Introduction
Background
Warty dyskeratoma (WD) is a benign epidermal proliferation that was first described in 1957 because of its distinctive histologic findings.1 Warty dyskeratoma commonly manifests as an umbilicated lesion with a keratotic plug, usually limited to the head, neck, or face. Lesions are generally solitary and sporadic and may be associated with a follicular unit. Oral involvement,2,3 particularly the hard palate, and genital involvement have been reported.4 Multiple lesions may occur in the same patient.5,6
Although originally referred to as isolated keratosis follicularis, no data support that patients with a warty dyskeratoma bear germline mutations in ATP2A2, the gene responsible for Darier disease. Patients with Darier disease have been reported with warty dyskeratomas occurring spontaneously or during treatment with systemic retinoids.
Pathophysiology
Warty dyskeratoma represents a sporadic localized error in epithelial maturation and cohesiveness. James Fitzpatrick, MD, of Dermatopathology Consultants of Colorado, has shown that these tumors lose SERCA2 staining by immunohistochemistry, thus providing evidence that an acquired genetic mutation in ATP2A2 plays a role in tumor development. Malunion and premature keratinization of epithelial cells occur. Attempts to demonstrate that human papillomavirus plays a role have proven unsuccessful.7 Other adhesion molecules may also play a role.
Frequency
United States
Warty dyskeratoma is an uncommon lesion. Involvement of the mucosal surfaces is also uncommon.
Mortality/Morbidity
Although warty dyskeratoma is histologically similar to Darier disease and appears to share the same absent protein (SERCA2) in at least some cases, no evidence indicates that patients with warty dyskeratoma are at risk for other disorders, including Darier disease.
Race
No racial predilection is known.
Sex
By a modest margin, warty dyskeratoma affects men more commonly than women.
Age
The average patient age at diagnosis for focal, oral warty dyskeratoma is 52.2 years; 10 of 13 reported patients were between the fifth and seventh decades of life.
Clinical
History
- Warty dyskeratoma 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.
Physical
- Warty dyskeratoma 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
- Rarely, subungual presentation has been documented.8 One extraordinary case presented as a longitudinal red ridge within the nail plate.
- Multiple lesions may occur in exceptional cases.5,6
Causes
- James Fitzpatrick, MD, of Dermatopathology Consultants of Colorado, has demonstrated in unpublished work that warty dyskeratoma demonstrates absence of SERCA2 staining by immunohistochemistry. This at least suggests that acquired genetic mutations in ATP2A2, as demonstrated by the absence of its protein product, SERCA2, play a role in the development of warty dyskeratoma.
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 warty dyskeratoma for mutations in ATP2A2 (gene encoding SERCA2) have not been reported in the literature.
- Two reports have demonstrated immunohistochemical staining of warty dyskeratoma with antikeratin antibodies HKN-6 and HKN-7, and this reaction was interpreted as suggesting a follicular origin for warty dyskeratoma.
- 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.7
More on Warty Dyskeratoma |
 Overview: Warty Dyskeratoma |
| Differential Diagnoses & Workup: Warty Dyskeratoma |
| Treatment & Medication: Warty Dyskeratoma |
| Follow-up: Warty Dyskeratoma |
| Multimedia: Warty Dyskeratoma |
| References |
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References
Tuominen H, Sormunen R, Kallioinen M. Non-erythroid spectrin (fodrin) in cutaneous tumours: diminished in cell membranes, increased in the cytoplasm. Br J Dermatol. Oct 1996;135(4):576-80. [Medline].
Kaugars GE, Lieb RJ, Abbey LM. Focal oral warty dyskeratoma. Int J Dermatol. Mar 1984;23(2):123-30. [Medline].
Azuma Y, Matsukawa A. Warty dyskeratoma with multiple lesions. J Dermatol. Jun 1993;20(6):374-7. [Medline].
Duray PH, Merino MJ, Axiotis C. Warty dyskeratoma of the vulva. Int J Gynecol Pathol. 1983;2(3):286-93. [Medline].
Griffiths TW, Hashimoto K, Sharata HH, Ellis CN. Multiple warty dyskeratomas of the scalp. Clin Exp Dermatol. Jul 1997;22(4):189-91. [Medline].
Heymann WR. Warty dyskeratoma appearing in a patient with Darier's disease. Int J Dermatol. Sep 1988;27(7):521-2. [Medline].
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. Sep 2002;47(3):423-8. [Medline].
Baran R, Perrin C. Focal subungual warty dyskeratoma. Dermatology. 1997;195(3):278-80. [Medline].
Szymanski FJ. Warty dyskeratoma; a benign cutaneous tumor resembling Darier's disease microscopically. AMA Arch Derm. Apr 1957;75(4):567-72. [Medline].
Brownstein MH. The benign acanthomas. J Cutan Pathol. Jun-Aug 1985;12(3-4):172-88. [Medline].
Harrist TJ, Murphy EF, Mihm MC. Oral warty dyskeartoma. Arch Dermatol. 1980;116:929.
Further Reading
Keywords
warty dyskeratoma, WD, focal acantholytic dyskeratosis, isolated keratosis follicularis, Darier disease, Grover disease
