Frances Lawlor

The impact of chronic urticaria on the quality of life

The impact of chronic urticaria (CU) on the quality of life is undocumented. We assessed quality of life in patients with CU, including patients with associated delayed pressure urticaria (DPU). One hundred and forty–two out–patients completed self–administered questionnaires: a disease–specific, purpose designed questionnaire, and the Nottingham health profile (NHP). Many patients reported problems attributable to their skin condition in facets of everyday life including home management, personal care, recreation and social interaction, mobility, emotional factors, sleep, rest and work. The NHP part I scores showed restriction in the areas of mobility, sleep, energy, and demonstrated pain, social isolation and altered emotional reactions. Part II of the NHP showed that patients experienced difficulties in relation to work, looking after the home, social life, home relationships, sex life, hobbies and holidays. The patients with DPU had significantly more problems with mobility, gardening and choice of clothing than the uncomplicated CU patients. They also suffered more pain, had more problems with work and were more restricted in their hobbies.

Delayed pressure urticaria, objective evaluation of a variable disease using a dermographometer and assessment of treatment using colchicine

A randomized double‐blind placebo controlled trial of colchicine in the treatment of 13 patients with delayed pressure urticaria enabled us to assess some of the variables in this disorder. We have modified a previously described method of pressure testing using a calibrated dermograph‐ometer and shown a pressure induced papular dose response curve. Assessment of disease activity was based on the number of pressure weals which occurred, the size of delayed pressure induced papules using a dermographometer calibrated at 9.75 × 105 pascals for five separate time periods on the back and estimations of erythrocyte sedimentation rate and the acute phase protein levels. We have been unable to show a therapeutic effect using colchicine 0.5 mg bd for 1 week.

Harlequin fetus successfully treated with etretinate.

A harlequin fetus seen at birth was treated with etretinate and more general measures, including careful attention to fluid balance, calorie intake and temperature control. She improved, continued to develop, and had survived to 5 months at the time of this report.

Reticulate acropigmentation of Dohi

Two cases of reticulate acropigmentation of Dohi are reported, both patients demonstrating the typical features of this disorder.

Increased interleukin 6, but reduced interleukin 1, in delayed pressure urticaria

Interleukin 1 (IL‐1) and interleukin 6 (IL‐6) were measured by bioassays in suction‐blister exudates from lesional skin, from skin immediately following a pressure challenge, and from control skin (not subjected to pressure) of patients with delayed pressure urticaria. IL‐6 activity in lesional exudates was significantly higher than in exudates from the other two sites. IL‐1 activity in lesional exudates was not significantly higher than in the control exudates, but significantly less IL‐1 activity was found immediately after pressure challenge than from the control site.

Vibratory angioedema lesion induction clinical features laboratory and ultrastructural findings and response to therapy

We report the investigation and treatment of a 28‐year‐old woman with the rare condition of non‐familial vibratory angiodema.

Successful treatment of scleromyxoedema with high dose intravenous immunoglobulin.

We report a 53-year-old female patient with scleromyxoedema who has demonstrated and maintained a significant improvement in her condition when treated with high dose intravenous immunoglobulin (IVIG) therapy. She presented with a 1-year history of a progressive, infiltrating, papular eruption affecting her face, trunk and limbs which exhibited the Koëbner phenomenon and coalesced into indurated areas of sheeted erythema (see Fig. 1a). The presence of a monoclonal IgG paraprotein with lambda light chains in the blood and a skin biopsy which showed a marked proliferation of fibroblasts with prominent collagen and mucin deposition confirmed the diagnosis. Scleromyxoedema is a difficult condition to treat as all recommended treatment options have serious potential complications and relapse is frequent. The use of melphalan is restricted by toxicity, and cyclophosphamide, cyclosporin, photophoresis, PUVA and total skin electron beam therapy have all met with limited overall improvement. Interferon-a has resulted in deterioration in some cases, and plasma exchange is reserved for patients with central nervous system involvement. Systemic steroids have been used concurrently in many patients and their individual efficacy is difficult to determine. Autologous stem cell transplantation is described as an emerging therapy for this condition. IVIGs are used as treatment in a range of immunemediated diseases. Their efficacy has been shown in a variety of autoimmune medical and dermatological conditions, and they are a relatively safe, albeit expensive, treatment option. Our patient commenced IVIG as a firstline therapy. She received Vigam-S at a dose of 2 g ⁄ kg given as an infusion over a 5-day period on a monthly basis (0.4 g ⁄ kg ⁄ day) and noticed an improvement within 8 weeks. She has shown marked functional improvement with clinical resolution of the erythema and induration of her skin (Fig. 1b). She continues on 6-weekly IVIG infusions as maintenance therapy and her condition has currently been maintained in remission for 12 months. There are five case reports of scleromyxoedema responding successfully to IVIG. One patient showed an initial improvement in skin scores but subsequently relapsed with severe neuromuscular disease progression which failed to respond to ongoing maintenance therapy; another showed a dramatic improvement in skin scores and sustained his remission over a 10-month follow-up with ongoing 10-weekly IVIG infusions. The other three patients responded well to therapy, with one showing resolution of disease-associated encephalopathy; interestingly, while one of these patients required ongoing monthly treatment to maintain remission during a 2-year follow-up, the other two maintained remission with no ongoing therapy over a 3-year period, suggesting that maintenance therapy may not be necessary in a subset of patients. The mechanism behind the response of scleromyxoedema to IVIG is unclear. However, the ability of scleroderma and pretibial myxoedema to also respond favourably to IVIG suggests a common pathway, and there is some evidence suggesting that IVIG affects the process of matrix turnover and fibrosis through alterations in levels of matrix metalloproteinases. In summary, we feel that IVIG should be considered as both first-line treatment and maintenance therapy for scleromyxoedema. Its safety profile is favourable in Figure 1 Scleromyxoedema (a) before and (b) after treatment with high dose intravenous immunoglobulin Correspondence

Symptomatic dermographism: wealing, mast cells and histamine are decreased in the skin following long-term application of a potent topical corticosteroid.

Clobetasol propionate 0.05% ointment and an otherwise identical steroid‐free base were applied topically to a 10 cm2 area on the anterior thighs of six patients with symptomatic dermographism for 6 weeks. Four patients showed a significantly decreased wealing response to stroking of steroid pretreated skin compared to that of control sites. There was a parallel decrease in mast cell numbers and histamine levels in skin biopsies taken from the steroid treated areas. At 6 weeks two patients demonstrated a decrease in flare areas following the intradermal injection of compound 48/80 in steroid pretreated skin compared to base treated sites. Flare areas following intradermal injection of histamine in these two patients were equivalent in base and steroid treated skin.

Skin exudate levels of interleukin 6, interleukin I and other cytokines in mycosis fungoides

The role of locally released cytokines in inducing lymphocyte activation and infiltration in the skin lesions of mycosis fungoides has been investigated. The levels of selected cytokines were measured in chamber fluid samples from lesional and control skin. Biologically active interleukin 6 was significantly elevated in lesional samples and a recombinant form of this cytokine was shown to induce lymphocyte migration in an in vitro assay. Biologically active interleukin 1 was detected in all control chamber fluid samples. Significantly reduced levels of this cytokine were present in lesional samples, which may be the result of the release of preformed material. Interleukin 2 and tumour necrosis factor activity, and γ interferon and granulocyte macrophage colony‐stimulating factor immunoreactivity, were not detectable in any of the samples. Interleukins 1 and 6 may play a role in the pathogenesis of the lesional lymphocyte infiltrates in mycosis fungoides.

Arachidonic acid transformation is not stimulated in delayed pressure urticaria

Little is known about the molecular mechanisms or inflammatory mediators involved in delayed pressure urticaria (DPU). Pressure sufficient to provoke lesions was applied to the back of six patients with DPU. The levels of products of arachidonic acid transformation in skin exudate from the pressure challenged skin were estimated immediately after pressure was removed and 6 h later when lesions were present. These were compared to levels estimated in a similar way from unchallenged skin in these patients. Levels of leukotriene C4/D4/E4, prostaglandin E2, 12‐hydroxyeicosatetraenoic acid and leukotriene B4 were not raised in lesional skin. Our results suggest that arachidonic acid metabolism is not stimulated in DPU.