Pemphigus and Quality of Life Shien-Ning Chee, MBBS, Dédée F. Murrell, MA, BMBCh, FAAD, MD, FACD* Skin diseases are rarely life threatening, but their impact on a patient’s quality of life (QOL) can be massive. For example, the impact of psoriasis on QOL has been found to be comparable with the impact of heart failure. 1 Contributing factors include physical discomfort, time spent on treat- ment, staining of sheets and clothes with skin scales or blood, and the visible nature of skin diseases, which often brings negative reactions from the public, thus diminishing self-esteem. 2 Measurement of the impact of disease on QOL is of utmost importance in dermatology and interest in the area has been recently growing. 1 QOL is increasingly recognized as an important outcome for evaluating effectiveness of care, capturing aspects of patients’ health status that are important for the patient, but may not correlate with clinical severity as assessed by physicians. 3 Pemphigus is a skin disease on which few QOL studies have been conducted, and for which a disease-specific QOL questionnaire has not yet been developed. ABOUT PEMPHIGUS Pemphigus is a group of autoimmune blistering diseases characterized by loss of adhesion between keratinocytes giving rise to blister forma- tion. The intraepithelial blister formation is caused by acantholysis: the loss of adhesion between ker- atinocytes caused by autoantibodies directed against the intercellular adhesion structures. 4 The different forms of pemphigus may be distinguished by the specificity of the autoanti- bodies for different targets or by the location of blister formation. With the most common form, pemphigus vulgaris (PV), blisters are located just above the basal skin layer. With pemphigus foliaceus (PF), blisters occur within the upper layers of the epidermis. Other subtypes of the pemphigus group are paraneoplastic pemphigus (usually occurring in patients with lymphoma) and drug-induced pemphigus. Rare pemphigus variants include pemphigus herpetiformis and pemphigus erythematosus. 4 INSTRUMENTS THAT HAVE BEEN USED TO STUDY QOL IN PEMPHIGUS There are a wide variety of QOL measures. They may categorized as genetic, skin specific, or disease specific. 5 Generic measures allow for comparisons across all diseases. The Medical Outcome Study 36-item Short-form Survey (SF-36) is a generic instrument that measures health-related QOL and is thus useful in comparing QOL in patients with pemphigus with the general population. It uses a multi-item scale that assesses 8 health concepts 6 : (1) limitations in physical activities because of health problems, (2) limitations in social activities because of physical or emotional prob- lems, (3) limitations in usual role activities because of physical health problems, (4) bodily pain, (5) general mental health, (6) limitations in usual role activities because of emotional problems, (7) vitality, and (8) general health perceptions. A score of 0 to 100 is calculated for each domain, with high- er scores correlating with better QOL. 5 Skin-specific measures used in dermatology allow for comparisons across skin diseases. The Dermatology Life Quality Index (DLQI) was derived Department of Dermatology, St George Hospital, University of New South Wales, Gray Street, Kogarah, Sydney, NSW 2217, Australia * Corresponding author. E-mail address: d.murrell@unsw.edu.au KEYWORDS Pemphigus Quality of life Skin Autoimmune condition Dermatol Clin 29 (2011) 521–525 doi:10.1016/j.det.2011.03.009 0733-8635/11/$ – see front matter Ó 2011 Elsevier Inc. All rights reserved. derm.theclinics.com