Relationship of dietary gluten intake to dapsone dose in dermatitis herpetiformis.

The gluten intake was quantitated utilizing a dietary history method in 43 patients with dermatitis herpetiformis on non-restricted diet. The mean daily gluten intake was 15 g. The individual intake of gluten was related to the maintenance dose of dapsone. It was significantly higher in patients on 100-150 mg dapsone daily than in those taking 0-25 mg daily. There was a significant correlation between amount of gluten in the diet and the dapsone dose (p less than 0.01, rs = 0.43). Villous atrophy was not related to the dapsone dose. It is suggested that the gluten-sensitive enteropathy changes the intestinal permeability and thus contributes to the development of blisters.

Spontaneous remission of dermatitis herpetiformis: dietary and gastrointestinal studies.

Out of 98 patients with dermatitis herpetiformis (DH) living in Gothenburg, 14 were in spontaneous remission (29% of the patients without gluten free diet). Eight of these volunteered for dietary interviews and further studies. They do not seem to differ from symptomatic DH patients in the frequency of HLA-B8, achlorhydria or small-bowel enteropathy. Their estimated mean daily intake of gluten was below 12 g in six. The mean gluten intake of the eight patients in remission is significantly less than in a group of 34 patients with dapsone-requiring DH on non-restricted diet. Urinary iodine excretion was low in five, all previously instructed to restrict their iodine intake. Dietary factors could thus be suspected to be responsible for some spontaneous remissions in DH.

Malabsorption of vitamin B12 in dermatitis herpetiformis and its association with pernicious anaemia.

Serum vitamin B12 concentrations were determined in 129 patients and a pentagastrin test performed in 116 patients with dermatitis herpetiformis (DH). Low serum vitamin B12 levels were found in 27 patients (21%) and achlorhydria in 30. Forty-two patients with achlorhydria and/or vitamin B12 deficiency were subjected to further haematological and gastrointestinal investigations. Functional or morphological involvement of the small intestine was seen in almost all patients with vitamin B12 deficiency but, in addition, atrophic gastritis resulting in defective secretion of intrinsic factor (pernicious anaemia) was found in 8 cases (7% of all DH patients). Thus, a low vitamin B12 level in DH patients should result in a careful gastrointestinal examination as both an atrophic gastritis and a coeliac-like enteropathy have further clinical implications.

Serum antibodies to gliadin and small-intestinal morphology in dermatitis herpetiformis. A controlled clinical study of the effect of treatment with a gluten-free diet.

Serum gliadin antibodies of the IgA and IgG classes were determined by the diffusion-in-gel enzyme-linked immunosorbent assay in 41 patients with dermatitis herpetiformis before treatment with a gluten-free diet. Increased gliadin antibody levels were found more frequently in patients with subtotal villous atrophy (9 out of 17 patients, or 53%; p less than 0.05) than in patients with partial villous atrophy (2 out of 13 patients, or 15%) or normal villous appearance (2 out of 10 patients, or 20%). The gliadin antibody levels were negatively correlated with the urinary xylose excretion (r = -0.40, p less than 0.02 for the IgA class and r = -0.64, p less than 0.001 for the IgG class). Intestinal morphology improved and mean gliadin antibody levels of the IgA and IgG classes decreased during treatment with a gluten-free diet for 16-36 months (mean, 20 months) (p less than 0.005, n = 26), whereas no significant changes of the gliadin antibody levels or the small-intestinal morphology were observed in the other 15 patients, who continued on a non-restricted diet for 17-35 months (mean, 20 months). Thus, gliadin antibody levels in sera from patients with dermatitis herpetiformis seem to be correlated with the severity of the intestinal disease. However, all patients with villous atrophy are not detected by determination of serum gliadin antibodies.

Changes in food consumption and its nutritional quality when on a gluten-free diet for dermatitis herpetiformis.

Dietary intakes of energy and nutrients were calculated from diet history interviews in 30 patients with dermatitis herpetiformis, before and after 18 months on a gluten-free diet. In spite of great changes in the intake of different foods, the mean intake of dietary fibre did not decrease. There was only a small decrease in the intake of iron in women, while the intake in men did not change. Patients with a previous high intake of gluten had indirect evidence of malabsorption.

Gastric morphology and function in dermatitis herpetiformis and in coeliac disease.

Gastric acid secretory capacity was evaluated in 116 patients with dermatitis herpetiformis by means of the pentagastrin test. Endoscopic gastric mucosal biopsy specimens were obtained from both the body and the antrum in 90 of them. Forty-eight patients (41%) had a maximal acid output less than 10 mmol/h, and 30 of them (26%) were achlorhydric. The frequency of achlorhydria increased with age, and 27 out of 58 patients (47%) more than 50 years old were achlorhydric. Antrum-sparing chronic atrophic gastritis was present in 92% of the achlorhydric patients, and hypergastrinaemia and serum parietal cell antibodies were found in most of them. The prevalence of chronic gastritis of the body and of the antrum increased with age. There was no correlation between atrophic gastritis or achlorhydria and small-intestinal villous atrophy, the results of the D-xylose test, and blood folate and serum zinc determinations. The transferrin saturation index was lower in patients with achlorhydria. The frequency of achlorhydria was significantly higher in patients with dermatitis herpetiformis than in 69 patients with coeliac disease.

Influence of gluten-free diet on the gastric condition in dermatitis herpetiformis.

Achlorhydric atrophic gastritis occurs in approximately 25% of patients with dermatitis herpetiformis (DH). The effect of gluten withdrawal on the gastric condition was studied in 35 patients, with a control group of 20 patients continuing their habitual diet. Gastrointestinal examinations were performed initially and repeated after about 1 3/4 years. Adherence to the diet was confirmed by dietary interviews, improvement of malabsorption test results and intestinal villous structure, and decreased dapsone requirement. Neither the non-restricted diet nor the gluten-free diet had any effect on gastric morphology, the ability to secrete gastric acid, serum gastrin levels, or the frequency or titres of circulating parietal cell antibodies. The findings indicate that gluten is not responsible for the perpetuation of the gastric affection in DH, in contrast to the enteropathy.

Influence of the amount of dietary gluten on gastrointestinal morphology and function in dermatitis herpetiformis.

The individual daily intake of gluten was calculated in 45 patients with dermatitis herpetiformis (DH) on the basis of a depth interview about food habits. Gastric and small intestinal morphology and function were studied concurrently. Mean daily gluten intake was estimated to be 15 g, a figure which corresponds well to the average gluten intake in Sweden. There was a significant correlation between the degree of morphological mucosal changes of the small intestine and the quantity of gluten ingested. All patients with jejunal villous atrophy consumed more than 10 g gluten daily and all but one patient with normal jejunal villous structure had a gluten intake of less than 10 g/d. The findings suggest a dose-dependent effect of gluten on the intestinal mucosa. Conversely, the daily gluten intake was not correlated to gastric morphology, gastric acid secretion, serum gastrin levels or serum parietal cell antibodies. Patients with reduced ability to secrete gastric acid did not differ from the remaining patients in this respect. Whereas the coeliac-like enteropathy in DH seems to be caused by ingested gluten, the frequently occurring achlorhydric atrophic gastritis must be assumed to be of different immunopathogenesis.

Conventional malabsorption tests: do they detect the adult patient with villous atrophy?

A total number of 134 patients with subtotal or partial villous atrophy, of whom 49 had dermatitis herpetiformis, were investigated with blood folate assay and xylose and lactose absorption tests. Faecal fat excretion was determined in 71 patients without dermatitis herpetiformis (coeliac group). A comparison was made between three patient groups, the patients with dermatitis herpetiformis and the coeliac patients studied in 1970-74 and 1975-79, respectively. From clinical and biochemical analyses of these patients we conclude that although a combination of the four malabsorption tests used here still detect a majority of coeliac patients, small intestinal biopsy may reveal villous atrophy also in patients without any laboratory evidence for malabsorption by these commonly used tests. In dermatitis herpetiformis, however, the sensitivity of the tests used was low; these malabsorption tests therefore have little diagnostic value in this category of patients.

Incidence and prevalence of dermatitis herpetiformis in western Sweden.

The annual incidence of IgA-positive dermatitis herpetiformis (DH) during the years 1976-1981 in Gothenburg, Sweden, a city with approximately 430000 inhabitants, was estimated prospectively. The mean annual incidence was 1.1 per 10(5) inhabitants. The prevalence of DH as at 31 December, 1981 was 22.9 per 10(5) inhabitants or 19.6 if patients in long-standing spontaneous remission were excluded. These figures probably represent the minimum overall prevalence rate.

Linear IgA dermatosis: a study of ten adult patients.

Ten adult patients with homogeneous-linear deposition of IgA along the basement membrane zone have been studied. The direct immunofluorescence IgA pattern was stable, and there was no deposition of IgG or IgM. The clinical presentations were heterogeneous and resembled dermatitis herpetiformis (DH) (3 cases) or bullous pemphigoid (5 cases). Two patients had widespread gyrate blistering lesions of acute onset. Pruritus was constantly present. The course of the disease was chronic except for one patient who had a spontaneous remission after 5 years. The histology was indistinguishable from "classical" DH with granular IgA in dermal papillae. The patients studied in the present investigation did not show the high incidence of atrophic intestinal villi found in "classical" DH. Five of 9 cases carried the haplotype HLA-A1, B8, DR3. In spite of a close similarity between linear IgA dermatosis and DH, differences exist which indicate discrepancies in etiopathogenesis.

Endoscopic duodenal biopsy compared with biopsy with the Watson capsule from the upper jejunum in patients with dermatitis herpetiformis.

Forty-eight patients with dermatitis herpetiformis were investigated with duodenoscopy, endoscopic biopsy, and conventional upper jejunal biopsy. Three endoscopic biopsy specimens were obtained from the duodenal bulb, three to six specimens from the second part of the duodenum, and one suction biopsy from the upper jejunum. In 60%, 71%, and 65% of the patients subtotal or partial villous atrophy was found in biopsies from the three levels, respectively. In eight patients (17%) mucosal abnormalities were confined to the duodenal biopsies and in two patients to the jejunal biopsies. The technique of endoscopic biopsy is advocated because multiple visually selected duodenal specimens may be obtained and because this technique also enables a complete evaluation of the stomach.

[The lymphographic findings in the yellow-nail syndrome (author's transl)]. / Lymphographische Befunde beim Yellow-Neil-Syndrom.

Approximately 38 cases of the yellow nail syndrome have been described in the literature and one further case of this rare condition is added. The symptoms and various associated conditions are discussed. The lymphographic findings in one case are described in detail.

[Gold therapy in psoriasis arthropathica]. / Goldtherapie bei Psoriasis arthropathica.

For two patients with psoriasis arthropathica therapy with gold salts was started. In both cases a serious psoriasis pustulosa occurred after the first injection together with a leucemic reaction. The mechanism of gold side effects is discussed on the basis of the literature and these cases. The value of the therapy of the psoriatic arthropathie with gold is questioned.

Development of gastric dysfunction in dermatitis herpetiformis.

In order to study the development of atrophic gastritis, gastric secretory function was examined by a standard pentagastrin test 24 to 78 months after a previous examination (Ex I). The study included 12 patients with dermatitis herpetiformis (DH), 6 patients with functional signs of atrophic gastritis previously, and 8 healthy controls. The surrent examination (Ex II) also included microbiological culture of gastric juice and estimation of gastrin(s), parietal cell and thyroidal antibodies. Most of the controls had increased their maximum acidity and maximum acid output (MAO) between the examinations. This may indicate an altered potency of pentagastrin in recent years. Conversely, 5 of the 6 patients with atrophic gastritis showed a further reduction of maximum acidity and MAO, indicating progressive parietal cell atrophy. In the DH-group, two tendencies were observed: 6/12 patients had an increased MAO at Ex II. They had had lower mean age and higher mean MAO at Ex I, as compared with the remaining 6 patients who had a decreased MAO at Ex II. The latter group more often had parietal cell antibodies.

Systemic retinoid therapy of systematized verrucous epidermal nevus (with 1 colour plate).

In a patient with systematized verrucous epidermal nevus, marked improvement was obtained with oral administration of a new aromatic retinoid (Ro 10-9359). Mild cheilitis and thinning of scalp hair were the only side effects obser Withved. continuous treatment the good result was maintained for 10 months. Further investigation is needed to determine, whether long-term oral administration of this new drug is feasible.

Iron absorption in patients with dermatitis herpertiformis.

Iron absorption has been studied in patients with dermatitis herpetiformis (DH). Four patients out of 20 had iron deficiency, defined as absence of or only traces of haemosiderin in bone marrow smears. These four had adequate absorption of ferrous iron. The iron deficiency in at least 3 of them was ascribed to increased iron loss. The results indicate that, although having a mild to moderate malabsorption syndrome, DH patients can be expected to exhibit adequate absorption of orally administered iron. Explanations of a negative iron balance other than defective absorption should therefore be sought.

Auto-immune atrophic gastritis in patient with dermatitis herpetiformis.

Seventeen patients with dermatitis herpetiformis were tested for gastric hydrochloric acid secretion. Seven were found to be achlorhydric. Atrophic gastritis in these patients probably had an auto-immune pathogenesis, as judged by elevated serum gastrin level, high prevalence of antibodies against gastric parietal cells and antrum sparing of the gastric atrophy. This type of atrophic gastritis is considered to indicate a precursor state to pernicious anemia.