|Year : 2014 | Volume
| Issue : 1 | Page : 51-53
Emy Abi Thomas
Department of Dermatology, Christian Medical College, Ludhiana, Punjab, India
|Date of Web Publication||11-Feb-2014|
Emy Abi Thomas
Department of Dermatology, Christian Medical College, Ludhiana 141 008, Punjab
Source of Support: None, Conflict of Interest: None
Dermatitis herpetiformis (DH) is an uncommon condition. We describe a young boy who presented with pruritic, recurrent grouped fluid filled and papular lesions over the body. DH was confirmed by histopathological examination of the vesicle.
Keywords: Gluten sensitive enteropathy, severe pruritus, vesiculo-bullous disease
|How to cite this article:|
Thomas EA. Dermatitis herpetiformis. CHRISMED J Health Res 2014;1:51-3
| Case report|| |
A 13-year-old boy presented with history of severely pruritic, recurrent, grouped fluid filled, and papular lesions on the elbows, knees, and gluteal area of 1 year and 6 months duration. Patient did not give any history of abdominal pain, diarrhea, or any symptoms suggestive of malabsorption. There was no family history of similar lesions.
Physical examination revealed multiple excoriation marks, grouped papules, and vesicles and scaling on the extensor aspect of both elbows, knees, shoulder, lower back, and gluteal area [Figure 1] and [Figure 2]. Histopathological examination of vesicle revealed clefts at the dermoepidermal junction with neutrophilic microabscesses within the dermal papillae, which confirmed the diagnosis of dermatitis herpetiformis (DH) [Figure 3] and [Figure 4].
| Discussion|| |
Synonym: Duhring's disease
Dr. Louis Duhring described DH at the University of Pennsylvania in 1884.
DH, a pruritic bullous skin disease that predominantly affects the extensor surfaces of the elbows, knees, gluteal area, back, and scalp, is part of a spectrum of gluten sensitive disorders that includes celiac disease (CD) and possibly some forms of immunoglobulin A (IgA) nephropathy and gluten sensitive ataxia. Recent evidence had confirmed that DH is a direct consequence of an intestinally directed intolerance to gluten. 
Although over 90% of DH patients have evidence of a gluten sensitive enteropathy, only about 20% have intestinal symptoms of CD.
Human leukocyte antigen studies shows a very strong association with DR 3 and DQW2. , There is a difference in autoantibody profile between DH and coeliac disease; in that IgA antibodies to epidermal transglutaminases (tissue transglutaminase 3) are more common in DH than coeliac disease.  The IgA precipitates in the dermis contain epidermal transglutaminases  and there is colocalization of the granular IgA deposits and the epidermal transglutaminases in the upper dermis and blood vessels, thus DH may be an immune complex disease. 
DH affects men slightly more frequently than women. In a majority of cases, its onset is between the 2 nd and 4 th decades of life. However, it can appear in later years and even in childhood. The primary lesions consist of erythematous papules or vesicles. Grouping of these vesicles in a herpetiform configuration may sometimes be observed. However, the patient often presents with only multiple erosions secondary to scratching, because the eruption is characteristically intensely pruritic. Hence, the hallmarks of the disease are the grouping of these lesions, their symmetry and their distribution. Oral mucous membrane involvement in DH is rare.
Dapsone is currently the most widely used medication for DH, presumably because of its effect on the neutrophils and its dramatic effect on the rash. The rash usually begins to clear within 2 days with complete clearance usually within 1 week. However, relapse with discontinuation of dapsone is usually rapid. A dose of 100 mg daily of dapsone usually controls the rash. The use of sulfamethoxypyridazine has been suggested for the treatment of DH in those older than 50 years. The dose varies from 0.5 g twice weekly to as much as 4 g/day.
The underlying cause of DH relates to the oral intake of the particular proteins derived from wheat, barley, rye, and possibly oats. If gluten-free diet alone is used, this usually results in a slow clearing of skin lesions over many months and a reduction of relapse episodes. The diet, although somewhat restrictive, is readily achievable with appropriate education and motivation of the patient.
Although DH is usually a lifelong condition, the course may wax and wane. A spontaneous remission may occur in up to 10% of patients but most clinical remissions are related to dietary gluten restriction. 
Wheat (wheat flour, white flour, and wheat bran) wheat starch, semolina, barley, rye, oats (oat flour, oat bran, and oat meal) amaranth and millet are some of the foods that may contain gluten and should be avoided.
| References|| |
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|3.||Sachs JA, Leonard J, Awad J, McCloskey D, Festenstein H, Hitman GA, et al. A comparative serological and molecular study of linear IgA disease and dermatitis herpetiformis. Br J Dermatol 1988;118:759-64. |
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]