Janet Marks

CŒLIAC SYNDROME IN DERMATITIS HERPETIFORMIS

Abstract 24 patients with dermatitis herpetiformis have been studied. 9 had a flat jejunal mucosa, 8 a convoluted mucosa, 3 a ridged mucosa, and only 4 had fingers and leaves. Clinical evidence of malabsorption was present in only 4 patients in contrast to the finding of gross abnormality of the jejunal mucosa in 17. Biochemical evidence of malabsorption of fat, D-xylose, and lactose was found. There was no biochemical or radiological evidence of osteomalacia. In 10 patients mucosal-biopsy specimens from the lower jejunum or ileum were compared with the abnormal specimens from the upper jejunum: the abnormality was less severe in the lower small bowel in 6 of the 10 patients. This suggests a dietary cause for the enteropathy and our findings point to gluten as the dietary factor in some of the patients. Treatment by gluten-free diet did not benefit the rash nor affect the dose of dapsone required to control it. The relationship between dermatitis herpetiformis and the commonly associated enteropathy is evidently indirect. The findings in 4 close relatives of our patients suggest that the basis of the association may be genetic. The high incidence and severity of the enteropathy in patients with dermatitis herpetiformis make it essential that full small-intestinal investigations should be carried out in all patients with this dermatosis. The enteropathy may respond to treatment with a gluten-free diet but not in our experience the rash, for which dapsone should still be given.

Comparison of photochemotherapy and dithranol in the treatment of chronic plaque psoriasis.

A two-centre trial has been carried out on 224 patients with chronic plaque psoriasis randomly allocated to treatment with a standard dithranol regimen of 8-methoxypsoralen and long-wave ultraviolet light (P.U.V.A.). Lesions in 91% of the 113 in the P.U.V.A. group cleared satisfactorily compared with 82% of 111 in the dithranol group, but clearing took longer (34.4 +/- 1.8 S.E. days) with P.U.V.A. than with dithranol (20.4 +/- 0.9 S.E. days). P.U.V.A. treatment took less patient-time and nurse-time and was more convenient and acceptable to the patients. Patients in whom lesions had failed to clear with dithranol, and some who had needed methotrexate for control, responded satisfactorily to P.U.V.A. A few patients who had failed on P.U.V.A. were treated with dithranol and responded to it. There is a case for the use of P.U.V.A. for patients who would otherwise require methotrexate and those who cannot be managed successfully with dithranol. There is also no reason to withhold P.U.V.A. in patients of 60 years or above with chronic plaque psoriasis. However, despite its superiority in terms of cost and patient acceptability, P.U.V.A. cannot be recommended as the first line of treatment for patients with uncomplicated, dithranol-responsive plaque psoriasis until there is more information on relapse-rate and toxicity.

Cell kinetics in flat (avillous) mucosa of the human small intestine

A new method for the analysis of small-intestinal crypt-cell kinetics using routine peroral diagnostic biopsies is described. Untreated patients with childhood and adult coeliac disease and adults with the gluten-sensitive enteropathy of dermatitis herpetiformis were studied, together with groups of adult and childhood controls. In the classical flat avillous mucosae the increase in crypt size was found to be three-dimensional. The number of proliferating cells per crypt was shown to be markedly increased, and an even greater rise in the crypt-cell production rate was demonstrated. A significant increase in the mitotic index was also confirmed in the avillous mucosae. On the basis of these findings it is suggested that the characteristic crypt morphology in glutensensitive enteropathy can be explained as an adjustment to accommodate the expanded mass of proliferating and maturing cells necessary to support the augmented cell production rate. We may speculate that this in turn is a response to a pathologically rapid loss of cells from the mucosal surface.

Cyclosporin A in atopic dermatitis: therapeutic resonse is disociated form effects on allergic reactons

Summary Fourteen patients with severe chronic atopic dermatitis wer treated with cyclosporin A (CyA. Sandimmun® 5 mg/kg/day) for 7–16 weeks. All showed a marked clinical impovement and half could omit topical corticosteroid treatment during therapy. Adverse effects were minor, but two patients relapsed despite continued treatment. In the others, the disease recurred soon after stoppin CyA. Serum IgE levels and prick‐test responses were unchanged by CyA. Immediate and late‐phase cutaneous responses to intradermal house dust mite anitgen (HDM) were sinificantly increased during treatment; but a delayed response, present at 24 and 48 h, was unaffected. Four of six patients challenged with HDM patch tests to tape‐stripped skin during treatment showed eczematious reactions at 48 h. Thus, cyclosporin A has a powerful therapeutic effect in atopic dermatitis but does not reduce allergic resonses to inhalant antigens.

Azathioprine in Treatment of Bullous Pemphigoid

Azathioprine was given to 11 patients with pemphigoid who had been on long-term maintenance therapy with prednisone or prednisolone. In nine of these prednisone therapy was withdrawn and all were maintained symptom-free on azathioprine alone, while in two the dose of prednisone was considerably reduced. One patient who had never received corticosteroids was controlled by azathioprine alone during the initial acute phase of the illness. Since azathioprine acts slowly, it is recommended that corticosteroids should be used together with azathioprine during the acute stage. Thus azathioprine is valuable in long-term management of pemphigoid, particularly in patients showing corticosteroid toxicity or in whom the minimum maintenance dose is dangerously high.

HL-A ANTIGEN TYPE AND SMALL-INTESTINAL DISEASE IN DERMATITIS HERPETIFORMIS

Abstract Of twenty-eight patients with dermatitis Summary herpetiformis (D.H.) 68% had leucocyte antigen HL-A8 compared with a frequency of 17-30% among normal population groups. 84% of patients with associated villous atrophy (determined by small-intestinal biopsy), carried HL-A8 whereas only 33% of patients without gastrointestinal disease had HL-A8. 84% is similar to the frequency previously found for gluten-sensitive enteropathy (cœliac disease) and the frequency in patients without gut diseases does not differ significantly from normal. Thus, HL-A8 in D.H. is associated mainly with those patients who have gastrointestinal abnormalities.

The cell cycle time in the flat (avillous) mucosa of the human small intestine

A hyperproductive mucosal state in gluten-sensitive enteropathy has been proposed on the basis of an elevated mitotic index, but this parameter is dependent on the mitotic duration when used as an index of proliferative status. The mitotic duration was therefore measured in two control patients with normal villous mucosae and in two patients with the flat avillous mucosa of untreated gluten-sensitive enteropathy, using two different stathmokinetic techniques with vincristine. No significant difference in mitotic duration was found but values obtained for cell cycle time showed a halving in the flat mucosae. An increased rate of cell production in the small bowel mucosa of untreated gluten-sensitive enteropathy is thus confirmed.

A randomized double‐blind comparison of PUVA‐etretinate and PUVA‐placebo in the treatment of chronic plaque psoriasis

Twenty‐eight patients with chronic plaque psoriasis affecting 20–40% of their body surface were treated with either PUVA and placebo (thirteen patients) or PUVA and etretinate (0.75 mg/kg) (fifteen patients). PUVA was given three times a week for a maximum of 10 weeks after a 2‐week period on placebo or etretinate alone. Four patients failed to clear with PUVA and placebo compared with one patient with PUVA and etretinate, and the mean total UV‐A dose to clear was lower with etretinate (mean=62.1 J/cm2) than with placebo (mean=77.3 J/cm2) but none of these differences were significant. Because the additional response to etretinate was only marginal, whilst unwanted effects were common, we found no advantage in adding etretinate to PUVA.

Severe lichen planus clears with very low-dose cyclosporin

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Skin fragility and blistering due to use of sunbeds

6 observations de fragilisation de la peau et de formation de phlyctenes apres irradiation par ultraviolets pour bronzage