Continuing Education: Etiology, Diagnosis, and Treatment of Desquamative Gingivitis

The term desquamative gingivitis (DG) describes a clinical condition in which the gingival tissues are erythematous, blistering, and eroding. It is not a diagnosis but is instead a term applied to the manifestation of a multitude of mucocutaneous, systemic, allergic, and immunologic diseases. The majority of cases are caused by oral lichen planus, pemphigus vulgaris, and mucous membrane pemphigoid, but many less common sources need to be considered in the differential diagnosis as well. These include erythema multiforme, lupus erythematosus, drug-induced lesions, graft versus host disease, chronic ulcerative stomatitis, plasma cell gingivitis, linear IgA disease, dermatitis herpetiformis, psoriasis, epidermolysis bullosa acquisita, paraneoplastic and neoplastic disorders, and allergic reactions. The dental clinician can play a crucial role in the diagnosis of these conditions, some of which can cause significant morbidity and even mortality.

jcg140013f1

  • Oral Lichen Planus

I have previously discussed Lichen planus on my blog, including its management.

  • Pemphigus Vulgaris

PV is a rare, chronic, autoimmune disorder that results in blistering of the skin and mucosa. It has an incidence of one to five cases per one million people per year and is usually seen in middle-aged adults especially of Mediterranean, South Asian, and Jewish descent, with rare cases in children. Though very uncommon, if untreated it can lead to death from fluid loss, electrolyte imbalance, and septicemia. PV accounts for 3% to 15% of cases of DG and involvement of the oral mucosa in the early stages of PV can be observed in 70% of cases. Lesions of this disease present as fluid-filled blisters that rupture, leaving behind irregularly-shaped, erythematous, painful ulcerations. A positive Nikolsky sign where slight rubbing of the skin or mucosa elicits bulla formation in affected areas is a characteristic feature.

dpg140001fa
Biopsies should be taken from perilesional, not ulcerated tissue. Specimens usually show intraepithelial separation above the basal cell layer leaving behind a characteristic “tombstone” pattern. The cells of the epithelium break apart individually as diagnostic rounded cells called “Tzanck cells” in cytologic smears.

OLYMPUS DIGITAL CAMERA

Diagnosis is confirmed with direct immunofluorescence where antibody and complement are noted in the intercellular spaces of the epithelium and indirect immunofluorescence, which demonstrates circulating epithelial autoantibodies in patient serum. ELISA (enzymelinked immunosorbant assay) can be used to detect circulating autoantibodies as well.

27886
Treatment includes systemic corticosteroids, usually prednisone, with immunosuppressive agents, such as azathioprine or cyclosporin. Topical corticosteroids have been used with good results in the oral cavity as well, but treatment must include systemic therapy. Referral to a dermatologist who has experience with immunosuppressives is recommended. PV can undergo complete remission with therapy; however, it has a 5%-10% mortality rate usually due to long-term need for systemic corticosteroids.

  • Mucous Membrane Pemphigoid

MMP is a chronic, blistering, mucocutaneous autoimmune disease. It may represent a group of diseases all of which involve tissue-bound autoantibodies that are directed against components of the basement membrane.  The lesions of MMP are commonly found on the gingiva and may be painful. The average age of onset is between 50 and 60 years old, and women are affected twice as often as men. MMP can affect extraoral sites with conjunctival involvement leading to blindness and laryngeal involvement causing airway obstruction. It has classically been estimated to be the cause of 35%-48% (the majority) of cases of DG, but some recent studies suggest that it may be closer to 8%-14%. MMP appears clinically similar to PV; however, the clinician is more likely to be able to identify the vesicles or bullae of MMP at presentation due to subepithelial clefting of MMP versus intraepithelial clefting of PV. Positive Nikolsky sign is also observed. Biopsy should be taken of perilesional tissue which shows a split between the surface epithelium and underlying connective tissue below the basement membrane.

18101782814e470ec8a74f4

Microscopic diagnosis should be confirmed with direct immunofluorescence, which highlights a continuous linear band of immunoreactants at the basement membrane zone. The immunoreactants are usually C3 and IgG; but IgA and IgM may also be seen.

Referral to an ophthalmologist is mandatory even if the patient has no ocular symptoms as the scarring of MMP can cause blindness. If only oral lesions are present, treatment with topical corticosteroids in a custom tray with emphasis on oral hygiene is appropriate. If extraoral sites are involved or the patient is not responding to topical treatment, systemic corticosteroids and immunosuppressants should be prescribed.

  • Erythema Multiforme

EM is a mucocutaneous blistering and ulcerative condition that has an acute onset and is self-limiting but may be chronic and episodic as well. Patients are usually in their third or fourth decades of life and men are affected more often than women. Its cause is uncertain but may be an immunologic response to medications such as NSAIDs, barbiturates, sulfonamides, and certain antibiotics, or infectious agents, especially the herpes simplex virus. The severity of EM varies and includes; EM minor, EM major, Stevens-Johnson Syndrome (SJS) and toxic epidermal necrolysis (TEN).

Orally, hemorrhagic crusting of the lips and cutaneously, target lesions are characteristic symptoms. A positive Nikolsky sign is noted. EM minor is usually self-limiting but EM major and TEN can be progressive and life threatening. A recent study suggests that EM may be responsible for 2% of cases of DG.

erythema_multiforme_1_0306241360275814529
Perilesional biopsy shows subepithelial or intraepithelial vesiculation with mixed inflammatory infiltrate and sometimes basal cell and keratinocyte necrosis. There may be perivascular inflammation as well. Necrosis of the whole dermis or mucosa is seen in severe cases. Immunofluorescence is nonspecific but is helpful in ruling out other vesiculobullous diseases.

Treatment should begin with identification and removal of the cause, if one can be found. Minor forms will regress spontaneously and emphasis should be on hydration and topical analgesics. Use of corticosteroids for all forms of the disease may be detrimental and is not recommended. The best treatment for SJS and TEN is yet to be determined, but patients usually require in-patient treatment.

Let me know what you think about this article in the comments section!

Credits:

Scott Froum, DDS
Dr. Naomi Marie Ramer
Dr. Molly Cohen

Advertisements

Leave a Comment!

Fill in your details below or click an icon to log in:

WordPress.com Logo

You are commenting using your WordPress.com account. Log Out / Change )

Twitter picture

You are commenting using your Twitter account. Log Out / Change )

Facebook photo

You are commenting using your Facebook account. Log Out / Change )

Google+ photo

You are commenting using your Google+ account. Log Out / Change )

Connecting to %s