Herpetiform pemphigus without anti-desmoglein 1/3 autoantibodies
Article first published online: 26 FEB 2010
© 2010 Japanese Dermatological Association
The Journal of Dermatology
Special Issue: Blistering Diseases (pages 193-239)
Volume 37, Issue 3, pages 264–268, March 2010
How to Cite
NAKASHIMA, H., FUJIMOTO, M., WATANABE, R., ISHIURA, N., YAMAMOTO, A. I., HASHIMOTO, T. and TAMAKI, K. (2010), Herpetiform pemphigus without anti-desmoglein 1/3 autoantibodies. The Journal of Dermatology, 37: 264–268. doi: 10.1111/j.1346-8138.2009.00786.x
- Issue published online: 26 FEB 2010
- Article first published online: 26 FEB 2010
- Received 12 May 2009; accepted 6 December 2009.
- bullous diseases;
We report a patient with herpetiform pemphigus who was negative for autoantibodies to desmoglein (Dsg)1 or 3. He had erythemas with vesicles lining the margins on the trunk and extremities. Histopathology revealed intraepidermal blister with prominent eosinophil infiltration. Direct and indirect immunofluorescence demonstrated the presence of depositing and circulating immunoglobulin (Ig)G autoantibodies, but no IgA antibodies, to keratinocyte cell surface. Nonetheless, neither anti-Dsg1 nor Dsg3 antibodies were detected by enzyme-linked immunosorbent assay. Immunoblotting using human epidermal extracts also showed no reactivity with known intraepidermal or epidermal–dermal junctional substances. Immunoelectronmicroscopy revealed the reactivity on the portion of keratinocyte cell surface but not on the desmosomes. This case suggests that non-desmoglein antigen on keratinocyte cell surface can be targeted in some patients with this unusual variant of pemphigus.