Chronic nonhereditary blistering disease of the skin
or mucosae. Usually considered an autoimmune disease.
Bullous Pemphigoid Brunsting-Perry type.
Bullous pemphigoid affects the skin; cicatricial
pemphigoid affects the mucosal surfaces of the body and, occasionally, the
Clinical appearance of the eruption and skin biopsy.
Bullous pemphigoid: Multiple tense bullae arising from large, irregular urticarial plaques;
lesions occur most typically on the flexion surfaces of the extremities or
lower torso but may be generalized in severe cases. It may occur at any age,
but is most common in the elderly. Clinical course of exacerbations and
Cicatricial pemphigoid: Bullous eruptions involving mainly the oropharynx, conjunctiva, larynx,
esophagus, genitalia, and anus. The scarring of the mucosal surface as a
consequence of the recurrent blistering can result in blindness, nasal or
laryngeal obstruction, or esophageal stenosis.
It is often associated with dermatitis herpetiformis, also with multiple
sclerosis (with no involvement of mucous membrane, but with an early onset).
It is described as associated with autoimmune thrombocytopenia, nephropathy,
and ulcerative colitis.
History of the associated disease:
renal, ulcerative colitis, multiple sclerosis. History of medications,
usually high-dose corticosteroids, dapsone, azathioprine, and/or other
immunosuppressive agents such as cyclosporin.
The same considerations as for the other
blistering diseases: difficult venous access, friction of the skin or
mucosae may cause blistering; careful padding; avoid tapes; use petroleum
jelly gauze; lubricate blade and tubes well. In case of cicatricial
pemphigoid, airway instrumentation should be avoided if possible.
Many medications can induce
pemphigoid; the more common are furosemide, ibuprofen, d-penicillamine, α-aminobenzylpenicillin, penicillin G,
sulfasalazine, 5-fluorouracil, 8-methoxypsoralen; it is best to avoid these
medications if possible.
Chronic Benign Bullous Dermatosis of Childhood: Large tense
bullae of the skin, resembling bullous pemphigoid, occurring in
preschool-age children. Spontaneous remission usually occurs within 2 or 3
years. Therapy: corticosteroids, sulfa drugs.
Fellner MJ: Drug-induced bullous pemphigoid. Clin Dermatol