeratoma": analysis of clinicopathologic features of a distinctive follicular adnexal neoplasm. J Am Acad Dermatol. 2002 Sep. 47(3):423-8. [Medline].

  11. Baran R, Perrin C. Focal subungual warty dyskeratoma. Dermatology. 1997. 195(3):278-80. [Medline].

  12. Cohen PR. Longitudinal erythronychia: individual or multiple linear red bands of the nail plate: a review of clinical features and associated conditions. Am J Clin Dermatol. 2011 Aug 1. 12 (4):217-31. [Medline].

  13. Szymanski FJ. Warty dyskeratoma; a benign cutaneous tumor resembling Darier's disease microscopically. AMA Arch Derm. 1957 Apr. 75(4):567-72. [Medline].

 
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Absence of sarco/endoplasmic reticulum Ca2+ ATPase 2 (SERCA2) staining by immunohistochemistry within a warty dyskeratoma (right side of image), in contrast to the unaffected epidermis (left side of image). Photomicrograph courtesy of James E. Fitzpatrick, MD.
Warty dyskeratoma. An endo-exophytic squamous proliferation of cytologically benign, acantholytic, and dyskeratotic keratinocytes.
Villi lined by acantholytic keratinocytes, some of which are dyskeratotic (corps ronds and corps grains).
 
 
 
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