BULLOUS

PEMPHIGOID
DEPARTMENT DERMATO-
VENEREOLOGI
FACULTY OF MEDICINE
HASANUDDIN UNIVERSITY
DEFENITION
Bullous pemphigoid (BP) is a
subepidermal blistering skin
disease that is characterized by
large, tense blisters and the
immunopathologic finding C3 and
Ig G at the epidermal basement
membran.
EPIDEMIOLOGY
 Usually occurring in elderly ( > 60
years old)
 There’s no known ethnic, racial or
sexual predilection
ETIOLOGY
 No well defined causal agents
 It has been associated with :
 Several drugs
 Local irritation and damage to the
skin
 Some physical agents
 Systemic and autoimmune diseases
PATHOGENESIS
Recognition of BP antigen as “foreign”
protein
▼
BP autoantibody formation (Ig G class)
▼
BP autoantibody binds to DE junction
▼
PATHOGENESIS
Complement Fixation
▼
Recruitment of mast cell to DE junction
▼
Release of ECF-A
▼
PATHOGENESIS
Chemotaxis of eosinophilik to DE
junction
▼
Release of proteolytic enzymes or
soluble mediators
▼
Intra-lamina lucida separation
CLINICAL
MANIFESTATION
 A tense blisters that arises on either a
flesh colored or an erythematous base.
 Bullae are usually filled with clean fluid
but may be hemorrhagic.
 Most common on flexura body surfaces
 Pruritus is usually generalized and often
intense.
 Healing occurs without scarring.
HISTOPHATOLOGY
 The presence of subepidermal
blisters formation with eosinophils
within the blisters cavity and the
upper papillary dermis
 Superficial dermal infiltrate
LABORATORY
FINDINGS
 Presence of Ig G class circulating
anti-epithelial basement
membrane autoantibodies
DIAGNOSIS
 Best made by correlation of clinical
routine, histologic, and
immunopathologic findings
DIFFERENTIAL
DIAGNOSIS
 Cicatrial pemphigoid
 Epidermolysis bullosa acquisata
 Bullous eruption of Systemic Lupus
Erythematous
 Chronic bullous dermatosis
 Dermatitis herpetiformis.
 Pemfigus vulgaris.
TYPES OF BP
 Pemphigoid nodularis
 Localized disease
 Localized vulvar pemphigoid
 Localized oral pemphigoid
 Pemphigoid vegetans
 Lichen planus pemphigoides
TREATMENT
 TOPICAL :
 Methylprednisolone 15 mg/kg in 16
ml of bacteriostatic water over a
period of 30-60 minutes daily for
three doses
 Sodium succinate salt 0,4 mg/kg daily
for at least a week
TREATMENT
 SYSTEMIC :
 Prednisolone PO 20-40 mg / day and
reduced to 15-20 mg/day after a few
weeks
 Immunosuppresant
PROGNOSIS
 Usually self limited disease oven 5-
6 years period
 Most patients have a lasting
remission
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