Genital psoriasis: a questionnaire‐based survey on a concealed skin disease in the Netherlands

Background Psoriatic lesions may involve nearly all sites of the body. Involvement of the genital skin is frequently classified as part of intertriginous psoriasis without special awareness and treatment for this presentation of the disease. Gaining knowledge about the frequency of the involvement of genital skin in these patients will improve the overall care for patients with psoriasis. Objectives We studied the prevalence of genital psoriasis in the Netherlands and epidemiological characteristics of this specific presentation of the disease. Furthermore, we studied the relation between flexural and genital psoriasis. Patients/Methods A self‐administered questionnaire was sent to all 5300 members of the Dutch Psoriasis Society. Sociodemographic patient characteristics and disease‐related data (such as localization of psoriatic lesions, involvement of the genitalia, age at onset of genital psoriasis and severity of genital psoriatic lesions) were collected and analysed. Results A response rate of 37% was achieved. Almost 46% of the responding patients with psoriasis, that is 16.5% of all potential responders (n = 5300), report genital involvement at some time during the course of their disease. The genitalia can become affected at any age. Many patients with current genital involvement (38%) do not have the flexural skin affected. Conclusions A large part of patients with psoriasis suffer from genital psoriasis, which was not associated with flexural involvement in at least one third of them. More attention to the genital region is required in the current standard treatment of both male and female psoriatic patients at any age.     

Functional studies on c.1347C>T,a polymorphism modulating phenotypic outcome in X-linked adrenoleukodystrophy

Tijdschrift voor Kindergeneeskunde -     

Heterodimerization and functional interaction between EGF receptor family members: a new signaling paradigm with implications for breast cancer research

Summary The EGF receptor (EGFR) and HER2 are members of a growth factor receptor family. Overexpression of either protein in advanced breast cancer correlates with poor prognosis. EGF stimulates growth by binding to EGFR, activating the receptor's intracellular tyrosine kinase. The initial consequence is phosphorylation of specific tyrosine-containing sequences in the receptor's carboxyl terminus. These phosphotyrosines serve as high affinity recognition sites for proteins that, in turn, transmit the growth signal inside the cell. Mechanistic studies suggest that EGF binds to a single EGFR, triggering dimerization with another like receptor molecule. This dimerization is thought to initiate the tyrosine kinase activation.The EGF receptor family was recently expanded with the sequencing of HER3 and HER4. Each of the four family members was postulated to regulate a unique growth or differentiation signaling repertoire when activated by a receptor-specific ligand. However, new data from numerous laboratories suggest that EGFR family members may play a complex and ultimately more flexible role in signaling by forming heterodimers between family members, e.g. EGFR:HER2 or HER4:HER2. These heterodimers may form even when only one member of the pair binds its ligand.This review summarizes current work on heterodimerization and attempts to predict the consequences for downstream signaling. In brief, when compared to ligand-dependent receptor homodimers comprised of two proteins with the same internalization sequence and phosphorylated tyrosine residues, heterodimers are likely to: i) expand substrate selection and downstream signaling pathway activation; ii) promote interaction between sets of substrates in the mixed receptor complexes that would not ordinarily be physically juxtaposed; iii) alter the duration of receptor signaling by changing rates of receptor internalization, ligand loss, kinase inactivation, recycling, etc.; and iv) alter rates of receptor and substrate dephosphorylation. In addition to understanding interactions of heterodimers with the internalization machinery, identification of receptor-specific substrates and binding proteins for each EGFR family member will be necessary to explicate the role of heterodimers in growth and differentiation.     

Latanoprost-associated recurrent herpes simplex keratitis

PURPOSE/METHOD: We present a case of a patient who developed recurrent epithelial herpes simplex keratitis after starting treatment with latanoprost. Her ophthalmic history was only remarkable for a past episode of herpetic keratitis 21 years previously. RESULTS/CONCLUSIONS: Episodes of herpetic keratitis were under remission only when latanoprost was discontinued. No recurrences of herpes simplex keratitis have been observed since then. Latanoprost usage might be associated with recurrent episodes of herpes simplex keratitis in patients with previous history of ocular herpes simplex virus infection.     

Peripheral multineuritis pointing at systemic sarcoidosis

We report a patient with a peripheral neuropathy as the first symptom of sarcoidosis. The systemic illness was proved by the presence of typic granulomes in the bone marrow. The fact that sarcoidosis is the cause for the neuropathy is supported by the temporary relation and by the good response of all clinical picture to the corticosteroid therapy.Sarcoid neuropathy can rarely be the presenting feature of sarcoidosis.     

山苍子水提物柠檬醛抗哮喘作用的实验研究

目的：研究山苍子水提物柠檬醛的抗哮喘作用及其机理。方法：通过豚鼠氯化乙酰胆碱-磷酸组胺喷雾致喘法、小鼠浓氨水喷雾引咳法、小鼠酚红排泻法观察柠檬醛的平喘、镇咳、祛痰作用，通过观察张力变化研究柠檬醛对豚鼠离体气管环张力的影响及乙酰胆碱对气管收缩的抑制作用。结果：柠檬醛能明显延长氯化乙酰胆碱-磷酸组胺喷雾引起的豚鼠哮喘潜伏期，能延长浓氨水喷雾诱发小鼠咳嗽反应潜伏期，明显减少咳嗽次数。能增加小鼠呼吸道酚红排泌量，能抑制乙酰胆碱对豚鼠离体气管平滑肌的收缩作用，使乙酰胆碱所致气管平滑肌量效曲线右移。结论：柠檬醛气雾剂具有一定的平喘、镇咳和祛痰作用，具有良好的支气管解痉作用。