Summary Fifty patients with a history of symptomatic dermographism were investigated. The mean age of onset was 25·75 years (range: ‘from birth’ to 52 years) and the peak age of onset was in the second and third decades. The mean duration at last follow‐up was 5–1 years (range: 3 months to 47 years). The duration was longer than 5 years in 22%, and longer than 10 years in 10% of the patients. Ninety‐two percent of patients, but not a single member of an age‐matched group of sixty control subjects, produced measurable wealing at a pressure of 3·5 × 105 Pa or less applied with a calibrated dermographometer. The incidence of the atopic diathesis was not increased in patients with symptomatic dermographism, and no associations with systemic diseases, food allergens or medications were established‐ Routine haematological, biochemical, microbiological and intradermal screening tests were not found to be helpful in the management of this condition. Protease inhibitor profiles revealed a significant reduction in the level of circulating a i‐antitrypsin inhibitor which was not the result of a genetic difference between the patient and control groups. In a double‐blind randomized controlled trial of eight different antihistamine regimes in twelve patients, a combination of hydroxyzine (10 mg qds) and cimetidine (400 mg qds) proved significantly superior to all other treatments in reducing dermographometer‐induced wealing, and was associated with fewest side‐effects.

Plasma levels of six protease inhibitors have been measured in patients with chronic urticaria, chronic urticaria with angio-oedema, cold and cholinergic urticaria. In chronic urticaria C1 esterase inhibitor activity was increased compared with a reference control population but there was no detectable abnormality of any other protease inhibitor. Patients with chronic urticaria/angio-oedema showed a reduction in inter-alpha trypsin inhibitor. They also manifested a rise in C1 esterase inhibitor. In cold urticaria there was a significant lowering of alpha 1 antichymotrypsin. The reduction in alpha 1 antitrypsin in this group probably reflects a genetic difference compared with the control population. Patients with cholinergic urticaria also showed a reduction of alpha 1 antichymotrypsin. The elevated levels of alpha 2 macroglobulin in the three groups are probably due to differences in the mean age of these groups compared with the reference population. Comparison of levels of subgroups of patients with and without active lesions suggest that a consumptive effect may contribute to the reduced values, although it seems unlikely to account for them entirely. The results suggest that involvement of pharmacologically active products of protein digestion may be involved in the pathogenesis of urticaria and should prompt attempts to identify these agents and encourage trial of medications which lead to inhibition of proteolytic activity in urticaria.

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neuropathies is essential as recent treatment trials show a remission rate of up to 40%. Aims Compare retrospective data on clinical, investigational and treatment factors in patients who have ceased IVIg with patients who have failed a cessation trial. Methods 15 patients who successfully suspended IVIg infusions were compared with 15 in whom decreasing or stopping IVIg was unsuccessful. Results 30 patients (12 with CIDP and 3 with MMN in both groups) were diagnosed 39.5 months from onset of symptoms in the successful group vs. 40.7 months in the unsuccessful group (p=0.953). There was a significant difference in the summed upper limb sensory amplitudes on electrophysiology prior to starting IVIg between the patients with CIDP (17.4 mV vs. 9.8mV p=0.007). There was no difference in the average doses between the groups. A successful cessation trial was attempted at a mean of 60.5 months post starting treatment, compared with 60 months in the unsuccessful patients. Conclusion There is a need for objective biomarker to measure disease activity because other than one neurophysiology marker, other factors did not help predict a successful cessation trial of IVIg.