Rituximab in diffuse cutaneous systemic sclerosis: an open-label clinical and histopathological study.

OBJECTIVES: The safety and potential efficacy of rituximab was examined in diffuse cutaneous systemic sclerosis (dc-SSc). METHODS: A 24 week open-label study in which eight patients with dc-SSc received an infusion of 1000 mg rituximab administered at baseline and day 15, together with 100 mg methylprednisolone at each infusion. Assessment included CD19+ peripheral blood lymphocyte number, skin sclerosis score, indices of internal organ functioning, the health assessment questionnaire disability index, the 36-item Short Form health survey and histopathological evaluation of the skin. RESULTS: Ritixumab induced effective B-cell depletion in all patients (<5 CD19+ cells/microl blood). There was a significant change in skin score at week 24 (p<0.001). Also, significant improvements were measured in the dermal hyalinised collagen content (p = 0.014) and dermal myofibroblast numbers (p = 0.011). Two serious adverse events occurred, which were thought to be unrelated to the rituximab treatment. CONCLUSIONS: Rituximab appears to be well tolerated and may have potential efficacy for skin disease in dc-SSc.

Incidence of scabies in Belgium.

A prospective survey on scabies in Ghent, Belgium was performed in 2004. Sixty-four individual cases were reported, corresponding to a crude incidence rate of 28/100,000 inhabitants. The incidence was higher in the elderly (51/100,000 in persons aged >75 years) and a higher incidence was also found in immigrants (88/100,000). More than 40% of the registered scabies patients had symptoms for more than 4 weeks at the time of presentation. In 54% of the consultations, the patient had already consulted a physician for his/her skin problem. Of this group, 44% had not yet received any scabicidal treatment, indicating that scabies was not yet diagnosed or that an inappropriate treatment was prescribed. The observations suggest that the diagnosis and/or treatment of scabies in this region can still be improved.

Recommendations for the use of etanercept in psoriasis: a European dermatology expert group consensus.

BACKGROUND: Psoriasis is a chronic, inflammatory skin disorder that has a significant impact on quality of life and, particularly in moderate to severe cases, adversely affects the patient's overall health and well-being. Biological treatments, such as etanercept, are being widely adopted across Europe for treatment of moderate to severe psoriasis due to favourable safety and efficacy profiles. The increase in usage, combined with a growing body of clinical evidence, has identified a need to clarify the best use of etanercept within its current treatment label. OBJECTIVE: To prepare a series of recommendations agreed by an expert group of dermatologists, relating to the most effective use of etanercept for psoriasis in Europe, within the product license. METHODS: An expert panel of dermatologists from across Europe completed a Delphi survey to address the current use of etanercept in psoriasis in Europe. In June 2005 the results were presented to the expert panel at their nominal group meeting, and a consensus was agreed. RESULTS: It was recommended that, where possible, patients are initiated on the 50 mg twice-weekly (BIW) dose. Etanercept should be given until remission is achieved (maximum 24 weeks) and retreatment should be initiated according to the physician's judgement. Before commencing treatment, contraindications, such as infection or previous malignancy (within 5 years), should be ruled out. CONCLUSIONS: The consensus presented herein provides valuable clarification of use of etanercept according to the label, which may have wider implications relating to the use of all biological therapies in psoriasis.

Psychosocial effects of vitiligo.

A limited number of studies have paid attention to the psychosocial well-being of patients affected with vitiligo. We review the psychosocial effects of vitiligo, how patients deal with them and the psychiatric morbidity in vitiligo patients. Given the appreciable comorbidity, it is important to consider these observations in the management of patients, for example in patient-physician interaction but also in treatment strategies and evaluation of treatments. The effects of the psychological state on the disease itself together with the potential therapeutic implications are reviewed. Based on these data, we suggest how to further improve patient's management.

Clinical and molecular abnormalities in lipoid proteinosis.

Lipoid proteinosis (hyalinosis cutis et mucosae) is a rare, autosomal recessive disease. The main clinicopathological features comprise skin and mucous membrane infiltration and scarring with deposition of hyaline material. In this report, we describe a 6-year-old boy in whom a diagnosis of lipoid proteinosis was first suspected when he presented with blisters and erosions at 4 years, a history of life-long dysphonia and a previous epileptic convulsion. The diagnosis was confirmed by histology and identification of a homozygous frameshift mutation, 501insC, in exon 6 of the gene encoding extracellular matrix protein 1, ECM1. Lipoid proteinosis may show protean clinical features and be difficult to diagnose on clinical grounds alone. This case report illustrates that lipoid proteinosis may show protean clinical features and yet remain undiagnosed for many years. Although the gold standard for definite diagnosis remains histology, molecular characterisation of the gene mutation will add to our understanding of genotype-phenotype correlation and perhaps to the development of a rationale for future therapeutics.

Effect of vitiligo on self-reported health-related quality of life.

BACKGROUND: Vitiligo is a pigmentary disorder affecting at least 1% of the total population. Although the disease does not produce direct physical impairment, it may considerably influence the psychological well-being of the patients. It has been suggested that patients suffer from low self-esteem, poor body image and a poor quality of life. The majority of the studies on the psychosocial impact of vitiligo were conducted in the U.S.A. and England. OBJECTIVES: This study aims to quantify the burden of vitiligo by estimating health-related quality of life in the Dutch-speaking Belgian population (Flanders). This is compared with the level of disability caused by psoriasis in a similarly recruited population. It is also our purpose to detect those at risk of experiencing a poor quality of life and to identify variables that might predict this impairment. PATIENTS AND METHODS: Patients with vitiligo (n = 119) and 162 patients with psoriasis were included in a postal survey. In order to obtain a patient-based measurement of quality of life we used the Dermatology Life Quality Index (DLQI), a widely validated questionnaire that is easy to use and allows comparison between several skin disorders. Other survey questions were related to demographic data and disease-related characteristics. RESULTS: We obtained excellent response rates in both patient groups. The overall mean DLQI score for vitiligo (4.95) was lower than that for psoriasis (6.26) (P = 0.01). Patients with vitiligo experienced significantly less impairment of life quality from the symptoms and treatment of the disease (P < 0.001). The highest individual mean scores in vitiligo were found for Q2 (feelings), Q4 (clothing), Q5 (social and leisure activities) and Q3 (daily routine). The interaction of disease and sex on the DLQI score was highly significant (P = 0.001). While men with vitiligo scored significantly lower than men with psoriasis (P < 0.001), we found a comparable overall DLQI score for women in these disease groups. The number of consultations (P = 0.005) and severity of the disease (P < 0.001) were independently related to the DLQI. CONCLUSIONS: Our study quantifies the burden on the quality of life caused by vitiligo and indicates specific areas of patients' lives which are most affected by the disease. Sex, number of consultations and subjective disease severity independently predict the quality of life. The quality of life impairment in women affected with vitiligo equals the impairment caused by psoriasis in our study population. These results should awake the interest of physicians in this 'cosmetic' disease, since appropriate treatment is likely to improve the quality of life of vitiligo patients.

Quality of life and stigmatization profile in a cohort of vitiligo patients and effect of the use of camouflage.

BACKGROUND: Few studies have paid attention to the effects of treatment interventions on the psychosocial consequences of vitiligo. OBJECTIVES: To quantify and analyse the psychosocial benefit of the use of camouflage in vitiligo patients. PATIENTS AND METHODS: 78 vitiligo patients completed the Dermatology Life Quality Index (DLQI) and an adapted stigmatization questionnaire, and 62 of them completed the DLQI after at least a 1-month use of camouflage. RESULTS: The initial mean overall DLQI score (n = 78) is 6.9 (SD 5.6). The mean global stigmatization score is 38%. Disease extent and disease severity are strong predictors of the DLQI (p < 0.0001). Vitiligo on the face/head/neck substantially affects the DLQI, independently of degree of involvement. The mean DLQI score before and after use of camouflage (n = 62) is 7.3 (SD 5.6) and 5.9 (SD 5.2; p = 0.006). Mainly the high-scoring items 'feelings of embarrassment and self consciousness' and 'choice of clothing' improve. Predictors of improvement are higher DLQI scores (p = 0.0005) and higher total severity scores (p = 0.03). CONCLUSIONS: Camouflage can be recommended, particularly in patients with higher DLQI scores or self-assessed disease severity. Patients with minor involvement of the face benefit from camouflage.

Screening for contact allergy to artificial nails.

27 patients (26 women and 1 man), all in contact with artificial nails, were tested for acrylic compounds, known to be present in nail cosmetics. The patch test results obtained in these patients demonstrated that 2-hydroxyethyl methacrylate and ethyl cyanoacrylate were the only 2 allergens needed to diagnose contact allergy to acrylic-containing nail cosmetics, except for 1 patient who reacted only to her nail preparation used and in which we were unable to identify the allergen. This concerns clearly a limited number of patients. The purpose was, however, to detect the most valuable allergen(s) to be used in a dermatologist's practice, in which not all possible allergens can be tested, in order to diagnose such cases.

Ciclosporin in psoriasis clinical practice: an international consensus statement.

The main recommendations for the use of ciclosporin in the management of psoriasis are: (i) intermittent short courses (average of 12 weeks duration) of ciclosporin are preferable; (ii) ciclosporin should be given in the dose range 2.5-5.0 mg kg(-1) day(-1) (doses greater than 5.0 mg kg(-1) day(-1) should only be given in exceptional circumstances); (iii) treatment regimens should be tailored to the needs of each patient; (iv) selection of patients should take into account psychosocial disability, as well as clinical extent of disease and failure of previous treatment; (v) each patient's renal function (as measured by serum creatinine) should be thoroughly assessed before and during treatment; (vi) each patient's blood pressure should be carefully monitored before and during treatment; (vii) adherence to treatment guidelines substantially reduces the risk of adverse events; (viii) long-term continuous ciclosporin therapy may be appropriate in a subgroup of patients; however, duration of treatment should be kept below 2 years whenever possible; and (ix) when long-term continuous ciclosporin therapy is necessary, annual evaluation of glomerular filtration rate may be useful to accurately monitor renal function.

Topical class I corticosteroids in 10 patients with bullous pemphigoid: correlation of the outcome with the severity degree of the disease and review of the literature.

BACKGROUND: Treatment of bullous pemphigoid (BP) with systemic immunosuppressive agents, in particular with systemic corticosteroids, has many long-term side-effects. A dozen reports were published regarding the efficacy of topical corticosteroids in the treatment of bullous pemphigoid. OBJECTIVE: To evaluate the efficacy of potent class I topical corticosteroids in relation to the affected body surface area (BSA) in patients with bullous pemphigoid and to review the literature. METHODS: An open prospective trial with 10 patients with BP with measurement of the affected BSA. Treatment protocol consisted of three steps: potent class I topical corticosteroid treatment, systemic tetracyclines and systemic corticosteroids. Follow-up period was between 24 and 72 months. RESULTS: Our study suggests a correlation between the success rate of topical corticosteroid treatment and the body surface area initially affected: all patients with an affected BSA of less than 20% healed with topical treatment only. The patients with more than 40% affected BSA needed systemic treatment with steroids. CONCLUSION: Topical class I corticosteroids seem to be effective in healing lesions of BP, especially if less than 20% of the BSA is affected. This study comprises only 10 patients, making further studies necessary to draw definite conclusions.

Autologous transplantation techniques for vitiligo: how to evaluate treatment outcome.

Effective methods for measuring treatment outcome in vitiligo are essential to accurately assess possible therapeutic modalities. This systematic review article aims to bring the problems concerning evaluation of treatment outcome in vitiligo studies using transplantation techniques to the attention of clinical investigators. Furthermore we highlight the interpretation of the achieved result from both physicians' and patients' view point using a questionnaire put to 558 dermatologists and 152 vitiligo patients in Belgium. There is no consensus about the choice of an evaluation method in surgical vitiligo studies. The interpretation of a 'successful' treatment result seemed to differ among dermatologists and vitiligo patients. We conclude that further research is needed to develop a universally accepted, objective, reliable and useful measurement method to evaluate the efficacy of surgical vitiligo treatments. A combination of both a clinical and a psychological measurement is likely to be the most appropriate choice.

Early treatment of hemangiomas with lasers. A review.

Hemangiomas can present a difficult problem in management. Much controversy exists over whether it is better to watch and wait for natural involution or to be more aggressive and attempt to prevent some of the potential negative sequelae. Different modalities have been employed in the treatment of hemangiomas, including systemic therapy - antiangiogenic drugs, i.e. systemic corticosteroids and alpha-interferon - and local therapy - surgical procedures, arterial embolization and cryotherapy. Overall, a very conservative approach to therapy has been recommended because of treatment risks, treatment inadequacy and lack of evidence showing superiority over natural involution. Recently laser treatment has been used in the therapeutic approach of hemangiomas. This review discusses the pros and contras of early laser treatment of hemangiomas. For superficial hemangiomas, the flashlamp pumped pulsed dye laser in particular has proven itself in numerous studies. In the treatment of hemangiomas with subcutaneous components, the Nd:YAG laser is the treatment of choice.

Adverse skin reactions to anti-TNF-alpha monoclonal antibody therapy.

Various adverse cutaneous reactions to anti-TNF-alpha monoclonal antibody have been reported. In clinical studies with infliximab (Remicade) adverse drug reactions were most frequently reported in the respiratory system and in the skin and appendages. We describe here 6 patients receiving anti- TNF-alpha therapy (infliximab) for Crohn's disease or rheumatoid arthritis who consulted our out-patient department for adverse cutaneous reactions between November 1999 and February 2002. The following diagnoses were made: leukocytoclastic vasculitis, lichenoid drug reaction, perniosis-like eruption (2 patients), superficial granuloma annulare and acute folliculitis.

[Motor proteins and pigmentation]. / Motoreiwitten en pigmentatie.

One essential part of the process of skin pigmentation comprises the production of melanosomes, the melanin-containing organelles, and correct transport towards their target cells, the keratinocytes. In this overview the molecular mechanisms of these processes are discussed in view of a number of pigmentation syndromes.

Efficacy and safety of a topically applied Avène spring water spray in the healing of facial skin after laser resurfacing.

BACKGROUND: Although laser resurfacing is a well-accepted treatment, tolerance problems may preclude patient's acceptance of the procedure. Post-laser protracted erythema is a prime problem, associated with the symptoms of pain, itching, stinging and tightening. OBJECTIVE: The present study was conducted to evaluate a low-salt medical spring water (Avène, Avène-les-Bains, France), in conjunction with the standard use of an emollient, to improve tolerance by hydrating the postlaser skin. METHODS: A controlled, randomized, open-labeled, multicentric and two parallel group study was completed after 84 days in 74 patients, 34 applying a petrolatum ointment alone (control group), and 40 applying petrolatum ointment in association with the Avène spring water spray ad libitum (water group). RESULTS: The water spray showed a pronounced effect on reducing erythema, from the second week of postlaser onwards, with a statistically significant difference in favor of the water group compared with the control group (p < or = 0.04 from day 14 to day 84, except a non-significant trend on day 28). In addition, the water spray was found to significantly reduce itching on day 28 (p < 0.05), and stinging and tightening on day 14 and day 21 (p < 0.05). Pain was not modified by the water. CONCLUSION: Topical water appears to be a helpful adjunctive treatment to petrolatum ointment for counteracting erythema, itching, stinging and tightening during the postlaser treatment period.

Microcystic adnexal carcinoma: an uncommon tumor with debatable origin.

BACKGROUND: Microcystic adnexal carcinoma is an uncommon skin appendage neoplasm exhibiting both pilar and sweat duct differentiation. This tumor remains a subject of controversy as to its differentiation profile, histogenesis, and classification which is reflected in the nomenclature used to designate the neoplasm in question. Beyond this controversy the tumor remains a diagnostic challenge because of its rarity, the histologic mimicry it may display, and its banal cytologic appearance; it also poses a therapeutic challenge, as it is characterized by slow but aggressive and destructive local growth extending beyond clinical margins together with a high tendency for perineural invasion and recurrence. OBJECTIVE: We report two cases of this unusual tumor illustrating some of its characteristics. Our review emphasizes the divergent opinions concerning its differentiation profile and its origin. An organoid nevus as the origin of microcystic adnexal carcinoma in one of our patients is discussed in this context.

CD-34 and Ki-67 staining patterns of basaloid follicular hamartoma are different from those in fibroepithelioma of Pinkus and other variants of basal cell carcinoma.

BACKGROUND AND AIMS: Basaloid follicular hamartoma is a rare disorder regarded as a developmental malformation. It may be solitary or generalized, linear or regionalized, and is sometimes associated with myasthenia gravis or alopecia. We compared immunohistochemical staining patterns of selected markers in order to differentiate this hamartoma from fibroepithelioma of Pinkus, a basal cell carcinoma variant it can be confused with. METHODS: The expression of three immunohistochemical markers--CD-34, Ki-67, bcl-2--was studied in a basaloid follicular hamartoma and in a fibroepithelioma of Pinkus. Two basal cell carcinomas, a nodular and a fibrosing type, and a trichoepithelioma were included as controls. RESULTS: Basaloid follicular hamartoma shows a low proliferation index and an at least focally circumferential expression of CD-34 around the epithelial strands. This compares to the findings in trichoepithelioma. In contrast, fibroepithelial tumor of Pinkus and two other basal cell carcinoma subtypes display a high proliferative index and an absence of CD-34 expression around the epithelium. These findings support the non-neoplastic nature of basaloid follicular hamartoma.