Changes in Hydroxyurea Use Among Youths Enrolled in Medicaid With Sickle Cell Anemia After 2014 Revision of Clinical Guidelines.

Importance: Youths with sickle cell anemia (SCA) are at risk of pain crises, stroke, and early death. Complications can be reduced by the oral disease-modifying medication hydroxyurea, and in 2014, the National Heart, Lung, and Blood Institute published revised guidelines that hydroxyurea should be offered to youths aged 9 months and older with SCA regardless of disease severity. Objective: To describe changes in hydroxyurea use among youths with SCA before and after release of the National Heart, Lung, and Blood Institute guidelines. Design, Setting, and Participants: This cross-sectional study was conducted using administrative data from 2010 to 2018 from Michigan and New York State (NYS) Medicaid programs. The study population included youths aged 1 to 17 years with SCA enrolled in the Michigan or NYS Medicaid programs for at least 1 year (Michigan: 2010-2018; NYS: 2012-2018). Youths with SCA were identified using validated claims-based definitions. Data were analyzed from June to October 2020. Main Outcomes and Measures: The main outcome was hydroxyurea use characterized as mean annual counts of days' supply of filled hydroxyurea prescriptions. Rates of hydroxyurea use over time were assessed using regression models (Michigan: zero-inflated negative binomial; NYS: negative binomial). Models included indicators for periods before and after guideline release. Results: A total of 4302 youths with SCA (2236 males [52.0%]; 2676 born 2005-2017 [62.2%]; 150 Hispanic [3.5%], 2929 non-Hispanic Black [68.0%], and 389 non-Hispanic White [9.0%]) contributed 12 565 person-years. The mean (SD) annual days' supply of hydroxyurea was 47.2 (93.6) days per youth in Michigan and 97.4 (137.0) days per youth in NYS. In Michigan, there was an increase in the odds of having nonzero days' supply after the guidelines were released (odds ratio, 1.52; 95% CI, 1.07-2.14). In NYS, no change was seen in the mean days' supply of filled hydroxyurea. Conclusions and Relevance: These findings suggest that hydroxyurea was substantially underused among youths with SCA, despite establishment as the primary disease-modifying therapy for SCA, and that there was incomplete clinician or patient uptake of newly released guidelines. Results suggest that expanding use of hydroxyurea may require a multifaceted approach that includes addressing multiple system- and patient-level barriers.

Is frictional lichenoid dermatitis a minor variant of atopic dermatitis or a photodermatosis.

CONTEXT: Frictional lichenoid dermatitis. BACKGROUND: Frictional lichenoid dermatitis (FLE) is an entity that is probably under diagnosed and has been variably associated with either friction and/or atopy with a distinctive seasonal variation. AIMS AND OBJECTIVES: To study correlation of FLE with UV index and to assess its association with atopic dermatitis. MATERIALS AND METHODS: A cross sectional analysis of children with FLE was done, over a period of 6 years in two tertiary hospitals. A detailed history and examination was done to assess the features of atopic dermatitis. The number of cases seen per month was compared with the mean monthly UV index. Two-tailed significance tests using Pearson's coefficient of correlation and T-test were used to interpret the data. (P < 0.05). RESULTS: One hundred seventy-four patients were studied using the UKC criterion 17.2% of the patients had AD while xerosis (40.3%) was the predominant cutaneous finding. The number of patients seen in summer was more than in winter (P < 0.05) but there was no statistical difference between the cases in winter and spring. There was a significant correlation of the number of cases per month with UV index (P = 0.019). Almost 42% of patients gave a history of recurrence. CONCLUSIONS: FLE is probably not associated with atopic dermatitis and is likely to be related to the ambient UV index though a larger cohort with meticulous follow up may be needed to draw a final conclusion. STATISTICAL ANALYSIS USED: The Pearson's coefficient of correlation was used for comparing the cases per month with the UV index. The tests of hypothesis used included the paired T-tests. F-test of variance, Welch test, Wilcoxon rank sum test and the Kolmogorov-Smirnov Test. P < 0.05 was considered significant.

Chilblain lupus erythematosus in an adolescent girl.

Chilblain Lupus Erythematosus (CHLE) is a rare form of cutaneous lupus erythematosus (LE), more frequently seen in middle aged females. It is characterized by erythematous to violaceous plaques over the acral areas induced by exposure to cold or drop in temperature unlike lesions of lupus erythematosus that worsen with sun exposure. Here, we present a case of chilblain lupus erythematosus in an adolescent girl with few unique features not previously reported.

Trichophyton tonsurans induced recurrent onychomadesis in a very young infant.

Onychomadesis, or nail shedding, is rarely seen in children and can be due to stress, systemic illnesses, trauma, and drug therapy or may be idiopathic. Fungal infection of the nail is only rarely reported as a cause of onychomadesis. We present here a case of Trichophyton tonsurans-induced onychomycosis and resultant onychomadesis in a 9-month-old boy with onset of lesions at 2 weeks of life.

Chanarin-Dorfman syndrome with eccrine gland vacuolation: a case report.

Chanarin-Dorfman syndrome is a rare congenital disorder of lipid metabolism characterized by ichthyosis, leukocytic vacuolation (Jordan's anomaly), and variable involvement of the liver and neuromuscular system, with about 40 cases described worldwide to date. We report one more case of this rare syndrome, with certain peculiarities, namely vacuolation in eccrine glands, in a young male adult.

A case of extensive linear porokeratosis with evaluation of topical tretinoin versus 5-flourouracil as treatment modalities.

A 19-year-old male presented with linear raised lesions over extensive areas of his body that followed Blaschko's lines. The lesions were asymptomatic with ridged borders. Histopathology confirmed a diagnosis of porokeratosis. He was given topical tretinoin and topical flourouracil for local application on specified areas. The cosmetic acceptability and the safety profile of tretinoin were found to be better than those of flourouracil. However, both the agents were equally efficacious. A rare case of extensive porokeratosis with comparative evaluation of two treatment modalities is hereby presented.