vesiculobullous lesions pdf.pdf

DENT4008 ORAL MEDICINE 6. Vesiculobullous Lesions Assist. Prof. Elif ÇELEBİ School of Dental Medicine Department of Oral and Maxillofacial Radiology [email protected] LEARNING OUTCOMES • Enlist various diseases that can manifest as vesicular and bullous lesions • Discuss the etiopathoge...

DENT4008 ORAL MEDICINE 6. Vesiculobullous Lesions Assist. Prof. Elif ÇELEBİ School of Dental Medicine Department of Oral and Maxillofacial Radiology [email protected] LEARNING OUTCOMES • Enlist various diseases that can manifest as vesicular and bullous lesions • Discuss the etiopathogenesis, clinical features, investigations, differential diagnosis and management of vesiculobullous lesions of the oral cavity OUTLINE 1. Viral Herpes simplex Varicella Zoster Hand Foot Mouth Disease Herpangina Rubella (measles) 2. Immunological diseases Pemphigus Vulgaris Mucous Membrane Pemphigoid Bullous Pemphigoid Dermatitis Herpetiformis Linear Immunoglobulin A Disease 3. Hereditary Diseases Bullous Epidermoid Pemphigus Group of autoimmune blister diseases of the skin and the mucous membrane characterized by intraepidermal or intraepithelial vesicle or bulla formation. Types: Pemphigus Vulgaris Pemphigus Foliceous Paraneoplastic Pemphigus IgA Pemphigus Drugs induced Pemphigus Vulgaris like lesion. Pemphigus Vulgaris Autoimmune mucocutaneous disease that frequently appears first in the oral cavity and can progress to involve other sites. Desmosomes forms the predominant components of intraepithelial cell adhesion. Specific lgG antibodies bind to an antigen on intercellular desmosomes of the epithelium acantholysis, intercellular spaces, and intraepithelial bulla. Pemphigus Vulgaris Clinical manifestations Skin: Thin-walled bullae on a noninflamed base > rapidly breaks and slowly (months) continues to extend peripherally > denuded skin - peripheral extension of intact bulla by pressure; - positive Nikolsky sign. Pemphigus Vulgaris Clinical manifestations NIKOLSKY'S SIGN Pressure to an apparently normal area Pulling away of the layer of the skin from the basal layer New bulla formed Pemphigus Vulgaris Clinical manifestations-Oral 60% cases start with oral lesions, then slowly (months) develop with skin lesions Most common on buccal mucosa, followed by labial mucosa, palate and gingiva (nonspecific desquamative gingivitis) Pemphigus Vulgaris Clinical manifestations-Oral Bulla on a noninflamed base > rapidly breaks and slowly (weeks) continues to extend peripherally > a thin layer of epithelium peels away > irregular, large, denuded base and ulcers. Pemphigus Vulgaris Clinical manifestations Oral Pemphigus Vulgaris Clinical manifestations Oral Pemphigus Vulgaris Lab tests: Biopsy at advanced edge of the lesion: acantholysis and intraepithelial separation. Tzanck smears: positive for acantholytic cells. Indirect immunofluorescent antibody test (blood sample): detect patient's specific circulating IgG in serum against control desmosome proteins. Direct immunofluorescent test (tissue sample): detect patient's specific lgG of desmosomeantibody complex in situ. Pemphigus Vulgaris Differential Diagnosis • • • Acute Viral İnfection, Erythema Multiforme, Recurrent Aphthous Stomatitis. Treatment: 1) Early diagnosis and treatment. 2) Combination of corticosteroids and immunosuppressive drugs Mucous Membrane Pemphigoid Also called as 'cicatricial pemphigoid', the word cicatricial is derived from the word cicatrix meaning scar. Chronic, recurrent, autoimmune disease that primarily affects mucous membrane and rarely skin (5- 10%). Mucous Membrane Pemphigoid Etiopathogenesis Auto antibodies mainly IgG (97%), C3 complement factor (78%) and to lesser degree IgA (27%), targeted to basal lamina of the epithelium. Mucous Membrane Pemphigoid Age: over 50 years Sex: female :male - 2:1 Site: gingival, buccal mucosa, palate, conjunctiva and skin Mucous Membrane Pemphigoid Skin lesions: Blister in the skin develops in 25 -30 % of the patients may be itchy. Bleeding may occur if traumatized Ocular lesions: Subconjunctival scarring leading to blindness in about 15% of the patients. Initial lesions may be limited to upper tarsal conjunctiva, where they escape detection if the eyelid is not everted. Mucous Membrane Pemphigoid Oral lesions: Blister form first on the gums near the teeth, palate, tongue, lips buccal mucosa, floor of the mouth and throat may be affected, painful and makes it difficult to eat. Lesion occurring in the throat (oesophagus, trachea and larynx) can be life threatening. Thick walled persist for 24 – 48 hours before rupturing and desquamation. Mucous Membrane Pemphigoid Oral lesions: Formation of ulcer surrounded by Erythema, erosion on cheek and vesicles on palate with narrower peripheral extensions. Gingiva is edematous and bright red, involvement is patchy and diffused, Nikolsky's sign may be elicited. Heals without scarring. Mucous Membrane Pemphigoid Oral lesions: Mucous Membrane Pemphigoid Oral lesions: Mucous Membrane Pemphigoid Oral lesions: Mucous Membrane Pemphigoid Lab tests: : Biopsy at advanced edge of the lesion: no acantholysis and subepithelial separation. Tzanck smears: negative for acantholytic cells. Indirect immunofluorescent antibody test: circulating lgG (+) at basement membrane. Direct immunofluorescent test: complex lgG (+) at basement membrane. Mucous Membrane Pemphigoid Differential Diagnosis:     Pemphigus vulgaris Bullous pemphigoid Erythema multiforme Lichen planus Mucous Membrane Pemphigoid Treatment: 1) Early diagnosis and treatment. 2) Choices of treatment for oral lesions: For mild disease: topical and intralesional steroids. For moderate disease: systemic corticosteroids. For severe disease: combination of systemic corticosteroids and immunosuppressive drugs. Bullous Pemphigoid Also called as 'para-pemphigus' or 'aging pemphigus'. Chronic mucocutaneous bullous disease that usually affects older individual. Bullous Pemphigoid Etiopathogenesis It is characterized by tissue-bound and circulating autoantibodies directed against bullous pemphigoid antigens (BP 180, BP 230), components of hemidesmosomes of the epidermal and/or epithelial basal cells that promote dermoepidermal cohesion. Bullous Pemphigoid Clinical features Age: adult above 60 years. Sex- affects women slightly more often than men ( 1.7 : 1 ) Sites: gingival, buccal mucosa, palate, floor of moth and tongue. Bullous Pemphigoid Clinical features The cutaneous lesions are multiform. During the nonbullous stage, they appear as intensely pruritic, erythematous, urticarial-like plaques. Later, tense bullae develop (bullous stage) on normal or erythematous base, 0.5 to 4 cm in diameter, usually containing clear or serosanguineous fluid. The bullae rupture leaving superficial ulcerations covered by hemorrhagic crusts. Bullous Pemphigoid Clinical features The oral mucosa is affected in about 30- 40% of cases, usually after skin involvement. Gingival lesions consists of generalized edema, inflammation and desquamation with localized area of vesicle formation. The oral lesions usually follow cutaneous manifestations and being as bullae that soon rupture, leaving shallow ulcerations. Signs: these vesicles and bullae are relatively thick walled and may remain intact for some days. Skin lesion: rash commonly on scalp, limb NIKOLSKY'S sign is negative Bullous Pemphigoid Clinical features Bullous Pemphigoid Clinical features Bullous Pemphigoid Clinical features Bullous Pemphigoid Differential diagnosis Erosive form of lichen planus. Pemphigus Subepithelial bullous dermatosis Bullous Pemphigoid Diagnosis Clinical diagnosis: bulla present on skin which does not extend peripherally. Laboratory examination: vesicles contains fibrinous exudates admixed with occasional inflammatory cells. Indirect immunofluorescence antibody test Tzanck smear negative to acantholytic cells. Direct immunofluorescence testing and complement fixation test. Bullous Pemphigoid Treatment Advised to maintain oral hygiene. Topical treatment: oral paste or ointment or intralesional steroids. Systemic steroids Immunosuppressive therapy References Jean M. Bruch; Nathaniel Treister. Clinical Oral Medicine and Pathology: Springer. 2016. Joseph Regezi, James Sciubba, Richard Jordan. Oral Pathology Clinical Pathologic Correlations. 7th Edition. Saunders; 2016 George Laskaris. Color Atlas of Oral Diseases: Diagnosis and Treatment. Thieme Medical Publishers; 4th editon Edward W Odell. Cawson's Essentials of Oral Pathology and Oral Medicine E-Book (9th ed.). Elsevier Health Sciences; May 2017