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What is the burden of living with autoimmune polyendocrinopathy candidiasis ectodermal dystrophy (APECED) in 2012? A health-related quality-of-life assessment in Finnish patients

Authors

  • Nicolas Kluger,

    Corresponding author
    • Departments of Dermatology, Allergology and Venereology, Institute of Clinical Medicine, University of Helsinki and Skin and Allergies Hospital, Helsinki University Central Hospital, HUS, Finland
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  • Martta Jokinen,

    1. Departments of Dermatology, Allergology and Venereology, Institute of Clinical Medicine, University of Helsinki and Skin and Allergies Hospital, Helsinki University Central Hospital, HUS, Finland
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  • Kai Krohn,

    1. Clinical Research Institute HUCH Ltd, HUS, Finland
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  • Annamari Ranki

    1. Departments of Dermatology, Allergology and Venereology, Institute of Clinical Medicine, University of Helsinki and Skin and Allergies Hospital, Helsinki University Central Hospital, HUS, Finland
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Correspondence: Nicolas Kluger, Departments of Dermatology, Allergology and Venereology, Institute of Clinical Medicine, University of Helsinki and Skin and Allergies Hospital, Helsinki University Central Hospital, Meilahdentie 2, P.O. Box 160, FI-00029 HUS, Finland.

Tel.: +358 (0) 9 471 86475; Fax: +358 (0) 9 471 86561;

E-mail: nicolaskluger@yahoo.fr

Summary

Objective

Autoimmune polyendocrinopathy candidiasis ectodermal dystrophy (APECED) is a rare disorder responsible for chronic candidiasis, a wide variety of autoimmune disorders and a risk of squamous cell carcinoma of the oral cavity or oesophagus. We investigated the impairment of quality of life in our cohort of Finnish patients.

Subjects, design and measurement

In a postal survey, 26 patients with APECED responded to three self-reported health-related quality-of-life questionnaires: RAND-36 (general health), RBDI (depression) and DLQI (dermatology life quality index).

Results

General health and vitality were the most affected items in our cohort. Male subjects presented higher impairment in emotional role limitations, social functioning, bodily pain, general mental health/emotional well-being, energy/vitality and general health perceptions but without reaching statistical significance. The number of accumulated diseases in APECED was not associated with lower results. But, age and duration of APECED correlated with fatigue (= 0·01), well-being (= 0·02) and general health (= 0·03) impairment. Depressive symptoms affected 29% of the patients. There was a statistical negative correlation between RBDI score and age and duration of APECED. Hair loss, alopecia areata universalis especially, affected more severely the quality of life of female patients. Vitiligo and candidiasis did not have any significant impact on both the genders.

Conclusions

We report the first study on specific impairment of quality of life related to APECED in a cohort of adult Finnish patients. General health, emotional well-being and vitality were the most diminished aspects of quality of life in our cohort. However, our results will need to be confirmed by additional controlled studies.

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