desquamative ginigivitis

desqamative gingivitis

1. DESQUAMATIVE GINGIVITIS

2. Contents Introduction History Epidemiology Classification Disorders associated with desquamative gingivitis Lichen planus Pemphigoid Pemphigus Lupus erythematosus Erythema multiforme Miscellaneous Conditions mimicking desquamative gingivitis Diagnostic pathways Clinical significance and implications Conclusion References

3. Introduction Current classification system Common characteristics Immunomediated pathogenesis Desquamative gingivitis

4. Introduction Gingivosis 75% cases – dermatologic genesis

5. History 1894 Prinz , 1932 McCarthy et al, 1960 Glickman and Smulow , 1964

6. Epidemiologic features Highest prevalence - lichen planus and mucous membrane pemphigoid Rarer conditions Disorder Number of cases of desquamative gingivitis Mucous membrane pemphigoid 35-48% Oral lichen planus 24- 45% Pemphigus vulgaris 3-15%

7. Peak incidence – fourth and fifth decade Female predilection ( Prinz 1932, Meritt 1993) Epidemiology

8. Classification 1) Dermatoses a) Cicatricial pemphigoid b) Pemphigus c) Lichen Planus d) Erythema Multiforme e) Lupus Erythematosis f) Linear IgA disease 2) Hormonal influence a) Estrogen deficiency following oophorectomy & post menopausal women b) Testosterone imbalance c) Hypothyroidism 3) Abnormal response to irritation 4) Chronic infection a) Tuberculosis b) Chronic candidiasis c) Histoplasmosis 5) Aging 6) Idiopathic

9. Disorders associated with desquamative gingivitis General clinical features Predominantly affects women ( Prinz 1932, Meritt 1993) Occurs in 4 th – 5 th decade Red, swollen & glossy gingiva Multiple vesicle and superficial denuded areas Difficult to eat hot, spicy food Nikolsky’s sign may be positive Types

10. Disorders associated with desquamative gingivitis Mild Moderate Severe

11. Disorders associated with desquamative gingivitis Lichen planus Pemphigoid Pemphigus Lupus erythematosus Erythema multiforme Miscellaneous

12. Lichen planus Inflammatory mucocutaneous disorder Immunologically mediated - host T lymphocytes play a central role (Ishii et al, 1987) 0.1 - 4% of the population (Scully et al 1998) Female : Male – 2:1

13. Etiology

14. Oral lesions Relapsing –remitting course Papule arrangement Wickhams striae Various clinical forms Atrophic Erosive Reticular Bullous Lichen planus

15. Painful erythematous areas Sensitive to heat, acid, spicy foods Asymptomatic Bilateral interlacing white strands Erythematous background Reticular Erosive Lichen planus

16. Gingival lesions Keratotic lesions Erosive or ulcerative lesions Vesicular or bullous lesions Atrophic lesions Lichen planus

17. Pathogenesis CD8+ T cell TNF- α Apoptosis CD4+ T cell IFN- γ , IL-2 ↑ immune response Keratinocytes IL-1,8,10,12; TNF- α Lichen planus

18. Histopathology Pullon et al (1969) Irregularity of nuclear membrane Increased thickening and granularity of epithelial tonofibrils Lichen planus

19. Immunopathology Direct immunofluorescence Indirect immunofluorescence Lichen planus

20. Differential diagnosis Lupus erythematosus Chronic ulcerative stomatitis Cicatricial pemphigoid Pemphigus vulgaris Lichen planus

21. Diagnosis of lichen planus Erosive/Ulcerative Asymptomatic No therapy Follow up 6-12 months Severe cases - injections of triamcinolone acetonide (10 to 20 mg) Topical steroids No resolution Resolution Refer to dermatologist Retinoids , Dapsone , Systemic steroids Wean off and monitor Treatment of Lichen planus

22. Recalcitrant cases - 0.1% tacrolimus Antifungal therapy Risk for malignant transformation ~ 5% (Becker et al 2006) Lichen planus

23. Pemphigoid Cutaneous , immune-mediated, subepithelial bullous diseases characterized by separation of the basement membrane zone Bullous pemphigoid Cicatricial pemphigoid

24. Parapemphigus Oral involvement – 1/3 of the patients Histologically , No acantholysis Subepithelial vesicles Epithelium separation Two major antigenic determinants Bullous pemphigoid 230- kDa protein plaque BP1 180-kDa collagen-like transmembrane protein BP2

25. Immunopathology Treatment Primary treatment - moderate dose of prednisone Steroid sparing strategies Localized lesions - high potency topical steroids or tetracycline Bullous pemphigoid

26. Mucous membrane pemphigoid Oral cavity, conjunctiva, mucosa of the nose, vagina, rectum, esophagus, urethra Female predilection Five subtypes Cicatricial pemphigoid Oral pemphigoid Multiple antigens pemphigoid Ocular pemphigoid anti-BP antigen mucosal pemphigoid Anti- epiligrin pemphigoid

27. Oral lesions Areas of erythema , desquamation, ulceration Bullae - thick walled Healing - 3 weeks or longer Ocular lesions Cicatricial pemphigoid

28. BP 1, BP2, laminin 5 and 6, uncein , α 6 β 4integrin Cytokine release Sequestration of leukocytes Complement activation Production of autoantibodies Blister formation Cicatricial pemphigoid Release of proteases, collagenases , elastases

29. Histopathology Non specific Subepithelial vesiculation Intact basal layer Immunopathology Direct immunofluorescence - IgG and C3 Indirect immunofluorescence - positive in <25% Cicatricial pemphigoid

30. Differential diagnosis Cicatricial pemphigoid

31. Treatment of Cicatricial pemphigoid

32. Pemphigus Cutaneous + mucous membranes blisters Types P. vulgaris P. vegetans P. foliaceous P. erythematosus Pemphigus vulgaris

33. 0.1 - 0.5 cases /100,000 individuals/year Higher predilection in women (Robinson et al 1997) Potentially lethal: mortality rate < 10% (Black et al 2005) Etiology Idiopathic Drug induced pemphigus Paraneoplastic pemphigus Pemphigus vulgaris

34. Oral lesions Siegel et al 1994 Painful small vesicles → large bullae Nikolsky’s sign Pemphigus vulgaris Soft palate > buccal mucosa > tongue > lower labial mucosa > gingiva

35. Serpiginous pattern with surface resembling pus Can be wiped off → red base → does not bleed nor painful Pemphigus vegetans

36. Pathogenesis Pemphigus vulgaris Harman et al 2001

37. Histopathology Intraepithelial vesiculation ↓ Fluid filled bulla Pemphigus vulgaris Acantholysis ↓ Intercellular bridges are lost "Tombstone" appearance

38. Cytology Tzanck cells Tzanck test Immunopathology Direct Indirect

39. Differential diagnosis Pemphigus vulgaris

40. Treatment Pemphigus vulgaris Diagnosis of pemphigus vulgaris Primary treatment Secondary treatment Prednisone Decrease dose → maintenance dose Azathioprine Cyclosporine Methyltrexate Plasmaphoresis Refer to dermatologist

41. Lupus erythematosus Clinical presentations Systemic Chronic cutaneous Subacute cutaneous

42. Systemic cutaneous Predilection for females (10:1) Affects vital organs – kidneys, heart Classic cutaneous lesion Oral lesions (36% of SLE patients) - ulcerative or lichen planus-like Tendency to bleed, surrounded by red halo Lupus erythematosus

43. Laboratory test LE cell inclusion phenomenon (Weiss and Swift et al 1955) Immunopathology Direct - Perilesional and normal tissue: Ig and C3 deposits at dermal-epidermal interface Lupus erythematosus

44. Chronic cutaneous Skin lesions – Discoid lupus erythematosus Produce scarring and atrophy Oral lesions - 9% of patients present lichen planus-like plaques Lupus erythematosus Soft palate > buccal mucosa > tongue > lower labial mucosa > gingiva

45. Immunopathology   Subacute cutaneous lupus erythematosus Lupus erythematosus

46. Differential diagnosis Lupus erythematosus

47. Treatment Lupus erythematosus

48. Erythema Multiforme Acute bullous and/or macular inflammatory mucocutaneous disease Types Etiology Erythema multiforme minor Erythema multiforme major/ Stevens-Johnson syndrome Mycoplasma infection Herpes simplex infection Drug reactions

49. Pathogenesis Eversole et al 1994 Erythema multiforme

50. Male predilection Symmetric distribution of macules , papules or vesicles “target”, “iris” or “bulls eye” appearance Erythema multiforme

51. Oral lesions 70% of patients with skin involvement (McCarthy 1980) Multiple, large, painful ulcers with an erythematous border Hemorrhagic crusting of vermilion border of lips Buccal mucosa > tongue > lower labial mucosa > floor of mouth > palate >gingiva Erythema multiforme

52. Steven Johnsons Syndrome Severe bullous form Abrupt occurrence of fever, malaise, photophobia and eruptions of oral mucosa, genitilia and skin Oral lesions → rupture → surfaces covered with thick white or yellow exudate Lips - ulceration with bloody crusting ANUG Erythema multiforme

53. Histopathology Epithelium Lamina propria Immunopathology – negative Erythema multiforme

54. Treatment Erythema multiforme

55. Miscellaneous Chronic Ulcerative Stomatitis Linear IgA Disease Dermatitis Herpetiformis Drug eruptions

56. Drug eruptions Stomatitis medicamentosa Stomatitis venenata or contact stomatitis Etiology Mercurial compounds Toothpaste Clinical presentation Vesicular or bullous Pigmented or nonpigmented macula Erosions Deep ulceration with purpura

57. Conditions Mimicking Desquamative Gingivitis Factitious lesions Candidiasis Graft versus host disease Wegeners granulomatosis Foreign body gingivitis

58. Diagnostic pathways Identification of desquamative lesion Immunopathologic assessment Histopathologic assessment Extra oral examination Intra oral examination Clinical history Diagnosis establishment General and periodontal management

59. Clinical history Onset Chief complaint Infections Topical substances Drug intake Acute, subacute Symptoms Intraoral examination Location Clinical presentation Extraoral examination Other mucosae Skin Internal organs Histopathology Epithelial changes Basement membrane alterations Lamina propria Cell –cell attachment, Acantholysis , epithelial thickening Inflammatory infiltrate BM integrity and staining Immunopathology Serum Involved tissue DIF IIF

60. Clinical significance Direct effect (Ramon et al 1999, Tricamo et al 2006) Indirect effect

61. Koebner phenomenon Clinical significance