Dermatitis herpetiformis in China: a report of 22 cases.
ABSTRACT Although dermatitis herpetiformis (DH) is a relatively common disease in Caucasian populations, it is very rare in the Far East, including China.
To investigate the clinical, histological and immunopathological characteristics of Chinese patients with DH.
The clinical data and serum samples of patients with DH diagnosed at Shandong Provincial Institute of Dermatology and Venereology from 2006 to 2010 were collected. Anti-endomysium antibodies (EMA) and anti-tissue transglutaminase (tTG) antibodies were tested using indirect immunofluorescence and enzyme-linked immunosorbent assay respectively. Biopsies of small intestine were performed in those patients who had given informed consent.
The study group consisted of 22 patients with DH. The male to female ratio was 2.1:1; the average age was 44 years. Five patients reported mild gastrointestinal symptoms. Dapsone was used to treat 14 patients, who achieved rapid relief. Direct immunofluorescence showed granular IgA deposits at the papillary tips and/or basement membrane zone in 95.5% (21/22) patients. Fibrillar IgA deposition limited to the papillary tips was also seen in one patient (4.5%). IgA antibodies against EMA and tTG were found in 50% (8/16) and 62.5% (10/16) of patient sera respectively. Small-intestinal biopsies of two patients confirmed the presence of coeliac disease.
The clinical, histological and immunopathological characteristics of Chinese patients with DH are similar to those seen in Caucasian populations. The positive rates of anti-EMA and anti-tTG antibodies were lower compared with those seen in Western countries. DH is not extremely rare in China and should be actively investigated.
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ABSTRACT: Dermatitis herpetiformis is an autoimmune bullous disease that is associated with gluten sensitivity which typically presents as celiac disease. As both conditions are multifactorial disorders, it is not clear how specific pathogenetic mechanisms may lead to the dysregulation of immune responses in the skin and small bowel, respectively. Recent studies have demonstrated that IgA and antibodies against epidermal transglutaminase 3 play an important role in the pathogenesis of dermatitis herpetiformis. Here, we review recent immunopathological progress in understanding the pathogenesis of dermatitis herpetiformis.Clinical and Developmental Immunology 01/2012; 2012:914162. · 1.84 Impact Factor
Article: DERMATITIS HERPETIFORMIS BODIES AND AUTOANTIBODIES TO NONCUTANEOUS ORGANS AND MITOCHONDRIA IN DERMATITIS HERPETIFORMIS[show abstract] [hide abstract]
ABSTRACT: Introduction: The precise nature of the previously described dermatitis herpetiformis bodies remains unknown. Aims: Our study was conducted to investigate the nature of dermatitis herpetiformis bodies in the skin in 7 cases of dermatitis herpetiformis, and to search for the presence of autoantibodies in other organs. Methods: We utilized clinical, histopathologic, and immunologic methods to evaluate these patients. Results: Dermatitis herpetiformis bodies were found to be comprised of an amalgamation of immunoglobulins A and M, as well as molecules reactive with antibodies to armadillo repeat gene deleted in velo-cardio-facial syndrome, desmoplakins 1 and 2, and plakophilin 4. In addition, we found immunologic colocalization with selected autoantibodies associated with mitochondria in the skin, heart, kidney, and peripheral nerves. The dermatitis herpetiformis bodies did not demonstrate immunologic colocalization with tissue/epidermal transglutaminase. Conclusions: The complete biochemical nature of dermatitis herpetiformis bodies requires further characterization. Dermatitis herpetiformis bodies in these patients appear to be distinctly different than cytoid bodies. Further studies are required to determine if the antibodies to noncutaneous organs are pathogenic, and/or contribute to systemic morbility in dermatitis herpetiformis patients. Introduction Dermatitis herpetiformis (DH), previously known as hydroa or Duhring-Brock disease is an autoimmune, subepidermal blistering disease characterized by IgA deposits in the dermal papillae [1-2]. DH predilects females and Caucasians, and is a rare disease. The eye and oral cavity are very seldom affected. Immunoglobulin, complement and epidermal transglutaminase (eTG) deposition has been previously detected in the cutaneous blood vessels in dermatitis herpetiformis . A gluten dependent enteropathy may accompany DH.: Dermatitis herpetiformis bodies and autoantibodies to noncutaneous organs and mitochondria in dermatitis herpetiformis. Our Dermatol Online. 2012; 3(4): 283-291Our Dermatology Online. 10/2012; 3(4):283-291.