case of keratosis follicularis or pachyonychia congenita. Systemic candidiasis or lichen planus with oral and skin lesions may also be considered.
Microscopic features
White patches will show acanthosis and hyperkeratosis. Discolored lesions will show melanin pigment within macrophages and free melanin in the dermis, hence the name incontinentia pigmenti.
Suggested course of action
Suspected cases should be referred to a dermatologist and/or a geneticist.
Treatment
No treatment is required.
Proliferative Verrucous Leukoplakia (PVL)
Nature of disease
A relentless progression from benign-appearing leukoplakia of only hyperkeratosis through to verrucous hyperplasia, verrucous carcinoma, invasive squamous cell carcinoma, and a final stage of an extremely aggressive and treatment-resistant squamous cell carcinoma.
Predilections
Adults over 50 years. Affects women more than men. Unrelated to tobacco products and high-risk human papillomaviruses. No racial predilection is known.
Clinical features
Depending on the stage and advancement of this disease, it may appear as a large surface area of leukoplakia or multiple exophytic verrucous lesions or a large tumor mass. As many as 90% of patients will have a skin and/or nail fungal condition as well as oral candidiasis.
Radiographic presentation
None until its later stages, when it invades bone to produce an irregular osteolysis.
Differential diagnosis
In its early and intermediate stages, PVL will appear similar to candidiasis and lichen planus. As it progresses, verrucous carcinoma unrelated to PVL and hypertrophic candidiasis may be considered. In its aggressive late stage, invasive squamous cell carcinoma unrelated to PVL and high-grade mucoepidermoid carcinoma and other high-grade cancers are considerations.
Microscopic features
Early stages will show a range from normal-appearing epithelium to varying degrees of dysplasia. A peculiar bulbous endophytic protrusion of epithelium with an intact basement membrane is often seen. In intermediate stages, a verrucous proliferation is noted. In later stages, an invasive squamous cell carcinoma with abundant mitotic figures is seen.
Suggested course of action
Suspicious cases should undergo an incisional biopsy and/or referral to an oral and maxillofacial surgeon.
Treatment
Initially, areas of clinical involvement are excised and skin grafted. Follow-up chemoprevention is not curative but slows down the progression of the disease. Oral nystatin swish and spit, oral-systemic griseofulvin 250 mg twice daily, and tocopherol 100 mcg daily are often used as chemoprevention. Advanced cases are treated with resection and sometimes chemotherapy or radiotherapy, although their value is uncertain.
Verrucous Carcinoma
Nature of disease
A noninvasive and nonmetastasizing exophytic proliferation of malignant epithelial cells. Note: Although often attributed to smokeless tobacco use, there is no clear evidence that smokeless tobacco other than betel nut/slaked lime causes verrucous carcinoma.
Predilections
Mostly adults over 50 years of age. Slightly more common in males than in females and mostly occurs on the buccal mucosa or gingiva. No racial predilection is known.
Clinical features
Lesions will appear as red-white velvet-textured exophytic masses arising from a broad surface area. Satellite lesions are common. Lesions are asymptomatic without induration or suspicious lymphadenopathy.
Radiographic presentation
None.
Differential diagnosis
Benign hyperkeratosis, squamous papillomas, and invasive squamous cell carcinoma should be considered. Hypertrophic candidiasis and lichen planus may also be considered.
Microscopic features
Bulbous endophytic and exophytic epithelial proliferations are seen together with an intact basement membrane and subjacent inflammation in the mucosa beneath the basement membrane. There are usually clefts with a hyperparakeratinization between the broad exophytic proliferations.
Suggested course of action
Incisional biopsy to include at least one margin and a depth into bleeding submucosa and/or referral to an oral and maxillofacial surgeon.
Treatment
Wide local excision into the submucosa while observing a 1.0- to 1.5-cm margin controlled by frozen sections.
Hypersensitivity Mucositis
Nature of disease
An immune-based inflammatory response to topical or systemic agents.
Predilections
Can occur at any age but is mostly seen in adults taking systemic medications. May also be attributed to some ingredients in dental preparations or to food spices. Systemic medications most well known to cause oral hypersensitivity lesions are thyroid-replacement and anti-hypertension drugs. Topical agents more commonly associated with mucosal reactions are common flavoring and nickel-containing materials. No sex or racial predilection is known.
Clinical features
Lesions will