Bullous Pemphigoid
• Autoimmune, subepidermal, blistering skin disease
• The single most common immunobullous disease in dermatology
Clinically:
• Usually an older patient (> 60 years), often
with other co-morbidities
• Does not normally involve the mucosa (unlike pemphigus)
• May begin as an urticarial eruption, often intensely
pruritic
• Tense blisters and bullae
• Common locations include the abdomen, flexor forearms,
and inner thighs
Nikolksky’s
sign is negative
• Rarely involves mucous membranes: 10% to 35%
• Drugs associated with bullous pemphigoid include: furosemide, ibuprofen and other
nonsteroidal anti-inflammatory agents, captopril, penicillamine, and
antibiotics
Histology: subepidermal blistering process, usually
with prominent eosinophils in the blister cavity and superficial dermis.
• Antigens
Bullous pemphigoid antigen 1 (BPAgI) 230 kDa- Intracellular portion
of hemidesmosome
plaque, part of the plakin superfamily,.
Bullous pemphigoid antigen 2 (BPAgII) 180 kDa, type XVII collagen, Transmembranous
protein with a collagenous extracellular domain.
Prognosis:
(BP180) correlate with
disease activity .
• Generally, a self-limited disease with good Prognosis . Fifty
percent enter remission within 2 to 6 years
• Therapy: topical steroids or oral prednisone
alone or in combination with tetracycline and nicotinamide, azathioprine,
cyclophosphamide, dapsone, methotrexate, plasmapheresis, and intravenous
immunoglobulin (IVIG)
