Dermatitis herpetiformis

Dermatitis herpetiformis (DH) is a chronic, polymorphic, pruritic skin disease that develops mostly in patients with latent gluten-sensitive enteropathy. DH patients usually present with skin manifestations only and are not aware of the underlying small-bowel problems. Owing to the granular immunoglobulin (Ig) A deposition at the tips of the papillary dermis and to the subepidermal blister formation associated with neutrophilic accumulations underlying the basement membrane, DH is considered to be an autoimmune blistering disease. Contrary to the other bullous disorders, DH patients have no circulating autoantibodies binding to the cutaneous basement membrane components or to other adherent structures of the skin, but they have gluten-induced IgA autoantibodies against transglutaminase (TG) 2 and TG3. The serum IgA against tissue TG2 is a most specific and sensitive serologic marker of gluten-sensitive enteropathy and is equivalent to the perviously described IgA endomysium antibodies. DH could be a cutaneous IgA-epidermal TG3 immunocomplex disease, developing only in a few patients with gluten-sensitive enteropathy as a second gluten-dependent disease. The main treatment of DH today is a strict, life-long gluten-free diet. Untreated DH patients should be regularly monitored for malabsorption and lymphomas. Associated autoimmune diseases are more common among DH patients. Family screening for gluten sensitivity is also strongly suggested.     

Dermatitis herpetiformis

The state of our understanding of the pathogenesis of DH relies on the integration of several key characteristics: (1) a high frequency of the HLA antigens HLA-B8, HLA-DR3, and HLA-DQw2, (2) an associated GSE, (3) the resolution of both the skin lesions and gut abnormalities in response to a gluten-free diet, and (4) the presence of granular deposits of IgA in normal and perilesional skin. The role of the HLA class II antigens expressed in patients with DH most likely relates to the afferent or initiating arm of the immune system. The association of the HLA-A1, -B8, -DR3, -DQw2 haplotype with Sjogren's syndrome, chronic hepatitis, Graves' disease, and other presumably immunologically mediated diseases, as well as the evidence that some normal HLA-B8, -DR3 individuals have an abnormal in vitro lymphocyte response to wheat protein and mitogens and have abnormal Fc-IgG receptor-mediated functions, suggests that this HLA haplotype or genes linked closely to it may confer a generalized state of immune susceptibility on its carrier, the exact phenotypic expression of which depends on other genetic or environmental determinants. It also is clear, from the association of DH with GSE and the ability to control the cutaneous manifestations of DH with a gluten-free diet, that the gut disease is a critical factor in the pathogenesis of DH. Several pathogenetic theories about the origin of the cutaneous IgA deposits in DH have been proposed, one of which states that the IgA is produced in the gut mucosa as a response to a dietary antigen or gut epithelial antigen and then cross-reacts with the skin of patients with DH. A second hypothesis is that the IgA produced in the gut binds to an antigen and is deposited in skin as an antigen-antibody complex. Finally, it could be that the gut mucosal abnormality simply allows an unknown antigen access to the central immune system where an IgA antibody is produced that binds to skin. The failure to detect circulating IgA anti-basement membrane zone antibodies in patients with DH suggests that either the structures to which the IgA binds are not present in normal skin without DH, that IgA cannot bind to these structures in vitro, or that the circulating IgA is too scant for detection with conventional methods. Finally, it must be considered that the IgA deposited in DH skin may bind as a result of non-antigen-antibody interactions that cannot be duplicated in vitro.(ABSTRACT TRUNCATED AT 400 WORDS)     

Dermatitis herpetiformis

A 68-year-old man with a 2-month itching, symmetrical, polymorphous, bullous eruption showing histological and immuno-histological changes compatible with dermatitis herpetiformis is described. The eruption could be provoked by iodine and subsided on an iodine-free diet.     

疱疹样皮炎的研究进展

疱疹样皮炎是一种与乳糜泻相关的自身免疫性大疱病,多发于携带HLA-DQ2或HLA-DQ8等位基因的高加索人.研究表明,疱疹性皮炎患者血清中存在多种针对转谷氨酰胺酶等抗原的抗体,表皮型转谷氨酰胺酶是其自身抗原.疱疹样皮炎的发病除与遗传和麦胶敏感有关外,还可能与肠道菌群微生态失衡有关.无麦胶饮食和氨苯砜仍然是疱疹样皮炎的主...     

Dermatitis herpetiformis

Dermatitis herpetiformis is a chronic, lifelong disease characterized both by symptomatic, characteristic skin lesions and by a gluten-sensitive, often asymptomatic, enteropathy. Cutaneous findings include intensely pruritic vesicles on symmetrical extensor locations. Treatment for disease may be pharmacologic agents or a gluten-free diet. Evidence for the genetic basis of disease has been elucidated.     

Dermatitis herpetiformis

The multifactorial pathogenesis of dermatitis herpetiformis is reviewed in light of current experimental data. Genetic background, gluten consumption, and abnormal immune and autoimmune reactions are the most important pathogenetic factors, but other agents also participate in the disease development. The predisposing and inducing factors are summarized, while the pathophysiological steps leading to the development of skin symptoms are detailed.     

Dermatitis herpetiformis Duhring

Celiac disease is a genetically determined bowel disease also influenced by exogenous factors in which exposure to grain components triggers a chronic immune response with intestinal symptoms. Dermatitis herpetiformis represents the cutaneous manifestation of celiac disease. While intense pruritus is the characteristic symptom, clinical signs can be highly variable, ranging from grouped papulovesicles with excoriations or eczema-like lesions to minimal variants of discrete erythema and digital purpura. Diagnosis depends on direct fluorescence studies of perilesional skin displaying granular IgA deposits in dermal papillae. Suspecting and then searching for dermatitis herpetiformis is often clinically challenging, as the disease is a true chameleon with many clinical faces. Dapsone therapy alleviates the cutaneous symptoms and signs, but does not prevent the systemic complications of celiac disease; thus, strict adherence to a gluten-free diet is strongly advisable.     

Dermatitis herpetiformis Duhring

Zusammenfassung Bei einer 36jährigen Frau tritt nach leichten Prodromalerscheinungen eine Dermatose auf, die einen bullös-erythematösen Charakter zeigt. Die ursprüngliche Diagnose: Erythema exsud. mult. bullosum muß im weiteren Verlauf fallen gelassen werden. Die Feststellung einer Gravidität läßt eine Beziehung zwischen dieser und der Dermatose wahrscheinlich erscheinen. Es wird über eine Reihe von Untersuchungen berichtet, auf Grund deren wir zu der Auffassung kommen, daß eine Störung innersekretorischer Organe als ätiologischer Faktor für die zur Gruppe der Dermatitis herpetiformis gehörenden Dermatosen in Betracht kommt. Der experimentelle Beweis konnte nicht erbracht werden.     

Dermatitis herpetiformis and vitiligo

We report the fourth case of coexisting dermatitis herpetiformis and vitiligo. This patient's HLA haplotype is HLA-B8 DR3, which is characteristic of dermatitis herpetiformis. The autoimmune features of both disorders suggest that their coexistence is not coincidental.     

Dermatitis herpetiformis Duhring necroticans

Ohne Zusammenfassung     

Dermatitis herpetiformis and thyrotoxicosis

Dermatitis herpetiformis has been associated with a variety of thyroid abnormalties. A case of thyrotoxicosis in a patient with pre-existing dermatitis herpetiformis is reported. Thyroid antibodies were present in the serum. This may suggest an immunologic relationship between dermatitis herpetiformis and thyroid disorders, that may be more than fortuitous.     

Jodempfindlichkeit bei Dermatitis herpetiformis

Ohne ZusammenfassungD. hd