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Dermatology > BENIGN NEOPLASMS
Warty Dyskeratoma
Article Last Updated: Feb 1, 2007
AUTHOR AND EDITOR INFORMATION
Section 1 of 10  
Author: Molly Hinshaw, MD, Assistant Professor, Department of Dermatology, University of Wisconsin School of Medicine and Public Health
Molly Hinshaw is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, Wisconsin Medical Society, and Women's Dermatologic Society
Coauthor(s):
Harry Sharata, MD, Clinical Associate Professor, Department of Dermatology, University of Wisconsin
Editors: Timothy McCalmont, MD, Director, UCSF Dermatopathology Service, Professor of Clinical Pathology and Dermatology, Departments of Pathology and Dermatology, University of California at San Francisco; Richard P Vinson, MD, Assistant Clinical Professor, Department of Dermatology, Texas Tech University School of Medicine; Consulting Staff, Mountain View Dermatology, PA; Rosalie Elenitsas, MD, Associate Professor of Dermatology, University of Pennsylvania School of Medicine; Director, Penn Cutaneous Pathology Services, Department of Dermatology, University of Pennsylvania Health System; Joel M Gelfand, MD, MSCE, Medical Director, Clinical Studies Unit, Assistant Professor, Department of Dermatology, Associate Scholar, Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania; Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Author and Editor Disclosure
Synonyms and related keywords:
WD, focal acantholytic dyskeratosis, isolated keratosis follicularis, Darier disease, Grover disease
Background
Warty dyskeratoma (WD) is a benign epidermal proliferation with distinctive histologic findings that 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, particularly the hard palate, and genital involvement have been reported.
Multiple lesions may occur in the same patient. Although originally referred to as isolated keratosis follicularis, any true relationship between warty dyskeratoma and Darier disease is doubtful. WD can also be thought of as one of the manifestations of focal acantholytic dyskeratosis, an epidermal reaction pattern that can be seen in several disorders, including Darier disease and Grover disease.
Pathophysiology
WD represents a sporadic localized error in epithelial maturation and cohesiveness of unknown etiology. Malunion and premature keratinization of epithelial cells occurs. This process may be secondary to autoimmune, viral, or neoplastic influences.
A veterinary equivalent of WD is theoretically possible.
Frequency
United States
WD is an uncommon lesion. Involvement of the mucosal surfaces is also uncommon.
Mortality/Morbidity
- No malignant potential is known.
- Although WD is microscopically similar to Darier disease, WD is thought to be unrelated to the systemic disorder.
Race
No racial predilection is known.
Sex
By a modest margin, WD affects men more commonly than women.
Age
The average patient age at diagnosis for focal, oral WD is 52.2 years; 10 of 13 reported patients were between the fifth and seventh decades of life.
History
- WD commonly presents as an asymptomatic keratotic nodule.
- Patients report an insidious onset or a slight recent enlargement of the lesion.
Physical
- WD presents as a whitish or grayish hyperkeratotic papule with an umbilicated center, often involving the face, the scalp, or the back. Lesions are usually less than 1-2 cm in size.
- Rarely, lesions with a similar appearance can be found involving the mucosal surfaces.
- Rarely, subungual presentation has been documented. One extraordinary case presented as a longitudinal red ridge within the nail plate.
- Multiple lesions may occur in exceptional cases.
Causes
The cause of WD is unknown.
- 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.
- Chemical carcinogens have been theorized to be causative, although no data exist to support this conclusion.
- 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 recent study showed that 13 of 13 cases did not show human papillomavirus DNA after polymerase chain reaction testing.
Actinic Keratosis
Familial Benign Pemphigus (Hailey-Hailey Disease)
Keratosis Follicularis (Darier Disease)
Squamous Cell Carcinoma
Other Problems to be Considered
Grover disease
Linear epidermal nevus
Lab Studies
- Because WD has repeatedly been shown to be a lesion not associated with systemic disease, no laboratory studies are necessary.
Imaging Studies
- No imaging studies are necessary.
Procedures
- A biopsy interpreted by a dermatopathologist is the criterion standard for diagnosis.
Histologic Findings
The pathologic process is that of a well-circumscribed endophytic or endo-exophytic epithelial proliferation of benign squamous cells demonstrating suprabasilar acantholysis, lacunae, and focal dyskeratotic cells. Dyskeratotic cells are referred to as corps ronds and grains. A central keratin plug may be identified.
Staging
Staging is not necessary for this benign lesion.
Medical Care
No medical care is required.
Surgical Care
Removal of the lesion by using a punch biopsy or an excisional biopsy is an effective therapy for these benign lesions.
Diet
This benign process is unrelated to dietary influences.
Medical therapy is not necessary for this benign lesion; it is cured with surgical removal.
Complications
- The only complications associated with WD are those associated with the minor surgical procedures required for the biopsy or the removal of these benign lesions.
- Potential postsurgical complications, including infection and bleeding, are possible after any surgical procedure.
Prognosis
- No known risk of malignant transformation of WD exists.
- Recurrence is extremely uncommon.
Patient Education
- Patients should be reassured about the benign nature of WD.
Medical/Legal Pitfalls
- The only medicolegal pitfall associated with WD occurs if a lesion is misdiagnosed as acantholytic squamous cell carcinoma. This can occur because both lesions display an endophytic pattern and exhibit acantholysis. However, usually, no more than a slight nuclear variability is present in WD. Thus, a confident and correct benign diagnosis is possible if careful attention is paid to the architectural and cytologic characteristics of the lesion.
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Warty Dyskeratoma excerpt Article Last Updated: Feb 1, 2007
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