BackgroundPsoriasis is a chronic and inflammatory disease that impairs quality of life
and causes psychological symptoms. Despite the high prevalence of psoriasis
in pediatric patients, studies investigating the impact of psoriasis in the
quality of life of children, adolescents and families are sparse.ObjectiveTo investigate the impact of psoriasis in the quality of life of children and
adolescents with psoriasis and their families and to determine depression
and anxiety levels of the patients.MethodsA total of 58 patients with psoriasis aged 7-18 years (median age: 11) and a
family member of each patient were included in the study. Children's
Dermatology Life Quality Index (CDLQI), Family Dermatology Life Quality
Index (FDLQI), Children's Depression Inventory (CDI) and State-Trait Anxiety
Inventory for Children (STAIC) were used in the study.ResultsThe median PASI score of the patients included in the study was 1.8. The
median CDLQI and FDLQI scores in the study groups were 5 and 10,
respectively. The median CDI score, STAIC-state and STAIC-trait scores of
the patients were 6, 28 and 32.5, respectively.Study limitationsLack of a control group and patient assessment of disease severity.
Relatively mild disease severity of the subjects.ConclusionsPsoriasis has a negative impact in the quality of life of children,
adolescents and their families, even in the presence of mild disease.
Considering that impairment in quality of life may be associated with
psychosocial morbidity, a combined approach with medical therapy, family
counseling and quality of life assessment may be beneficial in this patient
group.
Background: No vitiligo-specific quality of life scale exists. Objective: To develop a reliable and valid quality of life scale for vitiligo patients. Methods: The content was derived from in-depth interviews with vitiligo patients. The internal consistency, test-retest reliability and validity of the scale, VLQI (Vitiligo Life Quality Index), were evaluated. Results: Internal consistency was high with 30 patients and then with 183 patients (Cronbach's α 0.92 and 0.91). Test-retest scores were correlated (r = 0.86, p < 0.001). There was no difference between the test and retest scores (p > 0.05). VLQI was correlated with DLQI (Dermatology Life Quality Index) (r = 0.77, p < 0.001) and with the perceived severity by the patients (r = 0.57, p < 0.001). There was a significant relationship between the VLQI and the extent of the disease (p = 0.015). Factor analysis revealed six subscales. Conclusion: The VLQI is a valid and reliable instrument assessing quality of life of vitiligo patients.
Protein kinase C delta (PRKCD) has essential functions in controlling B-cell proliferation and apoptosis, development of B-cell tolerance and NK-cell cytolitic activity. Human PRKCD deficiency was recently identified to be causative for an autoimmune lymphoproliferative syndrome like disorder with significant B-cell proliferation particularly of immature B cells. Here we report a child with a novel mutation in PRKCD gene who presented with CMV infection and an early onset SLE-like disorder which was successfully treated with hydroxychloroquine.
We report a 5-month-old girl diagnosed with bullous pemphigoid who initially did not respond to systemic corticosteroids and dapsone but rapidly improved after the addition of intravenous immunoglobulin (IVIG) infusions. A literature search revealed anecdotal cases of infantile bullous pemphigoid treated with IVIG, although variable treatment regimens were used, and some resistant cases required additional medications such as rituximab for clinical remission.
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