Gómez-López-Hernández Syndrome: A Rare Cause of Bilateral Nonscarring Alopecia.

Gómez-López-Hernández syndrome is a rare neurocutaneous disorder characterized by the triad of rhombencephalosynapsis, parietal alopecia, and trigeminal anesthesia. We report a 16-year-old girl with bilateral parietotemporal alopecia in whom cranial magnetic resonance imaging revealed rhombencephalosynapsis, suggesting a diagnosis of Gómez-López-Hernández syndrome. Neurologic examination and neuroimaging may be warranted in select patients with parietal alopecia to exclude this uncommon entity.

Impact of psoriasis in the quality of life of children, adolescents and their families: a cross-sectional study.

BACKGROUND: Psoriasis 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. OBJECTIVE: To 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. METHODS: A 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. RESULTS: The 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 LIMITATIONS: Lack of a control group and patient assessment of disease severity. Relatively mild disease severity of the subjects. CONCLUSIONS: Psoriasis 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.

Efficacy, safety and drug survival of conventional agents in pediatric psoriasis: A multicenter, cohort study.

The data on long-term efficacy, safety and drug survival rates of conventional systemic therapeutics in pediatric psoriasis is lacking. The primary aim of this study is to investigate acitretin, methotrexate, cyclosporin efficacy, safety and drug survival rates in pediatric patients as well as predictors of drug survival. This is a multicenter study including 289 pediatric cases being treated with acitretin, methotrexate and cyclosporin in four academic referral centers. Efficacy, adverse events, reasons for discontinuation, 1, 2- and 3-year drug survival rates, and determinants of drug survival were analyzed. A 75% reduction of Psoriasis Area and Severity Index score or better response rate was obtained in 47.5%, 34.1% and 40% of the patients who were treated with acitretin, methotrexate and cyclosporin, respectively. One-year drug survival rates for acitretin, methotrexate and cyclosporin were 36.3%, 21.1% and 15.1%, respectively. The most significant determinant of drug survival, which diminished over time, was treatment response whereas arthritis, body mass index and sex had no influence. Although all three medications are effective and relatively safe in children, drug survival rates are low due to safety concerns at this age group. Effective disease control through their rational use can be expected to improve survival rates.

Neonatal lupus erythematosus: Report of a case with cutaneous, hematological and hepatobiliary findings.

Neonatal lupus erythematosus is an autoimmune disorder mainly affecting the heart and skin. It is the most common cause of congenital heart block. In addition, hematological, hepatobiliary and neurological involvement may occur. Herein, we report a 23-day-old infant presented with annular, erythematous, and scaly and atrophic lesions on the face and trunk. Based on the clinical, laboratory and histopathological findings, she was diagnosed as neonatal lupus erythematosus. Neonatal lupus eryhtematosus should be considered in infants presenting with annular skin lesions, and we present this case to highlight the value of high index of clinical suspicion in diagnosis.

Impact of psoriasis in the quality of life of children, adolescents and their families: a cross-sectional study

Abstract: Background: Psoriasis 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. Objective: To 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. Methods: A 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. Results: The 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 limitations: Lack of a control group and patient assessment of disease severity. Relatively mild disease severity of the subjects. Conclusions: Psoriasis 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.