ABSTRACT
Intravenous immunoglobulin (IVIG) has been used as an immunomodulator in a range of specialties including hematology, neurology, rheumatology and, more recently, dermatology1. Dermatological conditions treated with high-dose IVIG represent a small but expanding area encompassing autoantibody-mediated disorders (e.g. blistering diseases) as well as diseases characterized by cellular and complement-mediated pathogenesis (e.g. dermatomyositis) (Table 1). Dermatological disorders have contributed considerably to our understanding of the mechanism of action of IVIG2, and in view of the ready access to skin can be easily documented photographically or by established scoring systems as well as by biopsy. There has been the perception that dermatological conditions are trivial and not life-threatening, and this has led to these conditions sometimes being given a lower priority than other diseases. It is clear, however, that indications such as toxic epidermal necrolysis (TEN) have a mortality of between 25 and 35%, and that other conditions have a major impact on the quality of life of the sufferers. The main indications for the use of high-dose IVIG (outside conditions such as Kawasaki syndrome
Condition Number of reports (Medline)
Kawasaki syndrome 242 Dermatomyositis 80 Pemphigus vulgaris 28 Epidermolysis bullosa acquisita
Atopic dermatitis 18 Bullous pemphigoid 13 Toxic epidermal necrolysis
Chronic urticaria 7
Mucous membrane pemphigoid
Gestational pemphigoid 2 Linear IgA disease 2 Pyoderma gangrenosum 6 Scleromyxedema 3 Erythema multiforme 4 Pretibial myxedema 2 Psoriasis 1 IgA, immunoglobulin A
and TEN, where treatment is generally a single cycle) are treatment-resistant disease or unacceptable side-effects from conventional therapy3.
