Evaluating the association of central centrifugal cicatricial alopecia (CCCA) and fibroproliferative disorders.

BACKGROUND: In central centrifugal cicatricial alopecia (CCCA), a lymphocytic scarring alopecia that primarily affects black women, it has been postulated that there is a "pro-fibrotic" tendency and increased risk for systemic fibroproliferative disorders. OBJECTIVE: To determine whether women with biopsy-proven CCCA have a greater likelihood of systemic fibroproliferative disorders (FPDs) of the lungs (interstitial lung disease), arteries (atherosclerosis of the aorta), liver (non-alcoholic steatohepatitis), kidney (end stage renal disease), or uterus (uterine leiomyoma). METHODS: We conducted a retrospective matched cohort study evaluating 427 cases with biopsy-proven CCCA and 1281 age- and sex-matched controls. RESULTS: Black women with biopsy-proven CCCA, were not more likely to have interstitial lung disease (ILD), atherosclerosis of the aorta, non-alcoholic steatohepatitis (NASH), end stage renal disease (ESRD), or uterine leiomyoma. Central centrifugal cicatricial alopecia was associated with a history of never smoking and higher body mass index. CONCLUSION: In this large cohort of biopsy-proven women with CCCA, there was no association with specific fibroproliferative disorders when compared with age and sex matched controls. Future longitudinal studies may help confirm these results.

A randomized controlled trial of an emollient with ceramide and filaggrin-associated amino acids for the primary prevention of atopic dermatitis in high-risk infants.

BACKGROUND: Skin barrier dysfunction may precede infantile development of clinical atopic dermatitis (AD). Early-life emollient therapy to enhance barrier function may prevent or modify AD development in high-risk infants. OBJECTIVES: (a) To determine whether daily full-body application of an emollient with ceramide and amino acids (study emollient) can reduce the cumulative AD incidence compared to standard skin care at 1 year of age. (b) To evaluate the study emollient's effect on skin barrier function, natural moisturizing factor and the microbiome using non-invasive biophysical and biochemical techniques. METHODS: We performed a single-centre, investigator-blinded, randomized controlled trial enrolling infants at high risk for AD development determined by family history. The intervention was full-body once-daily application of the study emollient. The control arm was asked to not apply full-body emollient regularly and only use an emollient of their choice for dry skin. The primary outcome was the cumulative incidence of AD diagnosed at 12 months by a blinded investigator. RESULTS: Less than half the target sample size was enrolled (n = 100, goal sample was 208) with 28% lost to follow-up. Across all clinical end points, a numerical trend was observed in favour of the intervention, although not statistically significant likely due to lack of power from under-enrolment. AD was diagnosed in 13.2% vs. 25.0% at 12 months (P = 0.204) and 19.4% vs. 31.0% at 2 years (P = 0.296) in intervention vs. control groups, respectively. There were no significant differences between groups in skin barrier or microbiome assessments. While there were no serious adverse events, there were more cases of reported contact dermatitis in the intervention vs. control arms, 9.3% vs. 4.3%, respectively; however, these events were not related to the study emollient and most mild in severity. CONCLUSION: The observed trends suggest a protective effect of daily study emollient therapy compared to control.

Frontal fibrosing alopecia: a clinical review of 36 patients.

BACKGROUND: Frontal fibrosing alopecia (FFA) is a primary lymphocytic cicatricial alopecia with a distinctive clinical pattern of progressive frontotemporal hairline recession. Currently, there are no evidence-based studies to guide treatment for patients with FFA; thus, treatment options vary among clinicians. OBJECTIVES: We report clinical findings and treatment outcomes of 36 patients with FFA, the largest cohort to date. Further, we report the first evidence-based study of the efficacy of hydroxychloroquine in FFA using a quantitative clinical score, the Lichen Planopilaris Activity Index (LPPAI). METHODS: A retrospective case note review was performed of 36 adult patients with FFA. Data were collected on demographics and clinical findings. Treatment responses to hydroxychloroquine, doxycycline and mycophenolate mofetil were assessed using the LPPAI. Adverse events were monitored. RESULTS: Most patients in our cohort were female (97%), white (92%) and postmenopausal (83%). Apart from hairline recession, 75% also reported eyebrow loss. Scalp pruritus (67%) and perifollicular erythema (86%) were the most common presenting symptom and sign, respectively. A statistically significant reduction in signs and symptoms in subjects treated with hydroxychloroquine (P < 0·05) was found at both 6- and 12-month follow up. CONCLUSIONS: In FFA, hairline recession, scalp pruritus, perifollicular erythema and eyebrow loss are common at presentation. Despite the limitations of a retrospective review, our data reveal that hydroxychloroquine is significantly effective in reducing signs and symptoms of FFA after both 6 and 12 months of treatment. However, the lack of a significant reduction in signs and symptoms between 6 and 12 months indicates that the maximal benefits of hydroxychloroquine are evident within the first 6 months of use.