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PubMed1984

[Immunoblastic sarcoma associated with dermatitis herpetiformis].

Association between dermatitis herpetiformis and gluten enteropathy is well established. An increased incidence of malignant disease has been reported in patients with coeliac disease and also in dermatitis herpetiformis. Linear IgA dermatitis herpetiformis has a much lower incidence of associated enteropathy compared with patients with papillary dermatitis herpetiformis, but the risk of malignancy could be the same. We report a case of linear IgA dermatitis herpetiformis in which immunoblastic sarcoma subsequently developed.

Celiac Disease↗
PubMed1979

Juvenile dermatitis herpetiformis.

Juvenile dermatitis herpetiformis occurred in a 20-month-old girl. She had granular lgA, C3, and fibrin bound to the basement membrane zone of the skin by direct immunofluorescence and negative serum antibodies against the skin on indirect immunofluorescence. The HLA typing of peripheral lymphocytes was A1, Aw30, B8, Bw51 without clinical evidence of malabsorption syndrome. A rapid improvement was observed on dapsone therapy. These findings strongly suggest that juvenile dermatitis hepetiformis is a disease entity different from chronic bullous dermatosis of childhood.

Basement Membrane↗
PubMed2001

Parallel expression of macrophage metalloelastase (MMP-12) in duodenal and skin lesions of patients with dermatitis herpetiformis.

BACKGROUND: Dermatitis herpetiformis (DH) is a specific dermatological manifestation of coeliac disease and 80% of DH patients have gluten sensitive enteropathy manifested by crypt hyperplasia and villous atrophy. Matrix degradation mediated by collagenase 1 (MMP-1) and stromelysin 1 (MMP-3) has previously been implicated in the pathobiology of coeliac intestine and cutaneous DH blisters. AIMS: To study expression of stromelysin 2, metalloelastase, collagenase 3, and matrilysin in the intestine and skin of DH patients. METHODS: In situ hybridisation using 35S labelled cRNA probes was performed on duodenal biopsies of 15 DH patients, three samples each of control duodenal or jejunal mucosa, fetal ileal explants, lesional DH skin, and 19 serial biopsies of experimental DH blisters. Immunostaining was used to examine type IV collagen, macrophages (CD68), and 92 kDa gelatinase (MMP-9) in the specimens. RESULTS: Metalloelastase (MMP-12) was abundantly expressed by subepithelial macrophages in both coeliac intestine and spontaneous and induced DH rash. It was also upregulated in the experimental model of coeliac disease (staphylococcal endotoxin B stimulated fetal explants). The only other MMP detected was MMP-9 which did not colocalise with MMP-12. CONCLUSIONS: Upregulation of metalloelastase is associated with T cell mediated immune responses both in the intestine and skin. In addition to modulating macrophage migration, it may contribute to degradation of proteoglycans or basement membrane components in the subepithelial mucosa.

Adolescent↗
PubMed2002

Serum eosinophil cationic protein, myeloperoxidase, tryptase, eotaxin and Th2-L-like cytokines in Dermatitis herpetiformis.

BACKGROUND: Dermatitis herpetiformis (DH) is a subepidermal bullous disease characterized by a neutrophilic and eosinophilic infiltrate, together with activated lymphocytes that show preferential polarization towards the Th2-like phenotype. To date, the role of neither soluble mediators, such as cytokines, nor serum levels of myeloperoxidase (MPO), eosinophil cationic protein (ECP) and tryptase (TRY) have been studied in the peripheral blood of DH. In this study we investigated 40 DH subjects for possible alterations in MPO, ECP, TRY, eotaxin, interleukin (IL) 4 and IL-5 concentrations to evaluate the role of neutrophils, eosinophils and mast cells in the induction of skin lesions. Sera from 20 healthy subjects were tested as controls. METHODS: Eotaxin, IL-4 and IL-5 serum contents were determined by a solid-phase enzyme-linked immunosorbent assay. A double antibody radioimmunoassay was used to measure serum levels of MPO, ECP and TRY. RESULTS: Normal levels of eotaxin and IL-4 were found in all DH subjects; increased IL-5 levels were found only in 1 subject. The MPO levels were significantly higher in DH subjects than in healthy controls (p < 0.01). There was also a significant difference between the serum ECP levels of DH and healthy controls (p < 0.05). TRY was not revealed in the serum of DH subjects. CONCLUSIONS: The determination of Th2-like cytokines and TRY serum levels is not important in quantifying the involvement of activated lymphocytes and mast cells in DH; however, the finding of high MPO and ECP serum levels seems to confirm that these products are good markers of the presence of activated neutrophils and eosinophils.

Adolescent↗
PubMed1979

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.

Adolescent↗
PubMed2006

Dermatitis herpetiformis presenting as intertriginous dermatitis.

A 54-year old man with atypical dermatitis herpetiformis Duhring lasting about three years is reported. The lesions were located exclusively in the pubic area, groins, inner thighs and perianal regions. Routine histopathology and the direct as well as the indirect immunofluorescence supported the diagnosis. Application of a gluten-free diet and dapsone alleviated the symptoms.

Dermatitis Herpetiformis↗
PubMed2002

Dermatitis herpetiformis and neurological dysfunction.

Dermatitis herpetiformis and coeliac disease are gluten sensitive diseases, which have common immunopathological and genetic mechanisms. Neuropsychiatric complications have been reported in up to 26% of patients with coeliac disease. This is probably an overestimate, because of the chance associations with some common neurological conditions such as epilepsy. The pathogenesis is speculative but it has been postulated that gluten is neurotoxic possibly via immune mechanisms. The frequency of neurological dysfunction in patients with dermatitis herpetiformis has not been characterised. Patients with dermatitis herpetiformis might be expected to be particularly susceptible to neuronal damage as some continue to consume gluten when their dermatological symptoms are controlled by dapsone. Thirty five patients were recruited with dermatitis herpetiformis from dermatology clinics at St Mary's Hospital, London and Queen's Medical Centre, Nottingham and investigated for evidence of neurological abnormality. All patients underwent a full neurological examination and were asked about their neurological and general medical history by means of a structured questionnaire. Serum samples were taken and screened for the presence of anti-neuronal antibodies (anti-Hu and Yo) as well as anti-gliadin (IgA and G) anti-endomysial (IgA), and anti-tissue transglutaminase (IgA) antibodies. Neurophysiological tests were carried out where appropriate. Only two patients were identified with unexplained neurological abnormalities (one essential tremor, and one chorea). Two other patients had a history of migraine. The patient with chorea also had borderline/equivocally positive anti-Hu antibodies by immunofluorescence assay. All other samples were negative for anti-neuronal antibodies. Fifteen patients were positive for anti-gliadin antibodies (IgA and/or IgG), four for anti-endomysial antibodies (monkey oesophagus or umbilical cord), and six for anti-tissue transglutaminase antibodies. The presence of these antibodies did not correlate with the presence of neurological abnormalities. No cases of "gluten ataxia" were identified. In conclusion, there was no convincing evidence for immune mediated neurological damage in this pilot study of dermatitis herpetiformis.

Adolescent↗
PubMed1979

Atopic disease and dermatitis herpetiformis.

Patients with dermatitis herpetiformis were found to have an increased incidence of atopic disorders compared to an age and sex matched control group. The increase in incidence was statistically significant for atopic eczema and any atopic disorder. Serum immunoglobulins (including IgE) were estimated in forty-five dermatitis herpetiformis patients and no significant abnormalities were found.

Adolescent↗
PubMed2002

First-degree relatives are frequently affected in coeliac disease and dermatitis herpetiformis.

BACKGROUND: Coeliac disease and dermatitis herpetiformis are phenotypically distinct gluten-sensitive diseases. Coeliac disease is known to cluster in families, whereas there is little evidence for dermatitis herpetiformis and for the occurrence of both diseases in the same families. METHODS: The study group comprised 380 patients with coeliac disease and 281 patients with dermatitis herpetiformis, with a total of 3158 first-degree relatives, followed up for a mean of 14 years. The patients were questioned about affected first-degree relatives. The prevalence and incidence of biopsy-proven coeliac disease and dermatitis herpetiformis in relatives were determined. RESULTS: Seventy-three (19.2%) patients with coeliac disease and 51 (18.1%) with dermatitis herpetiformis had affected first-degree relatives. The prevalence among relatives was similar for both diseases; 4.7% and 3.9% of the relatives had coeliac disease and 0.8% and 1.5% had dermatitis herpetiformis, respectively. The disease prevalence was 7% among siblings, 4.5% among parents and 3.5% among children. The annual incidence was 3/1,000 relatives, which is 15 times higher than among the general population. Coeliac disease and dermatitis herpetiformis were mixed in several multiple-case families. CONCLUSIONS: The present long-term follow-up study of coeliac disease and dermatitis herpetiformis shows that every fifth patient can have affected first-degree relatives, and that the prevalence among relatives is 5.5%. Dermatitis herpetiformis segregates also in the families of patients with coeliac disease, and vice versa, indicating the same genetic background.

Adolescent↗
PubMed1989

IgA antiendomysial antibodies in dermatitis herpetiformis.

Sera from 24 patients with dermatitis herpetiformis and 80 control subjects (patients with other bullous diseases, nonbullous dermatoses, and noncutaneous diseases) were studied to determine the usefulness of assay for IgA antiendomysial antibodies (IgA-EMA) in the diagnosis of dermatitis herpetiformis. The overall sensitivity of IgA-EMA for the diagnosis of dermatitis herpetiformis was 79% and the specificity was 96%. When the three patients with dermatitis herpetiformis who were faithfully following gluten-free diets were excluded, the sensitivity was 90% and the specificity was 96%. No patient in the bullous disease control group (including patients with linear IgA bullous dermatosis) had circulating IgA-EMA. One patient, who did not have direct immunofluorescence evidence for dermatitis herpetiformis but had IgA nephropathy, had a positive IgA-EMA result, an interesting association in light of the rare reports of dermatitis herpetiformis in patients with IgA nephropathy and IgA antigliadin antibodies associated with IgA nephropathy. Although direct immunofluorescence testing of skin biopsy specimens remains the most definitive diagnostic test for dermatitis herpetiformis, indirect immunofluorescence assay of serum for IgA-EMA is a minimally invasive study with a high sensitivity and specificity for dermatitis herpetiformis.

Adult↗
PubMed1996

Malignancy and survival in dermatitis herpetiformis: a comparison with coeliac disease.

BACKGROUND: Dermatitis herpetiformis is a lifelong, gluten sensitive skin disease. Patients with dermatitis herpetiformis, similar to patients with coeliac disease not adhering to a gluten free diet, seem to have increased risk for lymphoma. AIMS: This study looked at the occurrence of malignancy and survival of patients with dermatitis herpetiformis and compared the results with those seen in patients with coeliac disease or in the general population. PATIENTS: A total of 305 adult patients with dermatitis herpetiformis diagnosed at the University Hospital of Tampere in 1970-1992 were studied. Most patients started a gluten free diet and at the end of the study 93% of the patients were adhering to the diet. A control group comprised 383 adult patients with coeliac disease, 81% of them adhered to a gluten free diet, 6% had a normal diet, and in 13% the diet history remained unknown. METHODS: The occurrence of malignant diseases and survival of the patients were assessed up to the end of 1993. Standardised incidence ratios (SIR) with 95% confidence intervals were used for the malignant diseases. The survival of the patients was compared with that of the general population. RESULTS: Thirteen (4.3%) patients with dermatitis herpetiformis developed 14 malignant disorders during the follow up (SIR 1.25; 95% confidence intervals 0.68 to 2.09). A non-Hodgkin's lymphoma occurred in four patients with dermatitis herpetiformis, significantly more than expected (SIR 10.3; 2.8-26.3). Thirteen (4.3%) patients with dermatitis herpetiformis died during the follow up but there was no increased general mortality. In coeliac disease, 13 (3.4%) patients developed malignancy (SIR 1.16; 0.62 to 1.97), 31 (8.1%) patients died but the survival rate did not differ from that in the general population. CONCLUSIONS: The incidence of non-Hodgkin's lymphoma was significantly increased in patients with dermatitis herpetiformis. The results also confirm that the patients with dermatitis herpetiformis treated mainly with a gluten free diet have no increased general mortality.

Adolescent↗
PubMed1995

A Japanese case of the fibrillar type of dermatitis herpetiformis.

BACKGROUND: Although dermatitis herpetiformis (DH) is a relatively common disease in Caucasian populations, this disease is very rare in Asian populations including the Japanese. METHODS AND RESULTS: We present a Japanese DH patient, who showed a fibrillar pattern of deposition of IgA, IgG, IgM and C3. The HLA typing revealed no B8/DR3. The survey of the Japanese literature and the comparison to studies on American or European DH revealed several interesting differences: high frequency of the fibrillar pattern, relatively high incidence of deposits of immunoglobulins other than IgA, rarity of gluten-sensitive enteropathy and HLA-B8/DR3 in Japanese DH. CONCLUSION: The present study suggests that there may be a significant difference in pathophysiology between Caucasian and Japanese DH patients.

Complement C3↗
PubMed1986

Long-term follow-up of dermatitis herpetiformis in children.

Dermatitis herpetiformis was diagnosed in seventy-six children by its clinical features and by detection of granular IgA deposits in the papillary dermis of perilesional skin. Enteric alterations demonstrated by measurement of D-xylose absorption and by small intestinal biopsies were detected in over 90% of all patients. A complete follow-up was obtained for all children who were followed for 3 to 10 years. Treatment with a gluten-free diet alone led to a reversal of the intestinal abnormality in 100% of our children and to the disappearance of cutaneous lesions in 82% of reported cases. This suggests that a complete remission of symptoms can be obtained with a gluten-free diet alone. Dapsone alone is effective therapy for the rash but does not affect the intestinal alterations. Furthermore, the lack of side effects to dietetic therapy for a long period of time makes the diet the treatment of choice in this disease.

Child↗
PubMed2005

Permeability, zonulin production, and enteropathy in dermatitis herpetiformis.

BACKGROUND & AIMS: Dermatitis herpetiformis (DH) is characterized by variable degrees of enteropathy and increased intestinal permeability. Zonulin, a regulator of tight junctions, seems to play a key role in the altered intestinal permeability that characterizes the early phase of celiac disease. Our aim was to assess both intestinal permeability and serum zonulin levels in a group of patients with DH having variable grades of enteropathy. METHODS: We studied 18 DH patients diagnosed on the basis of characteristic immunoglobulin (Ig)A granular deposits in the dermal papillae of noninvolved skin. Results were compared with those of classic celiac patients, patients with linear IgA dermatosis, and healthy controls. RESULTS: According to Marsh's classification, 5 patients had no evidence of enteropathy (type 0), 4 patients had type II, 2 patients had type IIIb damage, and 7 patients had a more severe lesion (type IIIc). Intestinal permeability (lactulose/mannitol ratio [lac/man]) was abnormal in all patients with DH. Patients with more severe enteropathy had significantly greater permeability ( P < .05). The serum zonulin concentration (enzyme-linked immunosorbent assay) for patients with DH was 2.1 +/- .3 ng/mg with 14 of 16 (87.5%) patients having abnormally increased values. In contrast, patients with linear IgA dermatosis had normal histology, normal intestinal permeability, and negative celiac serology. CONCLUSIONS: Increased intestinal permeability and zonulin up-regulation are common and concomitant findings among patients with DH, likely involved in pathogenesis. Increased permeability can be observed even in patients with no evidence of histologic damage in biopsy specimens. Patients with linear IgA dermatosis appear to be a distinct population with no evidence of gluten sensitivity.

Adolescent↗
PubMed1974

The small intestine in dermatitis herpetiformis.

Small intestinal biopsies from 43 patients with dermatitis herpetiformis have been studied. The diagnosis of dermatitis herpetiformis was made on clinical and histological criteria and the presence of IgA deposits in the uninvolved skin. The macroscopic appearance of the intestinal biopsy was flat in 13, convoluted in 10, leaves only in eight, and fingers and leaves in 12. Twenty small intestinal biopsies from patients who did not have dermatitis herpetiformis or gastrointestinal disorder showed leaves only in three and fingers and leaves in 17. The mean total lymphocyte count per 1000 epithelial cells for this control group was 159 +/- SE 13; for the dermatitis herpetiformis patients with flat biopsies it was 464 +/- SE 27; for the convoluted biopsies 365 +/- SE 59; for leaves only 535 +/- SE 39; and for the fingers and leaves biopsies 301 +/- SE 31. The counts for all four groups are significantly greater than the control group (P < 0.001). Three of the 43 patients with dermatitis herpetiformis had lymphocyte counts below 200 per 1000 epithelial cells, and four of our controls had counts greater than 200 but none above 300. In the control group the mean counts for lymphocytes per 1000 epithelial cells in the basal position of the intestinal epithelium was 79 +/- SE 8; in the dermatitis herpetiformis flat biopsies it was 89 +/- SE 11; for the convoluted biopsies 93 +/- SE 12; for the leaves only biopsies 121 +/- SE 18 and for the fingers and leaves 98 +/- SE 13. However, the mean lymphocyte count in the perinuclear position was 81 +/- SE 7 for the controls, 362 +/- SE 23 for the flat dermatitis herpetiformis biopsies; 251 +/- SE 46 for the convoluted specimens; 402 +/- SE 43 for the leaves only specimens, and 195 +/- SE 25 for the fingers and leaves biopsies. The mean lymphocyte count for the supranuclear position was 0.55 +/- SE 0.22 for the control group; 13.7 +/- SE 2.3 for the flat dermatitis herpetiformis biopsies; 20.0 +/- SE 6.9 for the convoluted biopsies; 14.5 +/- SE 3.3 for the leaves only biopsies; and 8.3 +/- SE 2.6 for the fingers and leaves biopsies. Thus in gluten-sensitive enteropathy the increase in lymphocytes in the intestinal epithelium is in the perinuclear and supranuclear position. The ratio of basal lymphocytes to peri- and supranuclear lymphocytes appears to be 1:1 in normal intestinal epithelium, but approximately 1:4 in the gluten-sensitive enteropathy of dermatitis herpetiformis.

Adult↗
PubMed1995

Intracorneal nuclear dust aggregates in dermatitis herpetiformis. A clue to diagnosis.

Dermatitis herpetiformis has a characteristic histologic pattern consisting of subepidermal blisters often containing fibrin, infiltrates of neutrophils and nuclear dust at tips of dermal papillae, and papillary dermal edema. These are features of early and evolving lesions. We present two cases of clinically typical dermatitis herpetiformis with previously unreported histologic features that may provide a significant diagnostic clue. In each of these cases there were focal collections of nuclear dust in the cornified layer of the epidermis, a finding that may represent a resolving phase of dermatitis herpetiformis, beyond the usual papillary dermal neutrophilic microabscesses seen in early lesions. Biopsy material was available for immunofluorescent studies in one of the cases presented. In addition to the granular pattern of IgA positivity at the dermal-epidermal junction, which is diagnostic of dermatitis herpetiformis, this biopsy also showed similar IgA positivity in the intracorneal nuclear dust aggregates. In the second case, initial sections showed only intracorneal nuclear dust, but at deeper levels there were more typical diagnostic microabscesses at the tips of dermal papillae.

Abscess↗