Dermatology

Warty dyskeratoma (isolated dyskeratosis follicularis, follicular dyskeratoma)

Warty dyskeratoma (isolated dyskeratosis follicularis, follicular dyskeratoma) ICD-9 528.79

Are You Confident of the Diagnosis?

What you should be alert for in the history

Warty dyskeratoma is a benign epidermal proliferation which is diagnosed most frequently by histologic evaluation.

Characteristic findings on physical examination

It presents typically as a single umbilicated or depressed lesion with a keratotic horn or plug on the head or neck. It often appears associated with a follicular unit. Lesions are small and are typically less than 2cm and are slow growing. Mucosal lesions, particularly on the hard palate, have been reported and there are rare reports of genital involvement. There is a case report of one subungual lesion and rare reports of small multiple lesions.

Expected results of diagnostic studies

On histopathology, warty dyskeratoma is a well circumscribed endo- and exo-phytic cup-shaped lesion. Epithelial proliferation of benign squamous cells surrounds a keratin-filled center or parakeratotic plug (Figure 1). At its base are elongated dermal papillae arranged as villous processes. There is suprabasilar acantholysis with dyskeratotic keratinocytes demonstrating corps ronds and corps grains (Figure 2). The lesion can be seen originating or centered around a hair follicle.

Figure 1.

Warty dyskeratoma. Exo-endophytic epithelial proliferation with a keratin filled center.

Figure 2.

Villous processes with suprabasilar acantholysis with dyskeratotic keratinocytes or corps ronds and grains

Diagnosis confirmation

As the histologic pattern is an epidermal reaction pattern of acantholytic dyskeratosis, the differential diagnosis often includes Grover’s disease, Darier’s disease, and acantholytic actinic keratosis or squamous cell carcinoma. Therefore, clinicopathologic correlation is necessary.

Grover’s disease, or transient acantholytic dermatosis is typically a benign, pruritic condition affecting multiple small erythematous papules on the chest or back of middle-aged men. Less commonly, women may also be affected. Histopathologically, focal acantholysis and dyskeratosis is seen as well as occasional spongiosis.

Darier’s disease, or keratosis follicularis, is an autosomal dominantly inherited condition with multiple greasy, keratotic papules located on the seborrheic areas of the face, upper chest/back and extremities. It is caused by a mutation in the gene ATP2A2. There are often characteristic warty papules on the dorsal hands similar to acrokeratosis verruciformis, and nail changes such as V-shaped nicks and red and white longitudinal bands on the nails. Histopathologic findings include multiple areas of suprabasilar acantholysis with dyskeratosis, corps ronds and columns of parakeratosis (grains).

Darier’s disease and Grover’s disease differ most significantly clinically from warty dyskeratoma in that warty dyskeratoma is most often a solitary lesion and Darier’s diease and Grover’s disease are not.

Finally, acantholytic actinic keratosis and squamous cell carcinoma may present with acantholysis and dyskeratosis, but differs in demonstrating atypical keratinocytic proliferation with cytologic atypia and mitoses.

Who is at Risk for Developing this Disease?

Warty dyskeratoma appear to have no racial predilection and may affect men slightly more commonly than women. The age at presentation is typically between the fifth and seventh decades of life.

What is the Cause of this Disease?

Etiology

Pathophysiology

The etiology of warty dyskeratoma is unclear. It has been classified as a follicular adnexal neoplasm. There is a report of absence of SERCA2 staining by immunohistochemistry, the protein product of ATP2A2, that suggests that warty dyskeratoma may represent an acquired genetic mutation of ATP2A2.

Systemic Implications and Complications

There are no systemic implications. The only complications would be those associated with the potential postsurgical complications from removal. Such complications may include incomplete removal, infection, bleeding and/or scar formation.

Treatment Options

Lesions are removed by punch biopsy or excisional biopsy.

Optimal Therapeutic Approach for this Disease

Surgical excision of the lesion is the optimal treatment.

Patient Management

Patients do not require further management or follow up after removal of the lesion.

Unusual Clinical Scenarios to Consider in Patient Management

Mucosal lesions, particularly on the hard palate, have been reported and there are rare reports of genital involvement. There is a case report of one subungual lesion and rare reports of small multiple lesions.

What is the Evidence?

Baran, R, Perrin, C. "Focal subungual warty dyskeratoma". Dermatology. vol. 195. 1997. pp. 278-80.

(Unique clinical presentation of a subungual warty dyskeratoma with pathologic correlation.)

Chau, MN, Radden, BG. "Oral warty dyskeratoma". J Oral Pathol. vol. 13. 1984. pp. 371-5.

(One of several reports of oral warty dyskeratoma for interest as these were initially thought to be invasive squamous cell carcinoma.)

Cooper, PH. "Acantholytic dermatosis localized to the vulvocrurual area". J Cutan Pathol. vol. 16. 1989. pp. 81-4.

(Interesting discussion of rare acantholytic presentations in the vulvocrural area and differential diagnoses.)

Hinshaw, M, Elston, D. "Warty Dyskeratoma". Emedicine Medscape.

(Nice summary review of warty dyskeratoma. Includes Dr. Fitzpatrick's image of absence of sarco/endoplasmic reticulum Ca2+ATPase2 (SERCA2) staining by immunohistochemistry within a warty dyskeratoma lesion.)

Kaddu, S, Dong, H, Gerlinde, M, Kerl, H, Cerroni, L. "Warty dyskeratoma - “follicular dyskeratoma”: Analysis of cliniopathologic features of a distinctive follicular adnexal neoplasm". J Am Acad Dermatol. vol. 47. 2002. pp. 423-8.

(Review of 46 cases of warty dyskeratoma with clinical presentations and histologic range. The finding of focal contiguity with a pilosebaceous unit in most cases (63%) and absence of HPV-DNA in 13 cases studied, lead authors to propose an alternative term, “follicular dyskeratoma”.)

McKee, PH. "Pathology of the Skin". Elsevier. 2005. pp. 158-169.

(Outstanding review of the different acantholytic dermatoses with dyskeratosis, including Hailey-Hailey disease, Darier's disease, warty dyskeratoma, acantholytic dermatosis of the genitocrural area, and acantholytic acanthoma, with excellent clinicopathologic correlation and images for comparison.)

Mesa, ML, Lambert, WC, Schneider, LC, Reibel, J. "Oral warty dyskeratoma". Cutis. vol. 33. 1984. pp. 293-4, 296.

(Good review of medical and dental literature of oral warty dyskeratoma.)
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