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PubMed1996

Incidence of familial dermatitis herpetiformis.

Dermatitis herpetiformis (DH) and coeliac disease (CD) are gluten-sensitive diseases which have a common immunogenetic background, with the histocompatibility locus antigen (HLA) alleles DQ A1*0501 and B1*0201 in the short arm of chromosome 6. CD is well known to cluster in families whereas DH has not been generally regarded as a familial disease. To study the familial incidence of DH, a prospective study was started in 1969 at the Department of Dermatology of Helsinki University Hospital and in 1976 at the Department of Dermatology of Tampere University Hospital. A total of 1018 patients with DH were diagnosed and questioned for positive family histories. Of the 999 unrelated DH patients, 105 (10.5%) had one or several affected first-degree relatives. The disease in the relatives was either DH (4.4%) or CD (6.1%). Analysis of the 105 families showed that 13.6% of parents, 18.7% of siblings and 14.0% of children were affected, a segregation pattern which fits well to a dominant mode of Mendelian inheritance. Gender may also be important because the first-degree relatives affected with DH were more often females and those affected with CD twice as often females as males. In contrast, there were more males among the 105 propositi with DH and also among the 894 DH patients with no affected relatives. The present study clearly shows that DH is a familial disease in which the first-degree relatives can be affected both with DH and CD, presumably because of a common genetic background. The environmental factors which could cause the rather high penetrance of DH and CD in the first-degree relatives of DH patients remain unknown.

Adolescent↗
PubMed1995

Absence of gluten-specific T lymphocytes in the skin of patients with dermatitis herpetiformis.

Dermatitis Herpetiformis (DH) is an immunobullous skin disease with an associated gluten-sensitive enteropathy. Withdrawal of gluten from the diet leads to the resolution of both the skin lesions and the enteropathy. A T cell-mediated immune response to gluten has been implicated in the damage to the gut; the possible gluten specificity of the T cell infiltrate in DH skin lesions has not, however, been investigated. T cell lines (TCL) were therefore established from the skin lesions of eight patients with DH by culturing skin fragments for 11-17 days with a medium supplemented with 20 U/ml of IL-2. In three cases, gliadin (fraction of gluten toxic to the DH gut) and irradiated, autologous peripheral blood mononuclear cells were also added. The TCL were stained for CD3, CD4, CD8, TCR alpha beta and gamma delta expression by indirect immunofluorescence, and their proliferative responses to mitogens and gluten fraction III (a peptic-tryptic digest of gluten) investigated. Of the eight CD3+ TCL, four were predominantly CD4+ (82.1-98.8%), three predominantly CD8+ (92.6-98.6%) and one TCL contained both 87.6% CD4+ and 95.2% CD8+ T cells, a substantial proportion of which were presumably double-labelled CD4+, CD8+ T cells. All eight TCL, which were almost exclusively TCR alpha beta +, proliferated in response to PHA whilst six out of the eight were stimulated by Concanavalin A. None of the TCL proliferated to gluten fraction III alone; however, two TCL showed increased proliferation to the antigen in the presence of exogenous IL-2 or IL-4 (10 U/ml) compared to cytokine alone.(ABSTRACT TRUNCATED AT 250 WORDS)

CD3 Complex↗
PubMed1987

Dietary management of dermatitis herpetiformis.

Dermatitis herpetiformis (DH) is an extremely itchy, papulovesicular skin disease characterized in part by the presence of IgA at the dermal-epidermal junction. Eighty-five percent to 90% of DH patients have granular deposits of IgA at the dermal-epidermal junction, and essentially all of these patients have an associated, for the most part asymptomatic, gluten-sensitive enteropathy (GSE). The association of GSE and DH suggested that the cutaneous manifestations of DH could be controlled by the use of a gluten-free diet. Institution of a gluten-free diet in patients with DH and granular IgA deposits has been shown to be effective in controlling the cutaneous eruption of DH. Seventy percent to 100% of patients who begin a strict gluten-free diet have been shown to be able to decrease the dosage of medication needed to control their DH after a mean of eight to 18 months on the diet. Furthermore, 40% to 70% of patients with DH can control their skin disease completely, without any medication, after longer periods of time on the gluten-free diet (two years and longer). Although the gluten-free diet has been shown to be of great benefit in the control of the skin manifestations of DH, at the present time there is no evidence to suggest that the gluten-free diet is in any way protective against the risk of intestinal lymphoma that has been documented in GSE. Evaluation of the cutaneous IgA deposits in DH skin after long periods of time on a gluten-free diet suggests that there may be a slight decrease in the intensity of the IgA deposits, but the true pathogenetic relationship between the cutaneous IgA deposits, the cutaneous manifestations of DH, and the associated GSE remains unknown.

Celiac Disease↗
PubMed1995

Dermatitis herpetiformis.

Dermatitis herpetiformis (DH) is a relatively rare skin disorder with an estimated incidence of 1:10,000 in the UK. It is characterized by urticarial plaques and blisters on the elbows, buttocks, and knees, although other sites may also be involved. The eruption tends to be persistent: only 10-15% of patients have spontaneous remission over a 25-year study period. The disease is characterized by the presence of IgA deposits in the upper dermis of uninvolved skin and the diagnosis should not be made in the absence of these deposits. Two-thirds of patients have a small intestinal enteropathy with villous atrophy as seen in coeliac disease (CD). However, the remaining third also show evidence of a gluten sensitivity in the intestine, as judged by increased lymphocytic infiltration of the epithelium. Villous atrophy also ensues after gluten challenge in those patients with previous normal villous architecture. The initial treatment of the rash is with one of the following three drugs, dapsone, sulphapyridine or sulphamethoxypyridazine. However, the rash also clears with gluten withdrawal. It must be stressed, however, that the average time to achieve significant reduction in drug requirements is 6 months and it can be over 2 years before drugs are no longer required. On re-introduction of gluten the eruption recurs. Patients with DH have a high incidence of auto-immune disorders, thyroid disease, pernicious anaemia, and insulin-dependent diabetes, and should be screened for those diseases on a yearly basis. As with coeliac disease there is also an increased incidence of lymphoma and a gluten-free diet appears to protect patients from this complication. The mechanism by which gluten causes the skin lesions has still to be elucidated, but current investigations implicate lymphocytes and cytokines in the pathogenesis. The original hypothesis of an antigen-antibody reaction in the skin with complement activation causing the skin lesions, may not be correct.

Adolescent↗
PubMed1989

Alterations in HLA-DP and HLA-DQ antigen frequency in patients with dermatitis herpetiformis.

Dermatitis herpetiformis (DH) is associated with a markedly increased frequency of the HLA class II antigens DR3 and DQw2. To investigate a possible role of HLA-DP (or closely associated genes) in the pathogenesis of DH as well as to confirm the previously described alterations of HLA-DR3 and HLA-DQw2 antigen frequency, we have typed 43 patients with DH for HLA-DP, HLA-DQ, and HLA-DR antigens. All patients with DH had typical clinical and histologic features, as well as granular deposits of IgA at the dermal-epidermal junction by direct immunofluorescence. HLA-DR3 was expressed in 41 of 43 (95%) DH patients, whereas HLA-DQw2 was expressed in all 43 (100%). The overall distribution of HLA-DP antigens in patients with DH was significantly different from that seen in all controls and in HLA-DR3 and HLA-DQw2 controls (p less than 0.02). Examination of the frequency of individual DP antigens revealed that HLA-DPw1 was increased (42% of patients with DH vs 11% of all controls and 26% of DR3 positive controls), but this increase was not statistically greater than that expected due to the disequilibrium linkage of DPw1 with DR3/DQw2. Patients with DH, however, did have a statistically significant decreased frequency of DPw2 (14% of patients vs 31% of all controls and 41% of DR3 positive controls) (pc less than 0.05). Studies of three informative families demonstrated that the DPw2 genes of the DH patients were not present on the haplotype thought to carry a DH susceptibility gene (HLA-A1, HLA-B8, HLA-DR3, HLA-DQw2). A role of HLA-DP region genes in the pathogenesis of DH is further suggested by the observation that HLA-DPw1 was expressed in 82% (9 of 11) of DH patients with IgA antibodies against dietary antigens as compared with only 33% (4 of 12) of patients without IgA antibodies. HLA-DP genes or genes closely linked to them may be important in DH either as markers of the disease haplotype or by direct involvement in its pathogenesis.

Antigens↗
PubMed1992

Comparison of the intestinal and serum antibody response in patients with dermatitis herpetiformis.

Dermatitis herpetiformis (DH) is an intensely pruritic, blistering skin disease characterized by cutaneous IgA deposits and an associated, most often asymptomatic, gluten-sensitive enteropathy. When patients with DH are placed on a gluten-free diet both the intestinal abnormality and the cutaneous manifestations of the disease are controlled, suggesting that a mucosal immune response is important in the pathogenesis of DH. Although patients with DH continue to ingest gluten only 40-50% have evidence of an ongoing mucosal immune response in their serum. In order to investigate directly the mucosal immune response in patients with DH the antibody response to dietary antigens was analyzed in intestinal secretions and compared to that found in the serum. Intestinal secretions from six patients with DH and five normal subjects were collected using an intestinal lavage solution and analyzed for total IgA, IgG, IgM, and IgA subclasses, and for IgG, IgA, and IgM antibodies against the dietary antigens bovine beta-lactoglobulin and gliadin. Intestinal secretions from patients with DH contained more IgA than those from normal subjects (mean total IgA: DH = 2.3 mg/ml; normal subjects (NL) = 0.143 mg/ml, P = 0.017). This increase in IgA in intestinal secretions from patients with DH was composed primarily of IgA1 (intestinal IgA: 86% IgA1, 14% IgA2; NL gut secretions: IgA1 = 54%; IgA2 = 46%). Increased IgA antibodies directed against beta-lactoglobulin and gliadin were detected in gut secretions of two of six patients with DH and in none of the normal subjects. Serum IgA antibodies against beta-lactoglobulin and gliadin were detected only in the two subjects who had detectable IgA antibodies in their intestinal secretions. Serum and intestinal IgA anti-beta-lactoglobulin antibodies had similar isoelectric spectrotypes (pI 5.0-6.5), IgA subclass composition, and antigenic reactivity by immunoblot analysis, demonstrating the close relationship between the serum and intestinal IgA antibodies. These data demonstrate that in patients with DH an ongoing mucosal immune response is present in the gut as evidenced by a significantly increased concentration of IgA, predominately IgA1. The strong correlation between detectable serum and intestinal IgA antibodies against dietary antigens demonstrates that the lack of serum IgA antibodies against dietary antigens in some patients with DH is not due to the presence of "blocking" IgA anti-dietary antigen antibodies in intestinal secretions.(ABSTRACT TRUNCATED AT 400 WORDS)

Adult↗
PubMed1985

Characterization of circulating and cutaneous IgA immune complexes in patients with dermatitis herpetiformis.

Dermatitis herpetiformis (DH) is a chronic, blistering skin disease characterized in part by deposits of IgA at the dermal-epidermal junction. Eighty-five percent of DH patients have granular IgA deposits and have an associated gluten-sensitive enteropathy (GSE). In contrast, 15% of DH patients have a linear pattern of IgA deposits and no associated intestinal abnormality. Although circulating IgA antibodies against skin are not present in these patients, 40% of DH patients do have IgA-containing circulating immune complexes (IgA-CIC). The role and origin of the cutaneous IgA and the IgA-CIC in patients with DH are unknown; however, the association of GSE with the granular IgA deposits suggests that a mucosal immune response may be important in the pathogenesis of DH. We have characterized the IgA subclass composition of the cutaneous IgA deposits in patients with DH, and have isolated and characterized the IgA-CIC from these patients. Twenty-nine of 29 patients with DH and granular IgA deposits were found to have only IgA1 deposits. Ten of 11 patients with linear IgA deposits also had only IgA1 deposits; one of 11 had IgA2 deposits. Isolated IgA-CIC from the sera of eight patients with DH and granular IgA deposits were found to contain both IgA1 (58% +/- 5, mean percent of total IgA +/- SEM) and IgA2 (42% +/- 5), as were IgA-CIC from two patients with ordinary GSE without cutaneous IgA deposits. The IgA subclass composition of the isolated immune complexes was significantly different from the serum IgA1 and IgA2 composition (serum IgA1 = 76% +/- 6; IgA2 = 24% +/- 5, p less than 0.025, Student's t-test), and suggests that the IgA-CIC may arise from gut-associated lymphoid tissue (GALT). Sequential anti-IgA1 absorption of serum which contained IgA-CIC did not remove all the IgA-CIC, suggesting that the complexes circulate as separate IgA1 and IgA2 complexes. The finding of IgA1 alone in the skin of patients with DH suggests that the cutaneous IgA may not arise from GALT, or that IgA1, possibly arising in GALT, is preferentially bound to DH skin. Because IgA-containing CIC which contain both IgA1 and IgA2 were found in the serum of patients with DH and with ordinary GSE, it seems unlikely that IgA-containing CIC are responsible for the cutaneous IgA deposits seen in DH.

Antibodies, Monoclonal↗
PubMed1985

Dermatitis herpetiformis in monozygous twins: discordance for dermatitis herpetiformis and concordance for gluten-sensitive enteropathy.

A monozygous female twin pair discordant for dermatitis herpetiformis and concordant for gluten-sensitive enteropathy is reported. The diagnosis of dermatitis herpetiformis was verified by demonstrating granular IgA deposits in the uninvolved skin. Gluten-sensitive enteropathy was confirmed according to the ESPGAN criteria. Monozygosity was proved by the standard genetic characteristics.

Celiac Disease↗
PubMed2002

Levels of F(1+2) prothrombin fragments and thrombin - antithrombin III (TAT) complexes in patients with dermatitis herpetiformis.

BACKGROUND: Dermatitis herpetiformis (DH) is a rare bullous disease of autoimmune etiology. It is an intestinocutaneous syndrome, in which vesicopapular cutaneous lesions are accompanied by gluten-dependent enteropathy. The diagnosis of DH is based on immunopathological investigation of unaffected skin fragment (granular IgA deposits present at the tops of dermal papillae and IgA - EmA present in the serum). MATERIAL/METHODS: The studied group consisted of 33 patients with dermatitis herpetiformis - 23 males and 10 females aged 22-78 (mean age 44.7). In all the patients, the following parameters of blood coagulation systems were determined in the plasma Levels of prothrombin fragments 1+2 (F(1+2)), levels of thrombin - antithrombin III (TAT) complexes, antithrombin III activity (AT III). RESULTS: In patients with dermatitis herpetiformis, the main level of F(1+2) fragments amounted to 9.08 nmol/l, as compared with 1.14 nmol/l in the control group (the difference statistically significant, p<0.0001). In DH patients, the levels of TAT complexes were over twice higher than among the controls and that difference also reached statistical significance (p<0.0001). Mean antithrombin III activity (AT III) in patients with DH reached 102.60% and was similar to that observed in the control group (104.68%). CONCLUSIONS: In patients with dermatitis herpetiformis, increased levels of F(1+2) prothrombin fragments and TAT complexes indicate enhanced in vivo thrombinogenesis. No statistically significant differences of F(1+2), TAT and AT III levels in DH patients related to their sex, age and duration of the disease were observed.

Adult↗
PubMed2000

TCR Vbeta expression in the small bowel of patients with dermatitis herpetiformis and gluten sensitive enteropathy. Limited expression in dermatitis herpetiformis and treated asymptomatic gluten sensitive enteropathy.

Dermatitis herpetiformis (DH) is a blistering skin disease characterized by cutaneous deposits of IgA and an associated, most often asymptomatic, gluten sensitive enteropathy (GSE). Gluten sensitive enteropathy is also seen in patients that do not have skin disease or cutaneous IgA deposits, but do have significant gastrointestinal (GI) complaints. Patients with DH and with GSE without skin disease have similar small bowel morphologic changes and HLA associations and both the skin disease and the GI symptoms can be controlled by a gluten free diet. It is not known what factors allow almost all patients with DH to continue to eat gluten and not develop symptomatic gastrointestinal disease. We have examined the expression of the Vbeta T-cell receptor (TCR) in the small bowel of patients with DH (n=11) and of patients with both symptomatic (n=10) and asymptomatic (n=7) GSE without skin disease to determine if differences in the pattern of TCR Vbeta expression are associated with differences in the clinical manifestations of these diseases. TCR Vbeta expression was analyzed using RT-PCR from small bowel biopsies. Patients with DH and those with GSE without skin disease that were on a gluten free diet and asymptomatic were found to express 6.6 and 5.6 out of 20 Vbeta families respectively, with no single family preference. Examination of peripheral blood lymphocytes from these patients did not reveal any restriction of TCR Vbeta family expression. In contrast, patients with symptomatic GSE expressed 12.6 Vbeta families (P< 0.05), with no consistent preferential expression of any single Vbeta family between patients. Patients with DH, who are continuing to ingest wheat, show a more restricted pattern of TCR Vbeta utilization, similar to that of treated patients with GSE without skin disease, and significantly different from GSE without skin disease patients eating gluten. These findings suggest that the restricted nature of the TCR Vbeta expression may play a role in the different clinical manifestations of dermatitis herpetiformis and isolated gluten sensitive enteropathy.

Adolescent↗
PubMed1995

Enhanced expression of interstitial collagenase, stromelysin-1, and urokinase plasminogen activator in lesions of dermatitis herpetiformis.

Because dermatitis herpetiformis is characterized by neutrophilic inflammation and destructive changes in the basement membrane zone, we studied the in situ expression of interstitial collagenase and stromelysin-1 in 11 lesions. A prominent signal for collagenase mRNA was consistently detected in the basal keratinocytes of rete ridges surrounding the neutrophilic abscesses in 10 of 11 lesions, and the expression was independent of the age of the lesion and the migratory state of the basal keratinocytes. Expression of stromelysin-1 was detected in seven of 11 lesions and co-localized with collagenase. No expression of the 92-kDa gelatinase mRNA or matrilysin protein was found in the vicinity of neutrophilic accumulations or the damaged basement membrane. Urokinase-type plasminogen activator mRNA was found in basal keratinocytes in seven of nine samples. Collagenase, stromelysin-1, and urokinase-type plasminogen activator were not expressed in normal-appearing skin of patients with dermatitis herpetiformis. Our results suggest that in lesions of dermatitis herpetiformis, collagenase and stromelysin-1 may be induced in basal keratinocytes by neutrophil cytokines or by altered cell-matrix interactions through contact of keratinocytes with the matrix due to damaged basement membrane. Stromelysin-1, in particular, may contribute to formation of blisters by degrading basement membrane components.

Collagenases↗
PubMed2002

Selected parameters of fibrinolysis system in patients with dermatitis herpetiformis.

BACKGROUND: Dermatitis herpetiformis (DH) is a very rare bullous dermatitis of autoimmune origin. It is a syndrome involving dermal and intestinal pathology, in which vesicopapular skin lesions are accompanied by gluten-dependent enteropathy. The diagnosis of DH is based on immunopathological investigation of unaffected skin bioptate (presence of granular IgA deposits in the upper portions of dermal papillae). MATERIAL/METHODS: The study was carried out in a group of 33 patients, including 23 males and 10 females aged 22-78 (mean age 44.7 years). The following fibrinolysis system parameters were determined in the sera of all patients: tissue plasminogen activator concentration (t-PA: Ag), urokinase plasminogen activator concentration (u-PA: Ag), plasminogen activator inhibitor concentration (PAI-Ag), plasminogen level, alpha-2 antiplasmin activity (alpha-2-AP), plasmin-- alpha-2 antiplasmin complexes concentration (PAP). RESULTS: Mean t-PA concentration in DH patients was 5.52 ng/ml, vs. 4.8 ng/ml in controls. The respective u-PA concentrations amounted to 0.33 ng/ml, and 0.39 ng/ml. PAI-1 concentration was markedly higher in DH patients (36.2 ng/ml) than in controls (22.40 ng/ml), whereas plasminogen level was significantly lower (86.0% vs. 115.9%). In patients, alpha-2-AP activity was 92% and was lower than in controls -103.4%. DH patients demonstrated also higher concentrations of PAP complexes (327.45 ng/ml) than the control group (203.03 ng/ml). CONCLUSIONS: Patients with DH were found to have lower plasminogen and alpha-2 antiplasmin levels and increased concentrations of PAP complexes, which indicates increased plasminogenesis in vivo. Increased type 1 plasminogen activator inhibitor level (PAI-1) may reflect a chronic inflammatory condition present in DH patients.

Adult↗