Background: Although dermatitis herpetiformis (DH) is a relatively com
mon disease in Caucasian populations, this disease is very rare in Asi
an populations including the Japanese. Methods and Results: We present
a Japanese DH patient, who showed a fibrillar pattern of deposition o
f IgA, IgG, IgM and C3, The HLA typing revealed no B8/DR3. The survey
of the Japanese literature and the comparison to studies on American o
r European DH revealed several interesting differences: high frequency
of the fibrillar pattern, relatively high incidence of deposits of im
munoglobulins other than IgA, rarity of gluten-sensitive enteropathy a
nd HLA-B8/DR3 in Japanese DH. Conclusion: The present study suggests t
hat there may be a significant difference in pathophysiology between C
aucasian and Japanese DH patients.