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Interleukin 17-antagonist secukinumab demonstrated high efficacy for treatment of psoriasis in randomized controlled trials. However, performance in daily practice may differ from trials. Drug survival is a comprehensive outcome covering effectiveness and safety, suitable for analyses of daily practice. The aim of this study was to evaluate drug survival of secukinumab in a daily practice psoriasis cohort. Data were collected from 13 hospitals. Drug survival was analysed using Kaplan-Meier survival curves, split for reason of discontinuation. In total, 196 patients were included (83% biologic experienced). Overall, 12 and 18 months drug survival of secukinumab was 76% and 67%, respectively, and was mostly determined by ineffectiveness. There was a trend towards shorter drug survival in women and in biologic experienced patients. Thirteen percent of patients experienced at least one episode of fungal infection. This is one of the first studies of drug survival of secukinumab in patients with psoriasis treated in daily practice.

Summary We investigated the clinical efficacy of topically applied calcipotriol in six patients with congenital ichthyosis, using a double-blind, bilaterally paired, comparative approach. Unilateral improvement, in favour of the calcipotriol-treated side, was observed in three patients with lamellar ichthyosis. A beneficial response was also observed in a patient with bullous ichthyotic erythroderma of Brocq. No clinical side-effects or laboratory anomalies were observed. This study indicates that calcipotriol constitutes a new and promising approach in alleviating disorders of keratinization characterized by hyperproliferation, other than psoriasis.

Liarozole, a novel imidazole derivative, inhibits the cytochrome P450-dependent 4-hydroxylation of retinoic acid, resulting in increased tissue levels of retinoic acid. Twelve male patients with ichthyosis were given oral liarozole, 150 mg twice daily, in an open study for 12 weeks. Immunohistochemical parameters of inflammation, epidermal proliferation and differentiation were assessed before and after treatment. Extent and severity of the skin lesions was markedly reduced in all patients. Clinical side-effects were reminiscent of those with synthetic retinoids. No relevant changes were found in the haematological, urinary and biochemical parameters. Immunohistochemical assessment showed a statistically significant induction of keratin 4 after liarozole treatment in 10 of 12 patients. In two of these patients keratin 13 was induced. This open study showed that oral liarozole treatment was efficacious and well tolerated in the treatment of different types of ichthyosis. The immunohistochemical results suggest a retinoid mechanism as the mode of action.

Pachyonychia congenita is a distinct hereditary disorder of keratinization, in which dystrophy of all nails is associated with palmoplantar keratoderma and other hyperkeratoses. Recently a late-onset type has been reported. We report a second family with late-onset pachyonychia congenita, showing a remarkable clinical heterogeneity. Furthermore, one patient demonstrated a number of associated hyperkeratoses not previously recognized. Acitretin proved useful in the treatment of this late-onset form of pachyonychia congenita.

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SUMMARY The palmoplantar keratoses (PPKs) comprise a heterogeneous group of disorders of keratinization, which can be subdivided into hereditary and acquired forms. Many authors have attempted to classify the hereditary forms,1–5 and most classifications have been based on the morphology, distribution, associated symptoms and mode of inheritance. Subsequently, many new forms have been recognized, and what were previously considered to be distinct types have been shown to be variants of a single type, both of which limit the usefulness of previous classifications. Hence, we propose a new, updated classification, which enables accurate diagnosis of these disorders.

In the period 1987-1990, 999 patients suspected of a contact allergy were patch-tested with Kathon CG 100 ppm active ingredients aq. Of these patients, 84 (8.4%) showed a positive reaction (70% females). In 62% of these cases clinical relevance was established. Most of them suffered from contact dermatitis of the face, the hands or the perianal region.

BACKGROUND: Fragrances are an important cause of allergic contact dermatitis. We presume that the traditional fragrance mix (FM) detects 70 to 80% of fragrance-allergic patients. FM has an irritant potential. Weak positive reactions may have a greater chance of being irrelevant than strong reactions.OBJECTIVE: To improve the appraisal of FM patch-test reactions, we studied the relevance of reactions of different strength. We also studied the predictive value of the following on the relevance of the initial FM patch-test results: patch-test results of a repeated FM test; results of patch tests with balsam of Peru, colophony, and ingredients of the mix; and (history of) atopic dermatitis.METHODS: One hundred thirty-eight patients who had doubtful positive (?+) or positive (+ to +++) reactions were included in the study. We determined relevance by history taking, location and course of the dermatitis, and additional patch testing. Patients were retested with FM and with each ingredient separately.RESULTS: The relevance of reactions to FM increases with the strength of the reactions. Predictors of relevance are the results of retesting with FM, the results of tests with the ingredients, and a history and/or present symptoms of atopic dermatitis.CONCLUSION: Retesting with FM and its ingredients may add to the benefit of patch testing.

In a prospective double-blind left-right comparative study, a cream containing 13-cis-retinoic acid 0.1% (13-cis-RA) and its cream base were applied to a test patch of skin of seven patients with Darier's disease for periods ranging from 3 to 10 weeks. A half-side reduction in papules was observed in four patients. The treatment had no effect on scaling and increased erythema in two patients. Clinical improvement of papules was statistically significant, whereas scaling and erythema did not change significantly. Side-effects of the study medication were only minimal and easily controlled by reduction of the application frequency. Biopsies for immunohistochemical examination were taken from representative skin lesions, one before and one from each side after treatment. Keratins 4 and 13 were induced by the treatment with topical 13-cis-RA in three and two patients, respectively. In conclusion, topical 13-cis-RA 0.1% cream is a well-tolerated therapy for Darier's disease. Induction of keratins 4 and 13 seem...

Inhibitors of 5-lipoxygenase have been studied with respect to antipsoriatic efficacy. Of these compounds, R-68151 is of particular interest as it proved to inhibit 5-lipoxygenase without inhibiting 12-lipoxygenase, cyclooxygenase and thromboxane-A2 synthetase. R-68151 has been shown to have a mild-to-moderate therapeutic effect in psoriasis. In the present study a new 5-lipoxygenase, R-85355, was investigated with respect to its efficacy in psoriasis. R-85355 is 3 times more potent compared to R-68151 with respect to inhibition of in vitro A-23187-stimulated leukotriene-B4 production by polymorpho-nuclear leukocytes. In a left-right double-blind comparative study, the compound was studied at saturation with respect to its antipsoriatic efficacy in 11 patients with chronic stable plaque psoriasis. In addition, various markers for epidermal proliferation, keratinization and inflammation were assessed. In no single patient was a left-right difference observed in favour of R-85355 compared to placebo with respect to clinical severity scores or the cell-biological markers. The present study indicates that a selective and highly potent 5-lipoxygenase inhibitor was not effective in the topical treatment of chronic plaque psoriasis.

Vitamin D3 analogues interfere with various aspects of cutaneous inflammation, keratinization and epidermal proliferation, and are effective in chronic plaque psoriasis. Recently their clinical efficacy has been investigated in other skin disorders. So far, the vitamin D3 analogue calcipotriol has proved to be effective in pustular psoriasis, congenital ichthyosis, inflammatory linear verrucous epidermal naevus, Graver's disease and pityriasis rubra pilaris. Information on the effect of other vitamin D3 analogues in these conditions is limited.

We report a patient who exhibited two paraneoplastic skin disorders, acrokeratosis paraneoplastica and acquired ichthyosis, in association with Hodgkin disease. Treatment of the Hodgkin’s disease resulted in rapid improvement of both skin disorders, confirming a causal relationship. The simultaneous presence of two paraneoplastic skin disorders suggests a common pathogenetic factor. Transforming growth factor a, produced by tumour cells, may play a part in the aetiopathogenesis of hyperproliferative paraneoplastic skin conditions. Acrokeratosis paraneoplastica was first described by Bazex in 1965,1 and is a rare but distinctive paraneo­ plastic dermatosis. It is associated with malignancies of the upper respiratory or digestive tract, or with cervical or mediastinal lymph node metastases from distant occult neoplasms. Clinically, the syndrome evolves in three stages. The first stage is characterized by erythema and psoriasiform scaling on the fingers and toes, which soon spreads to the bridge of the nose and to the helices of the ears. Nail changes are frequent. In the second stage, a violaceous keratoderma of the palms and soles develops, and the facial lesions spread to the pinnae and cheeks. In the third stage, the eruption extends locally and begins to involve the legs, knees, thighs, arms, trunk and scalp. The dermatosis may antedate the diagnosis of malignancy or appear after its identification. In the most typical cases, the neo­ plasm begins to produce its first symptoms when the skin lesions have progressed to the second stage. Almost all the tumours associated with acrokeratosis paraneoplastica are squamous cell carcinomas. Excep­ tions include adenocarcinomas of the lung,6 oesopha­ gus,7 stomach8 and uterus and anaplastic small cell

Summary A case of Noonan's syndrome is presented. In addition to the typical manifestations of the disease, widespread leucokeratosis of the lips and gingiva appeared to be a prominent feature.

Summary We report a patient with lichen nitidus of the palms of the hands and the toes, whose skin lesions responded to treatment with acitretin.

Linear and giant porokeratosis are both rare variants of this disorder of keratinization. We present a case exhibiting the clinical features of both variants coexisting in one patient, which to our knowledge has not been described previously. Furthermore, Bowen's disease was found to be present in the giant lesion, reflecting the enhanced risk of malignant transformation of this rare subtype of porokeratosis.

Palmoplantar keratodermia (PPK) varians and PPK punctata are considered different entities within the group of hereditary palmoplantar keratodermas. Keratosis punctata of the palmar creases constitutes a localized form of keratodermia palmoplantaris punctata. We describe a 31-year-old man exhibiting both nummular and papular keratoses on the soles, as well as small punctate keratoses confined to the palmar creases. In view of the co-occurrence of these two types of PPK, the question arises whether keratosis palmoplantaris of the varians and the punctate type represent distinct entities or represent variable manifestations of the same gene defect.