VESICOLOBULLOUS LESIONS(IMMUNOLOGIC DISEASES - PEMPHIGUS VULGARIS)

Question 1

CONCEPT

Answer 1

GROUP OF AUTOIMMUNE MUCOCUTANEOUS DISEASES

Question 2

CHARACTERISTICS

Answer 2

INTRAEPITHELIAL BLISTER FORMATION (RESULTS FROM A BREAKDOWN OR LOSS OF INTERCELLULAR ADHESION PRODUCING EPITHELIAL CELL SEPARATION (ACANTHOLYSIS)

Question 3

MECHANISM

Answer 3

• WIDESPREAD SUPERFICIAL ULCERATION FOLLOWING RUPTURE OF THE BLISTERS LEADS TO PAINFUL DEBILITATION, FLUID LOSS, ELECTROLYTE IMBALANCE
• BLISTERING: DUE ABNORMAL PRODUCTION OF AUTOANTIBODIES (DIRECTED AGAINST THE EPIDERMAL CELL SURFACE GLYCOPROTEINS, DESMOGLEIN 3 AND DESMOGLEIN 1(COMPONENTS OF DESMOSSOMOS)
• RESULT OF IMMUNOLOGIC ATTACK ON THE DESMOSOMES: SPLIT DEVELOPS WITHIN THE EPITHELIUM, CAUSING A BLISTER TO FORM WHICH IS LARGE SUPERFICIAL FRAGILE THAT BURST EASILY

Question 4

TYPES

Answer 4

1) PEMPHIGUS VULGARIS (MOST COMMON)
2) PEMPHIGUS VEGENTANS
3) PEMPHIGUS FOLIACEUS
4) PEMPHIGUS ERYTHEMATOSUS

Question 5

UNTREATED

Answer 5

PATIENT’S DEATH

Question 6

ORAL LESIONS

Answer 6

• FIRST SIGN OF THE DISEASE (DIFFICULT RESOLVE WITH THERAPY)
• LARGE AREAS ULCERATION: ANY ORAL MUCOSA (PALATE, LABIAL MUCOSA, BUCCAL MUCOSA, VENTRAL TONGUE, GINGIVA
• INTACT BULLAE: RARELY SEEN IN ORAL CAVITY

Question 7

TARGET PUBLIC

Answer 7

ADULTS (50 YEARS AVERAGE)

Question 8

HYSTOPATHOLOGIC FEATURES

Answer 8

• VESICLES/BULLAE FORMED ENTIRELY INTRA-EPITHELIALLY, JUST ABOVE THE BASAL LAYER OF CELLS (SUPRABASILAR VESICLES)
• SOMETIMES ENTIRE SUPERFICIAL LAYERS OF THE EPITHELIUM ARE STRIPPED AWAY, LEAVING ONLY THE BASAL CELLS, WHICH HAVE BEEN DESCRIBED AS “ROW OF TOMBSTONES”
• CELLS OF THE SPINOUS LAYER OF THE SURFACE EPITHELIUM TYPICALLY APPEAR TO FALL APART (ACANTHOLYSIS), AND LOOSE CELLS TEND TO ASSUME A ROUNDED SHAPE (TZANCK CELLS)

Question 9

DIAGNOSIS

Answer 9

• STANDARD BIOPSY/IMMUNOFLUORESCENCE (DIF) TESTING (DEMONSTRATE AUTOANTIBODY ALREADY ATTACHED TO THE TISSUE)
• WITH THIS PROCEDURE: ANTIBODIES (IgG/IgM) AND COMPLEMENT COMPONENTS (C3) CAN BE DEMONSTRATED IN THE INTERCELLULAR SPACES BETWEEN THE EPITHELIAL CELLS
• CHICKEN WIRE PATTERN

Question 10

TREATMENT

Answer 10

SYSTEMIC CORTICOSTEROIDS (PREDNISONE)/COMBINATION WITH OTHER IMMUNOSUPPRESSIVE DRUGS