Drug induced linear IgA bullous dermatosis

Linear IgA disease is an autoimmune subepidermal bullous disease in which linear IgA deposits are found at the basement membrane zone. It is classically idiopathic but a drug-induced variant seems to be individualized in which cutaneous lesions resolve spontaneously after cessation of responsible treatment. Among the commonly implicated drugs, vancomycin is the most frequently reported. One should not however ignore other precipitating events sometimes associated, particularly infectious diseases and non-lymphoid or lymphoproliferative malignancies. Authors present here clinical and histological features of this disease as well as drugs that have been implicated.     

Gastric lesion in dermatitis herpetiformis.

Five of 33 patients with dermatitis herpetiformis (DH) were found to have gastric parietal cell antibody in their sera, whereas it was not found in 30 healthy controls of comparable age distribution. Fifteen of the patients with DH underwent further studies to investigate the histological and functional state of their gastric mucosa. Atrophic gastritis was found in all five patients whose sera contained gastric parietal cell antibody and in three of 11 patients with no antibody in their sera. In addition, there was marked impairment of acid secretion in the DH group as a whole, but, apart from one patient with overt pernicious anaemia (PA), there was no evidence of malabsorption of B12.     

Tolerance to oats in dermatitis herpetiformis

Objectives—Recent studies on coeliac diseasehave shown that oats can be included in a gluten-free diet withoutadverse effects on the small bowel. The presence of a rash is also asensitive indicator of gluten ingestion in dermatitis herpetiformis,and this was used to study whether patients with this disease could also tolerate oats.
Patients/Methods—Eleven patients with dermatitisherpetiformis in remission on a gluten-free diet were challenged dailywith 50 g oats for six months. Clinical symptoms were recorded, serum samples taken, and skin and small bowel biopsies performed before andafter the oat challenge. A control group comprised of 11 patients withdermatitis herpetiformis on a conventional gluten-free diet was also studied.
Results—Eight patients challenged with oatsremained asymptomatic, two developed a transient rash, and one withdrewbecause of the appearance of a more persistent but mild rash. Three of the 11 controls also developed a transient rash. IgA endomysial antibodies remained negative in all patients. The small bowel villous architecture, the densities of intraepithelial CD3 and α/βand γ/δ T cell receptor positive lymphocytes and crypt epithelial cell DR expression remained unaltered during the oat challenge.
Conclusions—The results confirm the absenceof oat toxicity on the gluten sensitive small bowel mucosa and suggestthat the rash in patients with dermatitis herpetiformis is notactivated by eating oats.

Keywords:dermatitis herpetiformis; coeliac disease; gluten-free diet; oats

     

Small-intestinal bacterial flora in dermatitis herpetiformis

Examination of jejunal aspirates from 22 patients with dermatitis herpetiformis has shown that bacterial colonization of the upper small intestine often occurs. However, a high proportion of the patients had an impaired capacity to secrete gastric acid, and comparison of their jejunal flora with control subjects selected on the basis of gastric acid secretion showed similar bacteriological profiles. Thus colonization of the small intestine in dermatitis herpetiformis is not a primary feature of the condition itself, but is attributable to the frequently associated impairment of gastric acid secretion. Neither impaired acid secretion nor bacterial overgrowth in the small intestine appeared to be responsible for the high concentrations of immunoglobulins found in jejunal aspirates from patients with dermatitis herpetiformis.     

Celiac disease, dermatitis herpetiformis and erosive jejunoileitis

A 21-year-old man with coeliac disease and dermatitis herpetiformis presented successively with erosive ileitis (warranting surgical resection) and erosive jejunitis (proven by jejunoscopy). Discontinuous antibiotic therapy was associated with a gluten-free diet and evolution was favorable as judged with five years follow-up. Malabsorption with mucosal ulcerations can be due to: a) chronic ulcerative duodeno-jejuno-ileitis or Jeffries' disease; b) coeliac disease which may be classified as possible, probable, or certain according to the strictness of criteria. Our case is the fourth in which a villous response was proven after gluten-free diet. It is also particular in that the erosions were superficial; c) malignant lymphoma which can reasonably be excluded here. This observation confirms that a gluten-free diet may be effective after surgical resection of the ulcerated segment in complicated coeliac disease and shows that antibiotics may be an useful adjuvant to therapy.     

Polymyositis/dermatomyositis associated with dermatitis herpetiformis

We describe 2 patients with dermatitis herpetiformis who developed polymyositis/dermatomyositis. On HLA typing, both patients were found to be HLA-B8, DR3 positive. The concurrence of these two relatively rare diseases, both associated with immunologic abnormalities, further supports the role of autoimmunity in their pathogenesis and indicates a possible common genetic basis. It also suggests that myositis may be more common in patients with dermatitis herpetiformis than in the general population.     

Antibodies to gliadin in adult coeliac disease and dermatitis herpetiformis

Antibodies to gliadin, searched for by indirect immunofluorescence and a micro-ELISA, were detected in 16 (64%) of 25 sera from patients with adult coeliac disease and in 13 (45%) of 29 with dermatitis herpetiformis. Although the sensitivity of the two tests was relatively low in the whole groups, it increased when only cases with severe jejunum abnormalities were considered (93% for coeliac disease and 81% for dermatitis herpetiformis). A significant correlation was found between antigliadin antibodies and the severity of jejunum damage in both diseases. Moreover, most coeliac and dermatitis herpetiformis patients with antigliadin antibodies were on normal diet. The specificity of the tests was 100% for the immunofluorescence and fairly good for the micro-ELISA, as only 5 (11%) of the 46 disease control patients (Crohn's disease, ulcerative colitis) were positive for antigliadin antibodies. R1-reticulin antibody test was equally specific but less sensitive in both groups. We conclude that antigliadin antibodies are useful in the diagnosis of patients with active adult coeliac disease and dermatitis herpetiformis with gluten-sensitive enteropathy. Moreover, the two tests make it possible to monitor the compliance to gluten-free diet in both diseases.     

Small intestinal function and dietary status in dermatitis herpetiformis.

Small intestinal morphology and function were assessed in 82 patients with dermatitis herpetiformis, 51 of whom were taking a normal diet and 31 a gluten free diet. Methods used were histopathological evaluation of jejunal mucosal biopsy specimens, quantitation of intraepithelial lymphocytes, cellobiose/mannitol permeability test, tissue disaccharidase values, serum antigliadin antibodies, and formal assessment of dietary gluten content by a dietician. There was no correlation between dietary gluten intake and the degree of enteropathy in the 51 patients taking a normal diet, whereas biopsy specimens were normal in 24 of the 31 patients on a gluten free diet, all previously having been abnormal. Eighteen patients on gluten containing diets had normal jejunal histology and in seven of these all tests of small intestinal morphology and function were entirely normal. Intestinal permeability was abnormal and serum antigliadin antibodies were present in most patients with enteropathy. Studies of acid secretion in seven patients showed that hypochlorhydria or achlorhydria did not lead to abnormal permeability in the absence of enteropathy. This study shows that a combination of objective tests of small intestinal architecture and function will detect abnormalities in most dermatitis herpetiformis patients, including some with histologically normal jejunal biopsy specimens. Nevertheless there is a small group in whom all conventional intestinal investigations are entirely normal.     

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.     

Linear IgA bullous dermatosis responsive to a gluten-free diet

Dermatitis herpetiformis is associated with a gluten-sensitive enteropathy in >85% of cases. Both the skin lesions and the enteropathy respond to gluten restriction. Linear IgA bullous dermatosis has a much lower prevalence of histological small bowel abnormalities, and lesions are not known to respond to gluten restriction. We report a patient with linear IgA bullous dermatosis and gluten-sensitive enteropathy. This report addresses the issue of whether linear IgA bullous dermatosis can be associated with gluten-sensitive enteropathy. We evaluated the response to gluten restriction and normal diet by following the status of the patient's jejunal biopsies and skin lesions. The patient responded to gluten restriction, as shown by resolution of jejunal abnormalities and skin lesions and subsequently by recurrence of jejunal abnormalities and skin lesions with reinstitution of a gluten-containing diet. This report demonstrates that linear IgA bullous dermatosis can respond to gluten restriction if an underlying gluten-sensitive enteropathy is present.