Warty Dyskeratoma - Clinical Features, Diagnosis, and Treatment

What Is Warty Dyskeratoma?

Warty Dyskeratoma is a rare neoplasm of the skin. Four adnexal (appendages related to a particular organ) structures are present in the skin. They are hair follicles, sebaceous, apocrine, and eccrine glands. Warty dyskeratoma is considered a follicular adnexal neoplasm (tumor of a hair follicle) as it develops in association with pilosebaceous glands of the skin. The hair follicle, hair shaft, and sebaceous gland together constitute a pilosebaceous unit. Szymanski named it warty dyskeratoma in 1957, which Graham and Helwig first described in 1954 and was then called isolated driver's disease.

What Are the Characteristic Features of Warty Dyskeratoma?

It develops as a solitary lesion that has a characteristic nodular or papular growth

which is benign. The histological features look similar to invasive squamous cell carcinoma. Warty Dyskeratoma has an umbilicated appearance, with a depression in the center and consists of a keratotic plug. It is often associated with a pilosebaceous unit of the skin. This lesion tends to grow inward and is characterized by a rapid increase of the squamous epithelium, loss of structural integrity because of the loss of intercellular bridges, and abnormal keratinization.

Multiple lesions are found in a few cases. It is generally sporadically found on the head, neck, face, scalp, and vulva. Fews cases are reported to develop in the oral cavity, especially on the hard palate, eyelid, and genital involvement is also observed in a few cases. It is also called isolated dyskeratosis follicularis. It is mostly asymptomatic, and the color of the lesion may vary from red to brown, with a central depression mimics the umbilical region. Rarely are the lesions pruritic. It is an uncommon benign lesion occurring in middle-aged to older individuals. It is observed that it develops more in males than females.

What Is The Cause For Warty Dyskeratoma?

The cause of warty dyskeratosis is unclear to date. Many studies have been conducted to study etiology. Many histopathological samples are collected from individuals to enable the study of specimens under a microscope. James Fitzpatrick has conducted various histopathological studies, after which it is observed that warty dyskeratoma displays an absence of the SERCA2 gene. Acquired genetic mutations in ATP2A2 are the reason for the lack of the protein gene SERCA2. These studies have not been published yet. There is not one literature review report about this etiological factor or the study.

Acquired mutations of ATP2A2 can be attributed to

• Viral infection.

• Smoking

• Exposure to ultraviolet light.

• Autoimmune disorders.

Several theories are found to point to histological similarities to viral warts, which are caused by the human papillomavirus. Still, the lesions of warty dyskeratoma did not show any presence of the human papillomavirus (HPV).

What Does Warty Dyskeratoma Look Like?

Warty Dyskeratoma presents as an asymptomatic keratotic nodule commonly found on the face, neck, and scalp. It looks like a papule or nodule with colors ranging from whitish or skin or red or brown. They are verrucous with a central dip on the lesion and a distinguishing keratotic plug. They are solitary lesions and can grow to 2 cm in size. They are also known to occur in the oral cavity rarely. The lesions appear similarly on the oral mucosa, frequently found on the hard palate. Multiple lesions appear very rarely. These lesions are reported to occur infrequently on genital areas as well. Few lesions are associated with recurrent cheesy drainage from the lesion that gives out a foul smell.

How Do They Appear Under Microscope?

Under the microscope, it appears as a cup-shaped invagination (surface folding in to form a cavity) filled with a keratotic plug is observed. A solitary lesion generally involves a single hair follicle. However, multiple hair follicles might be involved in a solitary lesion. The lesions seem to be originating from the pilosebaceous unit, i.e., either it contains a hair follicle or a sebaceous gland. Besides cup-shaped formation, few histologic patterns displayed cystic and nodular patterns. Cystic lesions are large, well-defined, and lined with keratin. Nodular lesions are small, well-defined solid aggregations of epithelial cells.

What Is The Treatment For Warty Dyskeratoma?

Surgical excision is the only treatment needed for warty dyskeratoma. Therefore, extensive medical care is not necessary. Medicines are prescribed only to prevent secondary infection. Cases are presented with successful treatment of warty dyskeratoma with Tazarotene acid gel. Further studies need to be conducted to see if it treats other similar dyskeratotic disorders.

If used for treating warty dyskeratoma, the following procedures have shown recurrence. It includes-

• Curettement is a surgery done by scraping with a curette to remove bodily tissues.

• Electro-desiccation- This procedure dries the tissues using a high-frequency electric current applied with a needle-shaped electrode.

• Irradiation with X-ray.

However, surgical excision has shown zero recurrences.

What Are the Differential Diagnosis for Warty Dyskeratoma?

1. Squamous Carcinoma: is a common form of skin cancer.

2. Vulvar Dysplasia: They are skin changes in the vulvar region but not cancer.

3. Keratoacanthoma is a rapidly growing skin tumor with a centralized keratinous plug.

4. Keratosis Follicularis is also known as Darier's disease, a genetic skin disorder.

5. Actinic Keratosis is a rough, scaly patch that occurs due to years of skin exposure.

6. Transient Acantholytic Dermatosis is a widespread itchy skin eruption known as Grover's disease.

Warty dyskeratosis can be considered as one of the manifestations of several disorders like Darier's disease and Grover's disease. However, the most significant difference between Darier's disease and Warty dyskeratosis is that Darier's disease constitutes inherited skin lesions with multiple papular lesions, while warty dyskeratoma is solitary and uninherited.

Conclusion:

It is a rare localized epidermal disorder, a solitary and benign lesion. It is asymptomatic, which does not always need medical intervention. Its growth in size is limited and rarely occurs as multiple lesions in the same individual. It is not a life-threatening disease. Simple surgical excision is enough to treat the lesion. The prognosis is good for warty dyskeratoma.