Monday, October 24, 2011

A NOTE ON DISEASES CLINICALLY PRESENTING AS DESQUAMATIVE GINGIVITIS


DISEASES CLINICALLY PRESENTING AS DESQUAMATIVE GINGIVITIS
Lichen Planus
Lichen planus is a relatively common, chronic, dermatosis characterized by the presence of cutaneous   violaceous papules that may coalesce to form plaques. The current evidence suggests that lichen planus is an immunologically mediated mucocutaneous disorder where host T lymphocytes play a central role . Although the oral cavity may present lichen planus lesions with a distinct clinical configuration and distribution, the clinical presentation sometimes may simulate other mucocutaneous disorders. Therefore a clinical diagnosis of oral lichen planus should be accompanied by a broad differential diagnosis. Numerous epidemiologic studies have shown that oral lichen planus presents in 0.1% to 4% of the population." The majority of patients with oral lichen planus are middle-aged and older females with a 2:1 ratio of females to males. Although possible, children are rarely affected. In a dental setting, cutaneous lichen planus is observed in about one third of the patients diagnosed with oral lichen planus.  In contrast, two thirds of patients seen in dermatologic clinics exhibit oral lichen planus.
Oral Lesions
Although there are several clinical forms of oral lichen planus (reticular, patch, atrophic, erosive and bullous), the most common are the reticular and erosive subtypes. The typical reticular lesions are asymptomatic, bilateral, and consist of interlacing white lines on the posterior region of the buccal mucosa. The lateral border and dorsum of the tongue, hard palate, alveolar ridge, and gingiva may also be affected. In addition, it is not unusual for the reticular lesions to have an erythematous background, a feature that is associated with the coexistence of candidiasis. Oral lichen planus lesions follow a chronic course and have alternating, unpredictable periods of quiescence and flares.
The erosive subtype of lichen planus is often associated with pain and clinically manifests as atrophic, erythematous areas. Fine white radiating striations are observed bordering the atrophic zones. These areas may be sensitive to heat, acid, and spicy foods.
Gingival Lesions
Up to 10% of patients with oral lichen planus have lesions restricted to the gingival tissue that may occur as one or more types of four distinctive patterns:
1. Keratotic lesions. These raised white lesions may present as groups of individual papules, linear or reticulate lesions, or plaquelike configurations.
2. Erosive or ulcerative lesions. These extensive erythematous areas with a patchy distribution may present as focal or diffuse hemorrhagic areas. These lesions are exacerbated by slight trauma (e.g., toothbrushing).
3. Vesicular or bullous lesions. These raised, fluid-filled lesions are uncommon and short lived on the gingiva, quickly rupturing and leaving an ulceration.
4. Atrophic lesions. Atrophy of the gingival tissues with ensuing epithelial thinning results in erythema confined to the gingiva.

Histopathologic, Direct, and Indirect Immunofluorescence Findings in Selected Conditions That May Present Clinically as Desquamative Gingivitis

     Disease

     Histopathology
            Direct Immunofluorescence
Indirect
Immunofluorescence
Biopsy Perilesional
Mucosa
Biopsy Uninvolved
Mucosa
Pemphigus
Intraepithelial clefting
above the basal cell
layer. The basal cells
have a characteristic
"tombstone" appear-
ance. Acantholysis is
present.
Intercellular deposits in epithelium; IgG
in all cases, C3 in most cases.
Same as perilesional
mucosa.
Intercellular (IgG) anti-
bodies in >90% of
cases.
Cicatricial
pemphigoid
Subepithelial clefting
with epithelial separation from the underlying lamina propria, leaving an intact basal layer.
Linear deposits of
C3, with or without
IgG at the basement
membrane zone in
almost all cases.
Same as perilesional
mucosa.
Basement membrane zone (IgG) antibodies in 10% of cases.
Bullous
pemphigoid
Subepithelial clefting
with epithelial separa-
tion from the under-
lying lamina propria,
leaving an intact basal
layer.
Linear deposits of
C3, with or without
IgG at the basement
membrane zone in
almost all cases.
Same as perilesional
mucosa.
Basement membrane zone (IgG) antibodies in 40% to 70% of cases.
Epidermolysis
bullosa
acquisita
Similar to bullous and
cicatricial pemphigoid.
Linear deposits of
IgG and C3 at the
Basement membrane zone in almost all cases.
Same as perilesional
mucosa.
Basement membrane zone (IgG) antibodies in 25% of cases.
Lichen planus
Hyperkeratosis, hydropic degeneration of the basal layer, "saw-toothed" rete pegs.
The lamina propria
exhibits a dense, band-
like infiltrate primarily
of T lymphocytes. Colloid bodies are present.

Fibrilar deposits
of fibrin at the
dermal-epiderma
junction.
     



      Negative




   Negative
Chronic
ulcerative
stomatitis
Similar to erosive lichen planus
(hype rkeratosis,
acanthosis, basal cell
layer liquefaction, sub-
epithelial clefting, and
lympho-histiocytic
chronic infiltrate in a
bandlike configuration.

IgG deposits in nuclei of basal layer epithelial cells.

Same as perilesional
mucosa.
ANA specific for basal cells of stratified squamous epithelium.
Linear IgA
disease
Similar to erosive lichen planus.
Linear deposits of IgA at the basement membrane zone.
Same as perilesional
mucosa.
IgA basement membrane zone (IgA) antibodies in 30% of cases.
Dermatitis
herpetiformis
Collection of neutro-
phils, eosinophils, and
fibrin in connective
tissue papillae.
IgA deposits in
dermal papillae
in 85% of cases.
IgA deposits in dermal papillae in 100% of cases.
IgA endomysial antibodies in 70% of cases, gliadin antibodies in 30% of
cases.
Systemic lupus
erythematosus
Hyperkeratosis, basal
cell degeneration,
epithelial atrophy,
and perivascular
inflammation.
I g (G or M), with or without C3 deposits at dermal-epidermal
junction.
Same as perilesional
mucosa.
ANA in more than 95% of cases, DNA and ENA
antibodies in more than 50% of cases.
Chronic cutaneous lupus
erythematosus
Hyperkeratosis, basal
cell degeneration,
epithelial atrophy,
and perivascular
inflammation.
Ig (G or M), with or without C3 deposits at dermal-epidermal
junction.
 
       Negative

   Usually negative
Subacute lupus
erythematosus
Less inflammatory cell
infiltrate than systemic
and chronic cutaneous
lupus erythematosus
but with similar
microscopic features.
Ig (G or M), with or without C3 deposits at dermal-epidermal
junction in 60% of cases; granular IgG deposits in basal cell cytoplasm in 30% of cases.

Same as perilesional
mucosa.

ANA in 60% to 90%, SS-A(Ro) in 80%, RF in 30%, and RNP in 10% of cases.


Gingivitis: clinical features. A, Localized, diffuse, intensely red area facial of tooth #7 and dark pink marginal changes in the remaining anterior teeth. B, Generalized papillary gingivitis. C, Generalized marginal inflammatory lesion. D, Generalized diffuse inflammatory lesion. E, Papillary gingival enlargement. F, Different degrees of recession. Recession is slight in teeth #26 and 29 and marked in #27 and 28.
Note the irregular contours of the gingiva in #28 and the lack of attached gingiva in #27. G, Insertion of a probe into the gingival sulcus.
Note the lack of stippling, the slightly rolled margins, and the dark red color. H, Bleeding appears about 30 seconds after probing.

A, Necrotizing ulcerative gingivitis: typical punched out interdental papilla between mandibular canine and lateral incisor. B, Necrotizing ulcerative gingivitis: typical lesions with progressive tissue destruction. C, Necrotizing ulcerative gingivitis: typical lesions with spontaneous hemorrhage. D, Necrotizing ulcerative gingivitis: typical lesions producing irregular gingival contour. E, Primary herpetic gingivostomatitis: typical diffuse erythema. F, Primary herpetic gingivostomatitis: vesicles on the gingiva.
Erosive lichen planus presenting as desquamative gingivitis. The gingival tissues are erythematous, ulcerated, and painful.
Gingival mucous membrane pemphigoid. Lesions of cicatricial pemphigoid confined to the gingival tissues, producing a typical desquamative gingivitis appearance.
Pemphigus vulgaris of the gingiva. Clinical appearance of a patient with pemphigus vulgaris presenting oral lesions confined to the gingiva. The clinical diagnosis was consistent with desquamative gingivitis.
Pemphigus vulgaris of the oral cavity. Multiple and coalescent areas of ulceration covered by necrotic epithelium. This patient presented with large ulcers in the labial mucosa, tongue, and soft palate.
Chronic ulcerative stomatitis. Erythema and ulceration of the gingiva consistent with a clinical diagnosis of desquamative gingivitis.
Linear IgA. Intense erythema and ulceration of the gingiva consistent with desquamative gingivitis.
Lupus erythematosus of the oral cavity presenting as desquamative gingivitis. Intense erythema with ulceration bordered by white radial lines.
Plasma cell gingivitis. The gingiva presents a band of moderate to severe inflammation reminiscent of desquamative gingivitis.
Graft versus host disease in a recipient of an allogenic bone marrow transplant. The maxillary gingiva exhibits features consistent with desquamative gingivitis.
Wegener's granulomatosis affecting the gingi-val tissues. The classic "strawberry gums" appearance of the mandibular gingiva is seen in this patient. A slight resemblance with desquamative gingivitis is evident.
Erythema multiforme. Large, shallow, and painful ulcers involving the labial and buccal mucosae. Hemorrhagic crusting of the mandibular vermilion border of the lips is observed.

Treatment of lichen planus.
 Treatment of pemphigus vulgaris.
Treatment of cicatricial pemphigoid.

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