Recalcitrant facial pemphigus vulgaris: correlation of skin lesions with the ratio of antidesmoglein antibodies 1 and 3

Abstract

Pemphigus vulgaris (PV) is an autoimmune bullous disease characterized by autoantibodies against desmogleins. We report a case of recalcitrant PV, which progressed from the mucosal to the mucocutaneous type, with a corresponding increase in anti-desmoglein (Dsg)1 and decrease in anti-Dsg3 antibody titres. Thus, the clinical features seemed to correlate with the ratio of anti-Dsg1 and 3. The patient also had anti-Dsg4 antibodies, which might be related to the nonscarring diffuse hair loss and marked facial involvement she also had. The patient did not respond to treatment with systemic steroid, ciclosporin, azathioprine, cyclophosphamide or double filtration plasmapheresis, and eventually died from fulminant thrombotic thrombocytopenic purpura of unknown cause.

Clinical manifestations of alopecia in autoimmune blistering diseases: A cross-sectional study

“… 2 , 13 , 14 , 15 This may be because of higher levels of pemphigus antigens such as Dsg1 in the scalp. 9 , 16 , 17 , 18 , 19 Interestingly, in our study, Dsg1 ratios ( P < .001) were an independent predictor of hair loss in patients with PV. The role of Dsg1 antibodies in nonscarring alopecia is alluded to in the literature.…”

“…Similarly, in an earlier study, an initial high anti-Dsg3 to anti-Dsg1 ratio was observed in a patient with PV, which was associated with diffuse nonscarring alopecia as this ratio decreased. 19 …”