Cases reported "Dermatitis Herpetiformis"

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1/125. Childhood dermatitis herpetiformis: an unusual presentation.

    Childhood dermatitis herpetiformis (DH) is rare. The true prevalence and incidence of this condition are unknown. We report a 7-year-old boy presenting with nonpruritic inflammatory papules on the buttocks and extensor surfaces, clinico-pathologically consistent with Sweet's neutrophilic dermatosis. Immunofluorescence studies showed IgA deposits in the dermal papillae consistent with DH. Remission was achieved with a gluten-free diet and dapsone. Childhood DH may present different clinical signs to the adult form and misdiagnosis can occur if immunofluorescence is not requested on skin biopsy.
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2/125. Oral acantholytic itching disease responding to dapsone. dermatitis herpetiformis, pemphigus, or a new disease?

    A patient had a blistering and severely itching disease confined solely to the oral cavity. The histopathologic findings had the features of both pemphigus and dermatitis herpetiformis. There were no gastrointestinal symptoms, no IgA could be found in uninvolved skin, and no intercellular or basement membrane antibodies were present in the serum or epidermis. The disease responded favorable to dapsone (diaminodiphenylsulfone), which could be discontinued after 2 1/2 years. The literature is briefly reviewed, and there is a discussion of whether the patient was suffering from pemphigus or dermatitis herpetiformis. The conclusion is drawn that the diagnosis cannot be established with certainty and that we are perhaps dealing with a new disease. It is stressed that in such aberrant cases a therapeutic trial with dapsone should be made.
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3/125. dermatitis herpetiformis effectively treated with heparin, tetracycline and nicotinamide.

    We report a patient with severe dermatitis herpetiformis (DH) who was intolerant of dapsone, sulphapyridine, systemic steroids, and azathioprine. He was treated effectively with a combination of heparin, tetracycline and nicotinamide.
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4/125. A case of dermatitis herpetiformis with IgA endomysial antibodies but negative direct immunofluorescent findings.

    A patient with clinical findings of dermatitis herpetiformis (DH), negative direct immunofluorescent (DIF) findings for junctional IgA deposits in 2 biopsy specimens, and positive for IgA endomysial (AEmA) and tissue transglutaminase (tTG) antibodies responded initially to dapsone. After dapsone had to be discontinued because of side effects, a gluten-free diet and supportive therapy controlled the disease; the AEmA and tTG antibodies became negative. Our data on 10 consecutive DH cases examined by DIF and by serum studies for AEmA and antibodies to tTG, point to frequencies of 90% DIF positive and 70% AEmA and tTG positive cases. The use of both DIF and serum tests for AEmA and tTG reveals DH cases not detected by DIF alone that respond to gluten-free diet. Findings on autoantibodies to tTG, an enzyme that metabolizes gliadin, points to a role of tTG in the immunopathology of gluten-sensitive enteropathy and helps to explain the need for a gluten-free diet in the management of DH cases.
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5/125. dermatitis herpetiformis and vitiligo: report of a case and review of the literature.

    We describe the case of a 53-year-old woman presenting papulous and papulovesicular lesions that were highly pruritic, localized mostly in the achromic areas of vitiligo and symmetrically distributed on the elbows, the buttocks, the shoulders and the neck. The histopathological examination performed on the elbow's lesional skin showed the presence of neutrophils and fibrin microabscesses at the tips of dermal papillae, with a few eosinophils, and small separations between the dermis and epidermis just over the infiltrate. The overlying epidermis was uninjured. The performed tests detected IgA anti-endomysium, anti-thyrogloblin, anti-smooth muscle and anti-microsomal fraction autoantibodies; DIF showed the presence of IgA granular deposits at the dermo-epidermal junction, prevalently at the tips of dermal papillae. This is the tenth case reported of an association between dermatitis herpetiformis and vitiligo. Although the two disorders both have immunological pathogeneses, we think that the topographic coexistence of both disorders is coincidental.
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6/125. T cell lymphoma of the thyroid gland in celiac disease.

    Previous studies have reported the association between celiac disease and T cell lymphoma of the intestine as well as hepatosplenic lymphoma, a specialized peripheral type of T cell lymphoma. In this report, a 66-year-old woman with dermatitis herpetiformis and biopsy-defined celiac disease developed a thyroid mass that proved to be a T cell lymphoma. A T cell lymphoma in the setting of celiac disease appears to be unique. The thyroid gland, due to its shared embryological developmental links with the gastrointestinal tract, is possibly another site of extranodal lymphoma linked to celiac disease.
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7/125. dermatitis herpetiformis in a Japanese patient with anaplastic large cell lymphoma.

    We report a 73-year-old Japanese man with dermatitis herpetiformis which developed after diagnosis of anaplastic large cell lymphoma. The patient suffered fever, sweating, shivering, and multiple enlarged cervical lymph nodes. The diagnosis of anaplastic large cell lymphoma was confirmed by the histologic features of a biopsied cervical lymph node. The patient underwent combination chemotherapy. However, one month after the initial therapy, pruritic erythematous skin lesions with peripheral vesicles appeared on his buttocks. A skin biopsy showed subepidermal blister formation associated with polymorphonuclear and mononuclear cell infiltrates. Direct immunofluorescence examination of the area adjacent to the lesion showed granular deposits of IgA at the dermoepidermal junction. While it is well-known that dermatitis herpetiformis can develop into lymphoma, there have been only a few reports of its appearance after a diagnosis of lymphoma. This case suggests that dermatitis herpetiformis may be induced by anaplastic large cell lymphoma.
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8/125. dermatitis herpetiformis evolving into bullous pemphigoid: a probable example of 'epitope spreading'.

    We report a case of dermatitis herpetiformis which, 11 years after its original diagnosis, evolved into bullous pemphigoid. Only a few similar cases supported by immunofluorescence studies have been reported previously, and we believe that they represent examples of 'epitope spreading', an increasingly recognized phenomenon used to explain the coexistence of autoimmune diseases.
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9/125. dermatitis herpetiformis in japan: an update.

    BACKGROUND: Although dermatitis herpetiformis (DH) is a relatively common disease in Caucasian populations, it is rare in Asian populations including the Japanese. We encountered a Japanese case of DH which showed granular IgA and C3 deposits in the papillary dermis and which was associated with gluten-sensitive enteropathy but no HLA-B8/DR3/DQ2. OBJECTIVE: The purpose of this study is to describe the characteristics of Japanese DH cases, since most of them have been reported in Japanese language and dermatologists outside japan are not familiar with the characteristics of Japanese DH. methods: We have reviewed all 34 Japanese DH cases reported previously. RESULTS: We found several features of Japanese DH compared with Caucasian DH, such as a high frequency of the fibrillar pattern, rarity of gluten-sensitive enteropathy and an absence of the HLA-B8/DR3/DQ2 haplotype. CONCLUSION: There might be significant differences in pathophysiology between Caucasian and Japanese DH cases.
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10/125. dermatitis herpetiformis: should direct immunofluorescence be the only diagnostic criterion?

    We describe a 7-year-old boy with dermatitis herpetiformis (DH) diagnosed on clinical and histologic evidence, negative direct immunofluorescence (DIF) findings for junctional IgA deposits in uninvolved skin, positive IgA endomysial and gliadin antibodies, and jejunal biopsy revealing a gluten-sensitive enteropathy. Treatment with dapsone led to the disappearance of cutaneous lesions and pruritus within 48 hours. Demonstration of IgA immune deposits in the dermal papillae has been the only acceptable criterion for the diagnosis of dermatitis herpetiformis. However, considering several reports in the literature of DH with a negative DIF and our own case, we believe that in the absence of the characteristic DIF pattern, one needs the combination of clinical, histologic, and immunologic data to support the diagnosis of DH. We also discuss recent developments in the diagnosis of DH.
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