Clinical and immunopathological features of 159 patients with dermatitis herpetiformis: an Italian experience.

AIM: The aim of this paper to report the main clinical and immunopathological findings of our case series of 159 patients with dermatitis herpetiformis (DH).

METHODS: All DH patients that were diagnosed from 1995 to 2012 at the Section of Dermatology of the University of Florence were included in the study. Clinical data were collected for each patient. Moreover, histopathological examination on both the skin and the small bowel, direct immunofluorescence on perilesional skin as well as the search for anti-endomysium and anti-tissue transglutaminsase antibodies (tTG) were performed.

RESULTS: A total of 159 patients with a male predominance were enrolled. About 36% of the patients were below the age of 20. The most frequent clinical features seen in our DH patients were represented by figurate erythema, wheals, papules and scratching lesions, while the knees, elbows and buttocks were the most commonly involved sites. All the 22 patients that underwent a bowel biopsy showed the typical alterations found in celiac disease. Moreover, 100% of the patients showed granular IgA deposits at the papillary tips. Finally, anti-endomysium and anti-tTG antibodies were present in 90% and 96% of the patients, respectively.

CONCLUSION: We reported one of the largest case series of patients with DH from a single center. Our study confirmed most of the data from the Literature, and in particular the association of DH to histologically proven CD in all the biopsied cases. Another interesting finding of our study is the high prevalence of DH within pediatric patients, that is usually underreported.