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Palmar petechiae or purpura is an unusual finding in dermatitis herpetiformis (DH) that occurs in children but is only rarely reported in adults. We describe a 46-year-old man with DH who presented with the classic pruritic papulovesicular eruption and associated volar finger and palmar petechiae. We discuss recent advances in the pathogenesis and treatment of DH.
We report a case of dermatitis herpetiformis which, 11 years after its original diagnosis, evolved into bullous pemphigoid. Only a few similar cases supported by immunofluorescence studies have been reported previously, and we believe that they represent examples of 'epitope spreading', an increasingly recognized phenomenon used to explain the coexistence of autoimmune diseases.
Immunoelectronmicroscopic and ultrastructural identification of the structures which bind IgA in skin of patients with dermatitis herpetiformis (DH) reveals them to be, in part, a complex of fibrillar components which are covered with amorphous substances. One of the fibers has a diameter of 80--130 A and appears to be tubular, resembling dermal microfibrillar bundles or microtubular elements of elastic fibers. The structures, at times, are associated with the dermal microfibrillar bundles and are found within an elastic fiber system which is in close proximity to the dermal-epidermal junction. Taken together, these findings suggest that the structures which bind IgA are unique to DH and might be a part of an abnormal dermal microfibrillar bundle-elastic fiber system.
Twenty-one patients with dermatitis herpetiformis have been on a gluten free diet regularly followed up for at least one year (mean four years). Eighteen patients had a 'flat' mucosal appearance (grade III), one patient had moderately severe mucosal abnormality (grade II), one patient had mild mucosal abnormality (grade I), and one patient had a normal mucosal appearance (grade O). On the diet, 10 patients had no skin rash and took no dapsone, seven patients controlled the skin rash on a lower dose of dapsone, and four noticed no improvement. There was no correlation between pre-diet jejunal morphology and response of the skin. A repeat jejunal biopsy, on the gluten free diet, was possible in 15 patients. While all those with skin improvement showed some improvement in jejunal morphology, there was no association between the degree of skin improvement and the degree of recovery of the jejunal mucosa.
One-hundred and sixty-eight cases of dermatitis herpetiformis were reviewed to compare the clinical response to and incidence of side-effects from dapsone and sulphamethoxypyridazine. Thirty-seven received sulphamethoxypyridazine (0.25-1.5 g/day) as a single agent therapy at some stage during their care and 161 had dapsone only (50-450 mg/day). Thirty of these patients received both drugs, but at different times. Both were highly effective in controlling the skin disease in 97% of patients on dapsone and 89% on sulphamethoxypyridazine. While 36 (22%) of dapsone-treated subjects had intolerable side effects warranting a change in therapy, this occurred in only five (13.5%) of those treated with sulphamethoxypyridazine. Sulphamethoxypyridazine was also effective as a single agent in three patients with linear IgA disease who had suffered adverse effects from dapsone, and in 10 out of 15 patients with oral and cutaneous lesions of cicatricial pemphigoid.
We have studied the occurrence of associated diseases in a cohort of 305 patients with dermatitis herpetiformis (DH) followed up for a mean of 10 years. The results were compared with those from 383 patients with coeliac disease (CD). Twenty-nine (9.5%) patients with DH and 73 (19.1%) with CD had concomitant endocrine or connective tissue disorders. The following associations were found: auto-immune thyroid disease (4.3% of DH patients and 6.0% of CD patients), insulin dependent diabetes (1.0% DH and 5.5% CD), lupus erythematosus (1.3% DH and 0.3% CD), Sjögren's syndrome (1.0% DH and 2.9% CD), sarcoidosis (1.3% DH and 1.8% CD), and vitiligo or alopecia areata (1.6% DH and 0% CD). We have shown that patients with DH are similar to those with CD in that many have associated endocrine or connective tissue disorders. Most of these diseases began before DH had been diagnosed suggesting that those on a gluten-free diet are not at special risk of contracting them.
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Life-long gluten-free diet is the established therapy for coeliac disease and dermatitis herpetiformis. Diet therapy allows the intestinal mucosa to recover, improves nutrient malabsorption, osteoporosis and a weakened general condition. A gluten-free diet is without wheat, rye and barley and related products. Oats tested free of contamination by other cereals has recently been allowed for adult coeliac patients, but concern still remains regarding its general safety. Gluten-free flour mixes contain more starch and less proteins, vitamins, minerals and fibre compared to regular flour. Recently some questions have been raised as to the nutritional quality of the gluten-free diet. Successful therapy with gluten-free diet requires motivation and dietary counselling, including knowledge of the nutritional value of foods, labelling of prepackaged foods and practical training in cooking. The local chapters of the Norwegian Coeliac Society are active partners, as are the clinical nutritionists that work in most major hospitals. The physician making the diagnosis has a duty to see to it that all coeliac patients get adequate dietary counselling and management.
Circulating immune complexes have been detected in 100% of 59 patients with dermatitis herpetiformis (D.H.), and in 100% of 27 patients with coeliac disease (C.D.). Three methods for detecting immune complexes were employed: radiobioassay, which gave an incidence of 77% in D.H. and 81% in C.D.; C1q binding activity, with which the incidence was 83% and 96%, respectively; and precipitation with 4% polyethylene glycol (69% positivity in D.H., 100% in C.D.). The immune complexes in D.H. and C.D. were compared with those in sera from 23 patients with systemic lupus erythematosus (S.L.E.). Multiple complexes of differing properties were found in D.H. and C.D. but not in S.L.E. The varying nature of the complexes in D.H. and C.D. may account for the damage to different tissues (skin, small intestine, reticuloendothelial system). Low third component of complement was found in 49% and low C4 in 20% of D.H. patients. C3 hypocomplementaemia was found in 26% of patients with C.D.
A patient with clinical findings of dermatitis herpetiformis (DH), negative direct immunofluorescent (DIF) findings for junctional IgA deposits in 2 biopsy specimens, and positive for IgA endomysial (AEmA) and tissue transglutaminase (tTG) antibodies responded initially to dapsone. After dapsone had to be discontinued because of side effects, a gluten-free diet and supportive therapy controlled the disease; the AEmA and tTG antibodies became negative. Our data on 10 consecutive DH cases examined by DIF and by serum studies for AEmA and antibodies to tTG, point to frequencies of 90% DIF positive and 70% AEmA and tTG positive cases. The use of both DIF and serum tests for AEmA and tTG reveals DH cases not detected by DIF alone that respond to gluten-free diet. Findings on autoantibodies to tTG, an enzyme that metabolizes gliadin, points to a role of tTG in the immunopathology of gluten-sensitive enteropathy and helps to explain the need for a gluten-free diet in the management of DH cases.
In 32 patients with dermatitis herpetiformis (DH) we studied the effect of gluten-free (22 patients) and gluten-reduced (10 patients) diet for periods ranging between 15 and 43 months. Variables such as cutaneous manifestations, dependence on dapsone, IgA deposits in the skin, small-bowel function, and jejunal mucosal morphology were studied. 59% of the patients on gluten-free diet could stop dapsone medication and remain symptom-free, compared with 10% on gluten-reduced diet. The time needed to achieve this therapeutic response varied from 5 to 31 months. IgA decreased in the skin to a degree which roughly paralleled the morphological normalization of the jejunal mucosa. In no patient, however, did the IgA completely disappear. It is suggested that IgA is not the main factor inducing DH symptoms, but rather a secondary phenomenon. Repeated jejunal biopsies revealed normalization of the mucosal histology in 52% of the patients on gluten-free diet, compared with none in the gluten-reduced diet group.
The incidence and degree of patchiness of mucosal abnormality in both coeliac disease (CD) and dermatitis herpetiformis (DH) is documented. As judged by both stereomicroscopy and subjective histology, patchiness occurred frequently in both CDand DH patients. In most cases the difference of abnormality was of only one grade, but in approximately 25% as assessed by stereomicroscopy and 10% as assessed by histology the difference was of two or more grades. In control subjects with normal small bowel mucosa the variation of the mucosal appearance between the duodenum and proximal jejunum was studied. Contrary to popular belief, no significant difference of villous and crypt measurements or of apparent villous "bridging" and "branching" between these two sites was found, if only well-orientated sections were studied. The stereomicroscopic appearances were also similar at these two sites, although villi tended to be broader in the duodenal biopsies. The duodenal-jejunal variation was also studied in CD and DH patients and although by both stereomicroscopy and subjective histology the appearances were similar in most patients, in approximately 33% the duodenal abnormality was the most severe and, surprisingly, the jejunal abnormality was more severe in approximately 15%. It is concluded that multiple, precisely located biopsies of both the duodenum and proximal jejunum are invaluable in the investigation of small bowel disease and in assessing response to treatment.
BACKGROUND: The risk for lymphoma is increased in both dermatitis herpetiformis (DH) and in coeliac disease. The lymphoma most associated with coeliac disease is enteropathy-associated T-cell lymphoma. OBJECTIVES: To study the occurrence and type of lymphoma in a large series of patients with DH and their first-degree relatives. METHODS: The occurrence of lymphoma was studied in 1104 patients consecutively diagnosed with DH in two university hospitals during 1969-2001. A questionnaire was sent to 341 patients to examine the occurrence of lymphoma in their 1825 first-degree relatives. To analyse whether the DH patients with lymphoma had adhered to a gluten-free diet similarly to the patients without lymphoma, two age- and sex-matched patients with DH served as controls for each index case. Data on the gluten-free diet were collected from prospectively completed dietary forms and also from medical records. RESULTS: Eleven (1%) patients contracted lymphoma 2-31 years after the diagnosis of DH. Eight had B-cell-type lymphoma, two enteropathy-associated T-cell lymphoma and one remained unclassified due to missing material. Three (0.2%) of the first-degree relatives contracted lymphoma, all B-cell type. The 11 DH patients with lymphoma had adhered to a gluten-free diet significantly less strictly than the DH controls without lymphoma. CONCLUSIONS: The present study documents that patients with DH can have both B- and T-cell lymphoma. The DH patients with lymphoma had not adhered as strictly to the gluten-free diet as the control patients without lymphoma. The occurrence of lymphoma in the first-degree relatives was lower than in the patients with DH.
Thirty to forty percent of patients with dermatitis herpetiformis (DH) have IgA-containing circulating immune complexes (IgA-CIC); however, the antigenic composition of these complexes as well as the role they play in the pathogenesis of DH are unknown. The failure to detect wheat protein in these IgA-CIC, despite the association of DH with gluten-sensitive enteropathy, suggests that the IgA-CIC in DH may be similar to those seen in the IgA nephropathies and represent IgA rheumatoid factor (RF)-IgG complexes. We have examined the sera of 32 patients with DH, 16 non-DH patients positive for RF by latex fixation, and 15 normal subjects for IgA and IgM RF using enzyme-linked immunosorbent assays (ELISAs) and for IgA-CIC using an anti-C3 ELISA. Thirteen of 16 (81%) latex fixation test-positive patients had IgA RF by ELISA and 15/16 (94%) had IgM RF by ELISA. The total amount of RF detected by the ELISA (IgA + IgM RF) correlated with the latex fixation titer (r = 0.678, p = 0.004) in these latex fixation-positive patients. Six of the 16 (38%) latex fixation-positive patients also were found to have IgA-CIC. Solid phase absorption using goat antihuman C3 decreased the levels of immune complexes but not the level of IgA RF, suggesting the IgA-CIC detected do not represent uncomplexed IgA RF. In contrast, although 12 of 31 (39%) patients with DH had IgA-CIC ranging in amount from 0.331-26.0 micrograms IgA/ml (nl less than 0.150 microgram IgA/ml), only 1 of 32 (3%) DH patients had detectable levels of IgA RF (7.0 micrograms IgA/ml, nl less than 2.0 micrograms IgA/ml). Low levels of IgM RF were found in 8/32 (25%) of patients with DH (1.1-1.6 micrograms IgM/ml, nl less than 1.0 microgram IgM/ml). These data document that IgA RF is not present in the sera of patients with DH independent of the presence or absence of IgA-CIC and that it is unlikely that the IgA-CIC present are IgA RF complexed with autologous IgG.