VESICOLOBULLOU LESIONS (IMMUNOLOGIC DISEASES - MUCOUS MEMBRANE PEMPHIGOID)

Question 1

CONCEPT

Answer 1

• CHRONIC BLISTERING OR VESICULOBULLOUS DISEASE AFFECTS PREDOMINANTLY ORAL/OCULAR MUCOUS MEMBRANES
• NIKOLSKY’S SIGN

Question 2

MECHANISM

Answer 2

• ANTIBODIES (IgG/IgA) ATTACK FIBROUS ATTACHEMENT OF THE BASEMENT MEMBRANE AND COMPONENTS OF THE HEMIDESMOSOMES (BP180, LAMININ 5) WICH ATTACH THE EPIDERMAL CELLS TO THE BASEMENT MEMBRANE
• NO EVIDENCE ACANTHOLYSIS/TZANK CELLS

Question 3

TARGET

Answer 3

ADULTS (FEMALES) - (50-60 YEARS ONSET OF DISEASE)

Question 4

SITES

Answer 4

ORAL (MOST PATIENTS), CONJUNCTIVAL, NASAL, ESOPHAGEAL, LARYNGEAL, VAGINAL MUCOSA

Question 5

ORAL LESIONS

Answer 5

• BEGIN: VESICLES OR BULLAE (CLINICALLY IDENTIFIED BLISTERS RUPTURE, LEAVING LARGE, SUPERFICIAL, ULCERATED AND DENUDED AREAS OF MUCOSA
• GINGIVA: INVOLVED (RESULTING SLOUGHING AND PEELING DURING EATING OR TOOTH BRUSHING - DESQUAMATIVE GINGIVITIS)

Question 6

ULCERATED LESIONS

Answer 6

PAINFUL, PERSIST FOR WEEKS TO MONTHS (UNTREATED)

Question 7

COMPLICATION

Answer 7

OCULAR INVOLVEMENT (MAY LEAD TO BLINDNESS)

Question 8

BULLAE

Answer 8

SUBPITHELIAL (SPILT BETWEEN THE SURFACE EPITHELIUM AND THE UNDERLYING CONNECTIVE TISSUE IN THE REGION ON THE BASEMENT MEMBRANE)

Question 9

DIAGNOSIS

Answer 9

STANDARD BIOPSY (CONFIRMATION OF MUCOUS MEMBRANE PEMPHIGOID CAN BE MADE WITH THE USE OF DIRECT IMMUNOFLUORESCENCE - DIF - TEST)

Question 10

TREATMENT

Answer 10

CORTICOSTEROIDS