Validity and Reliability of the Dutch Adaptation of the Actinic Keratosis Quality of Life Questionnaire (AKQoL).

BACKGROUND: The Actinic Keratosis Quality of Life Questionnaire (AKQoL) is a disease-specific instrument to measure the impact of actinic keratosis (AK) on patients' lives. OBJECTIVE: To validate and test the psychometric properties of the AKQoL translated into the Dutch language (AKQoL-NL). METHODS: All new patients ≥50 years of age with untreated AK in a university medical center and a general hospital between August 2014 and August 2015 were eligible. The AKQoL was obtained and repeated after 2 weeks. The feasibility was tested by missing responses and response distribution. The internal consistency reliability of each domain was investigated with the Cronbach alpha, and test-retest reliability and validity with the Spearman correlation coefficient. AKQoL scores were compared to the Skindex-17 for convergent validity and to the Groningen Frailty Indicator scores for divergent validity. RESULTS: A total of 153 of 190 eligible patients consented to participate. Feasibility analysis showed that none of the items missed ≥10% of responses but 5 of the 9 items showed floor effect. The AKQoL subscales showed a moderate internal consistency (Cronbach α = 0.235-0.468) and an excellent test-retest reliability (interclass correlation coefficient = 0.997-1). The AKQoL correlated poorly with the symptom component and moderately with the psychosocial component of the Skindex-17 (ρ = -0.015 to 0.346 and 0.324 to 0.501, respectively), which is less than expected. The AKQoL scored poorly in both of the Groningen Frailty Indicator (GFI) components (ρ = -0.97 to 0.12 and 0.185 to 0.276, respectively), as expected. CONCLUSION: The AKQoL-NL is a feasible, moderately valid, and moderately reliable health-related quality of life questionnaire.

From sporadic atypical nevi to familial melanoma: risk analysis for melanoma in sporadic atypical nevus patients.

BACKGROUND: Atypical nevi (AN), present in either a familial or a sporadic setting, are strong indicators of increased melanoma risk. OBJECTIVE: To estimate the extent of this risk and the extent of reclassification of sporadic to familial cases during follow-up. METHODS: We studied 167 sporadic patients with AN (>or=5). At the end of follow-up we updated the family history regarding melanoma and performed germline mutation analysis of the known melanoma susceptibility genes. RESULTS: We found a relative risk for melanoma of 46.1 (95% confidence interval 21.0-87.5). Six of 167 patients were carriers of a CDKN2A mutation. At the end of follow-up, 10 of 136 patients with sporadic AN reported being a member of a melanoma family. LIMITATIONS: This study was conducted in an area with a founder mutation in many of its melanoma families; therefore the results may not be applicable to other populations. CONCLUSION: We report a high relative risk of 46.1 of melanoma development in patients with sporadic AN. A significant proportion of this Dutch cohort reported additional cases in their families over time.

Full-blown graft-versus-host disease presenting with skin manifestations, jaundice and diarrhoea: an unusual paraneoplastic phenomenon of a thymoma.

We describe a patient who presented with chronic diarrhoea, skin lesions and jaundice. Based on histopathological examinations of the affected organs combined with the clinical features, it appeared that the patient fulfilled the criteria for graft-versus-host disease (GVHD). GVHD occurs especially after allogeneic stem cell transplantation and sometimes after organ transplantations. However, this patient had never undergone such a procedure. Further examination revealed that the patient also suffered from a thymoma, which was concluded to be the cause of GVHD. Unfortunately, the patient died after resection of the thymoma. This patient is probably the second case with thymoma and full-blown GVHD and shows that GVHD can occur in the absence of a previous transplantation.