Localized scleroderma (also called morphea) can be classified into several subtypes: generalized, plaque-type, bullous, linear, and deep morphea. In addition, eosinophilic fasciitis can be distinguished from these.1 It is important to know more about the prevalence and severity of the frequently reported symptoms of itch, pain, and fatigue to develop guidelines for managing sclerotic skin diseases.2
We evaluated all patients seen at the Radboud University outpatient clinic from 1995 to 2007 for sclerotic skin disease (eg, localized scleroderma or eosinophilic fasciitis confirmed by dermatologic, rheumatologic, and if necessary pathologic investigation) (n = 120). Exclusion criteria consisted of severe psychiatric or mental disabilities, age younger than 16 years, systemic sclerosis according to American College of Rheumatology3 criteria, or pseudosclerosis.4
The demographic variables of age and sex were determined from the medical records. Time since diagnosis was measured in years. Comorbidity was measured using the comorbidity item of the Impact of Chronic Skin Disease on Daily Life instrument (ISDL).5 Disease severity was assessed using a validated 9-item skin status scale from the ISDL that measures the extent of the disease on different parts of the body (scale range, 9-36). Mean levels of itch, pain, and fatigue over the previous 4 weeks were estimated using the visual analog scale (VAS) of the ISDL (range, 0-100 mm). A VAS higher than 20 mm indicated experience of symptoms, while a VAS higher than 50 mm indicated relatively severe symptoms.2 Disease-related quality of life was measured with the 10-item scale of the Dermatology Life Quality Index (DLQI).6
Mean (SD) values were calculated for the whole group and for the disease subtypes. In addition, percentages for the previously validated cutoff scores2 were calculated. The relationship of symptoms to age, comorbidity, time since diagnosis, disease severity, and disease-related quality of life was explored by calculating Pearson correlation coefficients or binary correlations in case of sex, using P < .05 as the level of significance (2-sided).
Seventy-four completed questionnaires were suitable for analysis (62%). No significant differences were found between responders and nonresponders with regard to sex, age, duration of disease since diagnosis, or disease subtype. Demographic data, time since diagnosis, disease severity, and prevalence of the symptoms are summarized in the Table. Comorbidity was reported in 30% of patients (n = 22), with 18% reporting 1 comorbidity (n = 13), 10% reporting 2 (n = 7), and 3% reporting 3 comorbidities (n = 2).
Fatigue was the most frequently experienced symptom (n = 41; 55%) (Table), followed by pain (n = 24; 32%) and itch (n = 16; 22%). Forty-six patients experienced at least 1 of the 3 symptoms (62%), while 27 patients reported at least 1 severe symptom (VAS >50 mm) (36%). Most patients with generalized morphea (n = 15; 94%) and eosinophilic fasciitis (n = 9; 75%) reported fatigue. Pain and itch were experienced most often by patients with eosinophilic fasciitis.
Fatigue, itch, and pain were significantly related to a lower disease-related quality of life (r = 0.62, r = 0.44, and r = 0.43, respectively) (P < .01). Fatigue and pain, but not itch, were significantly associated with greater self-reported disease severity (r = 0.39 and r = 0.37, respectively) (P < .01). Fatigue was significantly related to a shorter duration since diagnosis of the sclerotic disease (r = 0.27) (P < .05).
Fatigue, pain, and/or itch were experienced by 62% of patients (n = 46). Fatigue was the most commonly reported symptom. Correlations with the DLQI, disease severity, and duration indicate that the physical symptoms were consequences of skin disease and might be particularly relevant in the early stages. Most patients with generalized morphea and eosinophilic fasciitis reported fatigue, and a substantial proportion of patients noted severe fatigue. Patients with eosinophilic fasciitis particularly reported pain and itch. In eosinophilic fasciitis, the sclerosis is more extensive and deeper than it is in localized morphea, and it is associated with peripheral eosinophilia. These facts may explain why patients with eosinophilic fasciitis experienced more pain and itch than other patients.
The study has several limitations, such as absence of a control group and small sample sizes, thus limiting comparison possibilities. Also, more detailed assessments of comorbidities and medications would be worthwhile. The disease severity was based on patient assessment rather than physician assessment. The questionnaires were not necessarily completed during a stage of active disease; thus, the presence and severity of symptoms might have been underestimated.2
This study describes the high impact of fatigue, pain, and to a lesser extent itch in patients with localized scleroderma and eosinophilic fasciitis. Physicians should be encouraged to assess these symptoms and, where appropriate, focus treatment on them.
Correspondence: Dr Kroft, Radboud University Nijmegen Medical Centre, PO Box 9101, NL-6500 HB Nijmegen, the Netherlands (email@example.com).
Author Contributions: Drs Kroft, de Jong, and Evers had full access to all of the data in the study and take responsibility for the integrity of the data and the accuracy of the data analysis. Study concept and design: Kroft, de Jong, and Evers. Acquisition of data: Kroft, de Jong, and Evers. Analysis and interpretation of data: Kroft, de Jong, and Evers. Drafting of the manuscript: Kroft, de Jong, and Evers. Critical revision of the manuscript for important intellectual content: Kroft, de Jong, and Evers. Statistical analysis: Kroft. Administrative, technical, and material support: Kroft, de Jong, and Evers. Study supervision: Kroft, de Jong, and Evers.
Financial Disclosure: None reported.
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