A Mixed Method Study in Young Children Participating in Clinical Research During A Kidney Transplantation Trajectory.

Introduction: Regulations designed to protect children participating in clinical research often restrict the availability of research data necessary for the development of age-specific therapies and drug dosing. Few data exist on how children experience participation in clinical research, and studies investigating young children undergoing an intensive medical treatment are lacking.

Methods: Mixed methods with semi-structured interviews and DISCO-RC questionnaires were used to explore young children's and their parents' experiences in clinical research participation during a kidney transplantation trajectory.

Initial Results of Secukinumab Drug Survival in Patients with Psoriasis: A Multicentre Daily Practice Cohort Study.

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.

Tophi as first clinical sign of gout.

Gout results from deposition of monosodium urate crystals in different body tissues, including the skin and soft tissues. With increasing prosperity a rising prevalence of the disease can be observed, especially in highly industrialized countries. Gout can present with acute and painful attacks of arthritis that can affect several joints.

Relevance of positive patch-test reactions to fragrance mix.

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.

Epidermodysplasia verruciformis: unsuccessful therapeutic approach with imiquimod.

Epidermodysplasia verruciformis (EV) is a rare heritable disease characterized by an unusual susceptibility to infection with specific types of human papillomavirus and a propensity for developing skin malignant tumors. Partial defects in cell-mediated immunity have been demonstrated in this disease. Here, we describe a patient with EV who was treated unsuccessfully with imiquimod.

Oral treatment of ichthyosis by the cytochrome P-450 inhibitor liarozole.

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.

[Keratosis palmoplantaris varians et punctata. Clinical variability of an single genetic defect?].

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.

Acquired ichthyosiform erythroderma and sarcoidosis.

We describe a patient with ichthyosiform erythroderma as a manifestation of sarcoidosis. This is the first report of the simultaneous occurrence of erythroderma and ichthyosis in sarcoidosis.

Flow-cytometric investigation of epidermal cell characteristics in monogenic disorders of keratinization and their modulation by topical calcipotriol treatment.

A flow-cytometric study was performed in monogenic disorders of keratinization, to assess DNA distribution as well as the expression of keratins and involucrin. In addition, the changes in expression of these markers under influence of calcipotriol treatment were investigated. Proliferation, measured by the percentage of epidermal cells in SG2M-phase of the cell cycle, was increased in Darier's disease, lamellar ichthyosis, congenital bullous ichthyotic erythroderma of Brocq and the Comel-Netherton syndrome, whereas normal proliferation was found in autosomal dominant ichthyosis vulgaris, X-linked recessive ichthyosis, keratosis pilaris, ichthyosis bullosa of Siemens and the Sjögren-Larsson syndrome.

Topical R-85355, a potent and selective 5-lipoxygenase inhibitor, fails to improve psoriasis.

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.

The diagnostic value of BAC for identifying alcohol-related problems among DWI offenders: another look.

The present study examines the association between breath alcohol concentration (BAC) at arrest and problem drinking for a sample of 1,283 male DWI offenders in the US Army. The results indicated a moderate but statistically significant association between BAC at arrest and DSM-III diagnosis. BAC's ability to indicate problem drinking was also compared with the diagnostic ability of three well-known, paper-and-pencil instruments designed for that purpose.

Acrokeratosis paraneoplastica (Bazex syndrome) occurring with acquired ichthyosis in Hodgkin's disease.

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.

Pachyonychia congenita tarda.

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.

Topical treatment of Sjögren-Larsson syndrome with calcipotriol.

Two patients with Sjögren-Larsson syndrome were treated with calcipotriol in ointment and the ointment base only for 12 weeks, using a double-blind bilaterally paired comparative study. Unilateral improvement was observed in both patients in favour of the calcipotriol-treated side. The present case is the first demonstration of a substantial clinical effect of calcipotriol in the Sjögren-Larsson syndrome.

Effect of topical calcipotriol on congenital ichthyoses.

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.

Widespread leucokeratosis in Noonan's syndrome.

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.

The hereditary palmoplantar keratoses: an updated review and classification.

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, 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.

The coexistence of linear and giant porokeratosis associated with Bowen's disease.

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.