Clinical relevance of autoantibodies in patients with autoimmune bullous dermatosis

Abstract

The authors present their experience related to the diagnosis, treatment, and followup of 431 patients with bullous pemphigoid, 14 patients with juvenile bullous pemphigoid, and 273 patients with pemphigus. The detection of autoantibodies plays an outstanding role in the diagnosis and differential diagnosis. Paraneoplastic pemphigoid is suggested to be a distinct entity from the group of bullous pemphigoid in view of the linear C3 deposits along the basement membrane of the perilesional skin and the "ladder" configuration of autoantibodies demonstrated by western blot analysis. It is proposed that IgA pemphigoid should be differentiated from the linear IgA dermatoses. Immunosuppressive therapy is recommended in which the maintenance dose of corticosteroid is administered every second day, thereby reducing the side effects of the corticosteroids. Following the detection of IgA antibodies (IgA pemphigoid, linear IgA bullous dermatosis, and IgA pemphigus), diamino diphenyl sulfone (dapsone) therapy is preferred alone or in combination. The clinical relevance of autoantibodies in patients with autoimmune bullous dermatosis is stressed.

Figure 1

Bullous pemphigoid and pemphigus vulgaris. (a) The clinical picture of generalized pemphigoid with tense blisters, crusts, and erythematous plaques. (b) The histological picture of BP; subepidermal blister. (c) Linear basement zone deposits of IgG in BP. (d) Circulating IgG antibodies bind to the epidermal site of SSS in a patient with BP. (e) The flaccid blister in PV. (f) The histological picture of PV with intraepidermal blister. (g) DIF analysis demonstrates binding of C3 to the intercellular cement substance (ICS) in the upper stratum Malpighii. (h) An IIF study with monkey esophagus as substrate demonstrates the presence of anti-ICS antibody with antihuman IgG conjugate.

Figure 2

Western blot investigations. (a) A “ladder” configuration can be seen in paraneoplastic pemphigus. (b) A “ladder” configuration of paraneoplastic pemphigoid on western blot analysis.

Figure 3

Epidermolysis bullosa aquisita. (a) Erosions in the oral cavity in EBA. (b) Blisters on the wrist in EBA. ((c) and (d)) Blisters on the fingertips in EBA.

Figure 4

Juvenile bullous pemphigoid and bullous eruption of systemic lupus erythematosus. ((a) and (b)) Tense blisters and hemorrhagic crusts on the extremities of a 5-year- old boy with juvenile BP. (c) Erythematous plaques and some blisters in a VBSLE patient. (d) Circulating IgG antibodies bind to the dermal site of SSS in VBSLE.