Desquamative gingivitis in dermatological disorders

Lichen planus

Lichen Planus (LP) is the most common cause of desquamative gingivitis. It is responsible for nearly 45% of cases of desquamative gingivitis.⁶ It most frequently affects the posterior buccal mucosa, lateral border of the tongue and gingiva.⁷ Desquamative gingivitis may be the only manifestation in 10% of cases of oral LP.⁵ The atrophic variant of oral LP often presents on the gingiva and in some cases may affect the whole thickness of the attached gingiva. Plaque induced gingivitis may complicate the clinical picture. Lesions of oral LP may be associated with vulvovaginal lesions of LP(“vulvovaginal-gingival syndrome”).^4,7 The vulvovaginal and gingival lesions are either coincidental, as is often the case, or one often follows the other within two years of its onset. Lichenoid oral lesions may also exist alongside lichen sclerosus lesions in the mouth and vulva which is referred to as “lichen planus/lichen sclerosus overlap syndrome”.⁸ Local examination of the gingiva in such patients may reveal erythematous gingiva with faint keratotic lines. The rest of the oral mucosa and the skin need to be carefully checked for classical lichenoid lesions [Figure 2].

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Figure 2:

Desquamative gingivitis in a female patient with lichen planus

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Immune mediated blistering disorders

Desquamative gingivitis is a presenting feature, in virtually every patient with mucous membrane pemphigoid.³ Nearly 35% of the cases of desquamative gingivitis have been attributed to mucous membrane pemphigoid [Figure 3].² A common presentation is erythematous gingiva with lack of stippling which spreads apically from the gingival margins to the alveolar mucosa. There may be areas of superficial erosions with tags of detached epithelium in the gingiva.⁹ Vesiculobullous lesions and positive Nikolsky’s sign may be present.

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Figure 3:

Desquamative gingivitis as an early presenting feature of mucous membrane pemphigoid

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Oral lesions are cardinal manifestations of many disorders of pemphigus group, particularly pemphigus vulgaris. It is characterized by severe gingival desquamation, erosions and ulcers from ruptured bullae. ⁵ Positive Nikolsky’s sign can be seen. Desquamative gingivitis is also seen in IgA pemphigus (intraepidermal IgA pustulosis) and paraneoplastic pemphigus.⁶ In some cases, oral lesions may be the sole manifestation which may accompany, precede, or follow cutaneous lesions.

Though desquamative gingivitis is rare in dermatitis herpetiformis, erythema with white lesions and erosions may be seen in the buccal and lingual gingiva along with a background of gluten-sensitive enteropathy.⁵

Adult linear IgA disease can produce oral vesicles, ulcers and gingival lesions in the form of desquamative gingivitis.¹⁰ Chronic bullous disease of childhood may present similarly and additionally be associated with scarring.¹¹

Chronic ulcerative stomatitis

It is a rare recalcitrant autoimmune disease characterized by painful chronic oral lesions similar to erosive LP. The findings include ulcers and erosions in the gingival, buccal and lingual mucosae. It may rarely be associated with genital and conjunctival mucosal lesions. DIF studies show nuclear deposits of IgG in basal 1/3^rd of the epithelium.¹¹

Erythema multiforme major

Erythema multiforme major almost always involves the oral mucosa. The oral lesions may arise before or after the onset of cutaneous lesions or in isolation. It includes swollen lips, labial erosions, intra-oral blisters and serosanguinous discharge.⁵ Though gingival involvement is reported to be uncommon, when present it may be overlooked due to the extensive oral ulceration and sloughing seen in this condition.¹²

Pyostomatitis vegetans

It is a rare disorder characterized by erythema and edema of oral mucosa with numerous small superficial yellow pustules. It may also have lesions of desquamative gingivitis. It is found to be clinically associated with inflammatory bowel disease - both Crohn’s disease and ulcerative colitis. The oral lesions follow the onset of gastrointestinal symptoms.¹³

Epidermolysis bullosa acquisita

This condition is characterized by oral mucosal fragility with recurrent blistering, scarring, microstomia and enamel defects. It may also have gingival involvement, in the form of gingival hyperkeratosis (as seen in epidermolysis bullosa simplex, Dowling – Meara type ) and desquamative gingivitis.¹⁵

Plasma cell gingivitis

This hypersensitivity reaction to mouth rinses, flavored mint/ chewing gum, etc., though rare, may present as desquamative gingivitis. It is referred to as stomatitis medicamentosa. A similar reaction may occur as a contact allergic response to drugs taken orally or parenterally (eg: penicillin, aspirin burns) and is referred to as stomatitis venenata.¹⁶

Toxic epidermal necrolysis

This condition may manifest with extensive gingival desquamation. This is particularly seen in patients with HIV-AIDS and is preceded by systemic symptoms such as fever, cough, malaise, etc.⁵

Tables 2 and 3 summarise the salient features of desquamative gingivitis in various dermatological and non-dermatological conditions, respectively.

Diagnosis of desquamative gingivitis

• History: History taking should focus on the onset, progression, aggravating factors, relieving factors and prior treatment taken. Patients may be asymptomatic or more commonly complain of persistent soreness of the gingiva, mild burning sensation or intense pain of the affected site.² The symptoms may be aggravated by intake of acidic foods such as citrus fruits. Also, look for intentional or unintentional aggravating local factors like masticatory forces, poor dental restorations, plaque accumulation, etc. Symptoms are known to occur in cycles of remission and exacerbation

• Examination: General examination is pivotal in determining the involvement of other mucosal surfaces and the pattern of distribution. A thorough examination to find out any cutaneous involvement is also important. It is also essential to identify any underlying systemic disease.

Local examination

Desquamative gingivitis clinically appears as fiery red, glazed, atrophic or eroded gingiva, particularly seen on the buccal/labial surface of the gingival epithelium (free and attached gingiva).^4,5 Less severe cases present with erythema and edema. In cases with greater severity, paraesthesia, mucosal desquamation with blister formation, erosions and ulcerations can be seen. A thorough local examination is important to not only characterize the peculiar features of desquamative gingivitis in different dermatoses [Table 2] but also to elicit Nikolsky’s sign (formation or spread of blister on application of lateral pressure to the normal looking mucosa) which is diagnostic of many vesiculobullous diseases.

Investigations

Histopathological examination

Biopsy of the perilesional mucosa is preferred to that of the ulcerated mucosa.^2,5

Formalin-fixed, paraffin-embedded tissue stained with conventional H and E is obtained for light microscopy examination [Figure 4].

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Figure 4:

Histopathology showing sub-epithelial cleft and basal cell degeneration, along with chronic inflammatory cells and haemorrhagic areas, in mucous membrane pemphigoid (H and E, ×100, low power magnification)

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Table 4 highlights the histological features of common mucocutaneous diseases presenting with desquamative gingivitis.

Table 4: Histological findings of desquamative gingivitis in various dermatological conditions

Disease	Histopathology
Lichen planus	Hyperkeratosis, hydropic degeneration of the basal layer and sawtoothed rete pegs; lamina propria shows dense, bandlike infiltrate, primarily of lymphocytes; colloid bodies are present
Mucous membrane pemphigoid	Subepithelial clefting with epithelial separation from the underlying lamina propria with an intact basal layer
Pemphigus	Intraepithelial clefting above the basal cell layer; basal cells have a characteristic “row of tombstone” appearance; acantholysis is present
Chronic ulcerative stomatitis	Hyperkeratosis, acanthosis, basal cell layer liquefaction, subepithelial clefting and chronic lymphohistiocytic infiltrate in a bandlike configuration
Dermatitis herpetiformis	Collection of neutrophils, eosinophils and fibrin in the connective tissue papillae
Bullous pemphigoid	Subepithelial clefting with epithelial separation from the underlying lamina propria, leaving an intact basal layer
Systemic Lupus erythematosus	Hyperkeratosis, basal cell degeneration, epithelial atrophy and perivascular inflammation
Epidermolysis bullosa acquisita	Subepithelial clefting with epithelial separation from the underlying lamina propria, leaving an intact basal layer

Immunofluorescence studies

In direct immunofluorescence, unfixed frozen sections are incubated with a variety of fluorescein-labeled, antihuman sera (i.e., anti-IgG, anti-IgA, anti-IgM, antifibrin and anti-C3) [Figure 5]. With indirect immunofluorescence, unfixed frozen sections of oral or esophageal mucosa from monkeys are first incubated with the patient’s serum to allow for the attachment of any antibodies to the mucosal tissue. Table 5 summarises the immunofluorescence findings in select dermatological conditions presenting as desquamative gingivitis.

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Figure 5:

Direct immunofluorescence showing a linear deposition of IgG and C3 at the dermo-epidermal junction, in mucous membrane pemphigoid

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Table 5: Immunofluorescence findings of desquamative gingivitis in various dermatological conditions

Disease	DIF	IIF
		Mucosa	Serum
Lichen planus	Fibrillar deposits of fibrin at the dermal-epidermal junction	Negative	Negative
Mucous membrane pemphigoid	Linear deposits of C3 with or without IgG at the basement membrane zone in almost all cases	Same as for perilesional mucosa	Basement membrane zone(IgG) antibodies in 10% of cases
Pemphigus	Intercellular deposits in the epithelium; IgG in all cases and C3 in most cases	Same as for perilesional mucosa	Intercellular (IgG) antibodies in ≥90% of cases
Chronic ulcerative stomatitis	IgG deposits in the nuclei of the basal layer of epithelial cells	Same as for perilesional mucosa	ANA specific for basal cells of stratified squamous epithelium
Dermatitis herpetiformis	IgA deposits in the dermal papillae in 85% of cases	Same as for perilesional mucosa	IgA endomysial antibodies in 70% of cases; antigliadin antibodies in 30% of cases
Bullous pemphigoid	Linear deposits of C3 with or without IgG at the basement membrane zone in almost all cases	Same as for perilesional mucosa	IgG antibodies against basement membrane zone in 40%-70% of cases
Systemic lupus erythematosus	IgG or IgM with or without C3 deposits at the dermal-epidermal junction	Same as for perilesional mucosa	ANA in >95% of cases; DNA and ENA antibodies in >50% of cases
Epidermolysis bullosa acquisita	Linear deposits of IgG and C3 at the basement membrane zone in almost all cases	Same as for perilesional mucosa	Basement membrane zone(IgG) antibodies in 25% of cases

DIF: Direct immunofluorescence, IIF: Indirect immunofluorescence, ANA: Anti-nuclear antibodies, ENA: Extractable nuclear antigen.

Foreign body gingivitis

It is a non-plaque induced chronic inflammatory process that may seldom involve the gingiva.¹⁷ It is triggered by the introduction of foreign particulate material such as dental restorative material or dental prophylaxis paint. It may present as a lichenoid or granulomatous response masquerading as desquamative gingivitis.

Candidiasis

Oral candidiasis, particularly in immune-compromised patients, may involve the gingiva. This gives it an erythematous and granular appearance, often mistaken as desquamative gingivitis.¹⁸

Wegener’s granulomatosis

Oral lesions are seen in around 6% of cases.¹⁹ They manifest as mouth ulcers and erythematous, painful gingival enlargement (“strawberry gingivitis”). This can be confused with desquamative gingivitis.²⁰

Graft-versus-host disease

Following allogeneic hematopoetic stem cell transplantation oral graft-versus-host disease which affect the gingiva may occur. This results in epithelial desquamation, erythema and erosions of the buccal, glossal and labial gingiva, presenting a “desquamative gingivitis” type of clinical appearance.²¹

Kindler’s syndrome

The oral manifestations include erosive and erythematous appearance of the gingiva. The salient cutaneous features include neonatal bullae, poikiloderma, photosensitivity and acral atrophy.²²

Factitious gingivitis

Self- inflicted oral injuries, though atypical in their site and nature of occurrence, may potentially appear like lesions of desquamative gingivitis.²³

Squamous cell carcinoma

Gingival squamous cell carcinoma arising from the keratinized mucosa results in gingival enlargement and may be misdiagnosed as desquamative gingivitis.¹⁹