Consensus statement on the diagnosis and treatment of sclerosing diseases of the skin, Part 2: Scleromyxoedema and scleroedema.

The term 'sclerosing diseases of the skin' comprises specific dermatological entities, which have fibrotic changes of the skin in common. These diseases mostly manifest in different clinical subtypes according to cutaneous and extracutaneous involvement and can sometimes be difficult to distinguish from each other. The present consensus provides an update to the 2017 European Dermatology Forum Guidelines, focusing on characteristic clinical and histopathological features, diagnostic scores and the serum autoantibodies most useful for differential diagnosis. In addition, updated strategies for the first- and advanced-line therapy of sclerosing skin diseases are addressed in detail. Part 2 of this consensus provides clinicians with an overview of the diagnosis and treatment of scleromyxoedema and scleroedema (of Buschke).

Interpretation of the skin cancer quality of life score: a validated quality of life questionnaire for non-melanoma skin cancer.

BACKGROUND: Health-related quality of life (HRQoL) instruments are used increasingly. In order to assign clinical meaning to HRQoL scores, the interpretation of instruments is essential but lagging in dermatology. OBJECTIVE: To establish a clinical interpretation of the Skin Cancer Quality of Life questionnaire (SCQoL), a newly validated HRQoL instrument for patients with non-melanoma skin cancer (NMSC), using an anchor-based method, and to test the responsiveness. METHODS: Receiver-operating characteristic analysis was used to propose clinically meaningful cut-off scores for SCQoL including 101 patients with NMSC. RESULTS: The following bands were established: score 0-3 corresponds to no impairment, 4-6 corresponds to mild impairment, 7-10 to moderate impairment and 11-27 to severe impairment of HRQoL. Testing the responsiveness shows a moderate effect size and significantly lower scores only for the domain emotion and the global item. CONCLUSION: Using proposed clinical cut-off scores for SCQoL may help clinicians in their decision-making, help monitoring clinical improvement and classify patients just as e.g. the Dermatology Life Quality Index can.

Abatacept is a promising treatment for patients with disseminated morphea profunda: presentation of two cases.

Morphea profunda is a rare disease that mainly affects young women and often has a progressive course with physical and psychological sequelae. The skin becomes sclerotic after an initial inflammatory reaction and joint contractures can develop. The aetiology is unknown. Until now, no successful therapy has been proven for this morphea variant. On the basis of new insights into the key role of effector T cells in scleroderma, in particular Th-17, T-cell directed therapies are expected to have promising effects. We report here the first two cases of morphea profunda treated with abatacept. Abatacept had a clinical effect on the active disease, in addition to softening old sclerotic lesions.

Nephrogenic systemic fibrosis in Denmark--a nationwide investigation.

BACKGROUND: Nephrogenic systemic fibrosis is a debilitating and painful disorder with an increased stimulation of the connective tissue in the skin and systemic tissues. The disease is associated with exposure to gadolinium-based contrast agent used in magnetic resonance imaging in patients with renal impairment. METHODS: The prevalence of nephrogenic systemic fibrosis has so far never been determined at a national level. In 2009, Denmark was the first country to design a guideline for the tracing of nephrogenic systemic fibrosis patients. The aim of this paper is to communicate the main findings of this quest. RESULTS: The outcome of the nationwide investigation revealed that Denmark had 65 patients with nephrogenic systemic fibrosis and thereby the highest prevalence of nephrogenic systemic fibrosis worldwide with 65 per 5.6 million inhabitants, or 12 per million. CONCLUSIONS: The nationwide investigation in Denmark revealed the highest prevalence of NSF worldwide. This may be rooted in a high level of awareness of NSF both among doctors, politicians and, not least, the media, combined with the fact that a nationwide NSF investigation was initiated.

Atopic dermatitis and risk of skin cancer: a Danish nationwide cohort study (1977-2006).

BACKGROUND: Recent data suggest a reduced risk of malignant melanoma (MM) among atopic dermatitis (AD) patients, but an increased risk of other skin cancers (including basal cell carcinoma [BCC] and squamous cell carcinoma [SCC]). OBJECTIVE: We examined the association between AD and skin cancers in a large cohort study in Denmark from 1977 through 2006. METHODS: Our cohort consisted of 31 330 AD patients recorded in the Danish National Patient Registry, including AD patients admitted to hospitals and specialized outpatient clinics. Linkage to the Danish Cancer Registry allowed ascertainment of skin cancers. We calculated standardized incidence ratios (SIRs) and associated 95% confidence intervals (CIs) by comparing the incidence rate of skin cancers among AD patients with that among the general Danish population. RESULTS: The overall observed number of MM cases among AD patients was 12, with 21 expected, yielding a SIR of 0.59 (95% CI 0.30, 1.02), with the most pronounced protective effect among AD patients with more than 5 years of follow-up (SIR = 0.46; 95% CI 0.19, 0.95). The corresponding SIRs for BCC and SCC were increased among AD patients (1.41 [95% CI 1.07, 1.83] and 2.48 [95% CI 1.00, 5.11], respectively). CONCLUSIONS: Our findings support an inverse association between AD and MM, but an increased risk of BCC and SCC among AD patients.

Intake of alcohol may modify the risk for non-melanoma skin cancer: results of a large Danish prospective cohort study.

Alcohol has not been linked definitively to non-melanoma skin cancer. We examined whether alcohol intake affects the risks for basal cell carcinoma (BCC) and squamous cell carcinoma (SCC) using data on 54,766 persons enrolled in the prospective Diet, Cancer, and Health cohort. Statistical analyses were based on the Cox proportional hazards model. All hazard ratios (HRs) were multivariate adjusted. Adjustment for exposure to UVR was not possible, but all analyses were adjusted for factors related to susceptibility to UVR, including sun sensitivity, degree of freckling, and number of nevi. A total of 2,409 BCC cases and 198 SCC cases were diagnosed within a median follow-up of 11.4 years. Total current alcohol intake was not associated with BCC risk, but beverage-specific analyses showed an increased BCC risk associated with intake of wine (HR=1.05, 95% confidence interval (CI): 1.02-1.08, current average alcohol intake, per 10 g per day) and spirits (HR=1.11, 95% CI: 1.02-1.21) and a decreased risk with beer (HR=0.97, 95% CI: 0.93-1.00). No convincing associations were found between total alcohol intake and risk for SCC, perhaps because of the limited number of cases. Our findings indicate that alcohol intake may increase the risk for BCC, but the relations seemed to depend on beverage type.

Registration in the Danish Regional Nonmelanoma Skin Cancer Dermatology Database: completeness of registration and accuracy of key variables.

OBJECTIVE: To validate a clinical database for nonmelanoma skin cancer (NMSC) with the aim of monitoring and predicting the prognosis of NMSC treated by dermatologists in clinics in the central and north Denmark regions. METHODS: We assessed the completeness of registration of patients and follow-up visits, and positive predictive value (PPV), negative predictive value (NPV), sensitivity, and specificity of registrations in the database. We used the Danish Pathology Registry (DPR) (n = 288) and a review of randomly selected medical records (n = 67) from two clinics as gold standards. RESULTS: The completeness of registration of patients was 62% and 76% with DPR and medical record review as gold standards, respectively. The completeness of registration of 1st and 2nd follow up visits was 85% and 69%, respectively. The PPV and NPV ranged from 85% to 99%, and the sensitivity and specificity from 67% to 100%. CONCLUSION: Overall, the accuracy of variables registered in the NMSC database was satisfactory but completeness of patient registration and follow-up visits were modest. The NMSC database is a potentially valuable tool for monitoring and facilitating improvement of NMSC treatment in dermatology clinics. However, there is still room for improvement of registration of both patients and their follow-up visits.

Chronic diseases requiring hospitalization and risk of non-melanoma skin cancers--a population based study from Denmark.

We examined the associations between chronic diseases requiring hospitalization and the risk of non-melanoma skin cancers (NMSCs) in a population-based case-control study of 4,187 patients diagnosed with a first primary NMSC in 1995 in Denmark. From the National Patient Registry covering all Danish hospitals, we obtained data on hospitalizations with chronic diseases, recorded before the date of NMSC diagnosis. Using incidence density sampling, we selected 10 age-, gender-, and residence-matched controls from the Danish Civil Registration System. We used conditional logistic regression to compute incidence rate ratios (IRRs) and 95% confidence intervals (CIs). Although no overall association was found between basal cell carcinoma (BCC) and hospitalization for chronic diseases, an elevated IRR for BCC was found among patients with connective tissue disease (IRR 1.34 (95% CI: 0.99-1.82)), transplants (IRR 8.00 (95% CI: 2.15-30)), and lymphoma (IRR 2.50 (95% CI: 1.29-4.84)). An overall association between squamous cell carcinoma (SCC) and hospitalization for chronic diseases was found and specific among patients with leukemia (IRR 7.75 (95% CI: 2.35-26)), lymphoma (IRR 3.86 (95% CI: 0.99-15)), and skin diseases (IRR 5.28 (95% CI: 1.95-14)). Our study supports the presence of an association between certain chronic diseases and NMSC, and further suggests that these results unlikely are due to bias. JID JOURNAL CLUB ARTICLE: For questions, answers, and open discussion about this article please go to http://network.nature.com/group/jidclub.