High-throughput T cell receptor sequencing identifies clonally expanded CD8+ T cell populations in alopecia areata.

Alopecia areata (AA) is an autoimmune disease in which cytotoxic T cells specifically target growing hair follicles. We used high-throughput TCR sequencing in the C3H/HeJ mouse model of AA and in human AA patients to gain insight into pathogenic T cell populations and their dynamics, which revealed clonal CD8+ T cell expansions in lesional skin. In the C3H/HeJ model, we observed interindividual sharing of TCRβ chain protein sequences, which strongly supports a model of antigenic drive in AA.

Childhood alopecia areata-Data from the National Alopecia Areata Registry.

Background/objectives: Alopecia areata may occur at any age and is the third-most-common dermatosis in children. The objective of this study was to investigate the clinical and epidemiologic features of children and adolescents with alopecia areata based on the data of the National Alopecia Areata registry on children and adolescents.

Methods: Two thousand two hundred eighteen children and adolescents with alopecia areata self-enrolled in the National Alopecia Areata Registry and completed a web-based, self-administered, short-intake screening questionnaire (first tier).

Molecular signatures define alopecia areata subtypes and transcriptional biomarkers.

Alopecia areata (AA) is an autoimmune disease typified by nonscarring hair loss with a variable clinical course. In this study, we conducted whole genome gene expression analysis of 96 human scalp skin biopsy specimens from AA or normal control subjects. Based on gene expression profiling, samples formed distinct clusters based on the presence or absence of disease as well as disease phenotype (patchy disease compared with alopecia totalis or universalis).

Genome-wide meta-analysis in alopecia areata resolves HLA associations and reveals two new susceptibility loci.

Alopecia areata (AA) is a prevalent autoimmune disease with 10 known susceptibility loci. Here we perform the first meta-analysis of research on AA by combining data from two genome-wide association studies (GWAS), and replication with supplemented ImmunoChip data for a total of 3,253 cases and 7,543 controls. The strongest region of association is the major histocompatibility complex, where we fine-map four independent effects, all implicating human leukocyte antigen-DR as a key aetiologic driver.

A single question can make the diagnosis.

Alopecia areata is the only condition that can cause complete hair loss in 3 months without symptoms or signs.

What looks like alopecia areata is not always alopecia areata.

The differential diagnosis of a strongly positive and painless hair pull test includes alopecia areata and loose anagen syndrome. A hair mount examined with low power light microscopy easily clarifies the diagnosis.

Health-Related Quality of Life (HRQoL) in alopecia areata patients-a secondary analysis of the National Alopecia Areata Registry Data.

Alopecia areata (AA) is a nonscarring and recurrent disease characterized by hair loss that may significantly affect patient health-related quality of life (HRQoL). Given the lack of reliable and accurate reporting of HRQoL status in patients with AA, we analyzed data from 532 AA patients from the National Alopecia Areata Registry whose registry record included HRQoL assessments using three validated instruments: Skindex-16, brief version of the Fear of Negative Evaluation Scale, and Dermatology Life Quality Index. The mean HRQoL scores were compared with previously reported HRQoL levels from healthy controls and patients with other skin diseases.

Bone mineral density in patients with alopecia areata treated with long-term intralesional corticosteroids.

Background: Intralesional corticosteroid injections are a common treatment for patchy alopecia areata, the most prevalent subtype of this autoimmune hair disorder. To date, no studies have examined the potential adverse effects of this therapy on bone mineral density (BMD).

Methods: In this retrospective, cross-sectional case series, 18 patients with patchy alopecia areata treated at 4- to 8-week intervals with intralesional triamcinolone acetonide for at least 20 months were evaluated for BMD using dual-energy x-ray absorptiometry (DXA).

How to diagnose hair loss.

This review presents a systematic approach to the diagnosis of hair loss. An accurate diagnosis is based on history, clinical examination, laboratory tests, and scalp biopsy. Whether the hair loss is a cicatricial or noncicatricial alopecia guides one's history taking.

Sterol intermediates of cholesterol biosynthesis inhibit hair growth and trigger an innate immune response in cicatricial alopecia.

Primary cicatricial alopecia (PCA) is a group of inflammatory hair disorders that cause scarring and permanent hair loss. Previous studies have implicated PPARγ, a transcription factor that integrates lipogenic and inflammatory signals, in the pathogenesis of PCA. However, it is unknown what triggers the inflammatory response in these disorders, whether the inflammation is a primary or secondary event in disease pathogenesis, and whether the inflammatory reaction reflects an autoimmune process.

The "Fringe Sign" - A useful clinical finding in traction alopecia of the marginal hair line.

Introduction: Traction alopecia is hair loss caused by prolonged or repetitive tension on the hair. Diagnostic challenges are encountered when the clinical suspicion is not high and when a history of traction is remote or not obtained. We have made the observation that the presence of retained hairs along the frontal and/or temporal rim, which we termed the "fringe sign," is a finding seen in both early and late traction alopecia, and may be a useful clinical marker of the condition.

A practical, algorithmic approach to diagnosing hair shaft disorders.

The hair shaft is a unique structure composed of an inner cortex and a protective outer cuticle. Any defects in this normal structure due to genetics or the environment can lead to variations in physical properties. Thus one should suspect a hair shaft disorder if a patient presents with an abnormality or change in hair texture, appearance, manageability or ability to grow hair long.

Abnormal inner root sheath of the hair follicle in the loose anagen hair syndrome: an ultrastructural study.

Background: Loose anagen hair syndrome (LAHS) is a disorder in which the hair pulls out easily and painlessly from the scalp. It first manifests in early childhood when the main concern of parents is that the sparse hair does not grow. The hair density and length improve with age, but the looseness persists into adulthood.

The spectrum of hair loss in patients with mycosis fungoides and Sézary syndrome.

Background: Alopecia can be a manifestation of mycosis fungoides (MF) and Sézary syndrome (SS), but the prevalence is unknown.

Objective: We sought to describe the clinicopathologic presentation and molecular features of alopecia in patients with MF/SS.

Methods: A retrospective chart review of a prospectively collected MF/SS database was used to identify patients with alopecia.

Genome-wide association study in alopecia areata implicates both innate and adaptive immunity.

Alopecia areata (AA) is among the most highly prevalent human autoimmune diseases, leading to disfiguring hair loss due to the collapse of immune privilege of the hair follicle and subsequent autoimmune attack. The genetic basis of AA is largely unknown. We undertook a genome-wide association study (GWAS) in a sample of 1,054 cases and 3,278 controls and identified 139 single nucleotide polymorphisms that are significantly associated with AA (P View Article and Find Full Text PDF

Efficacy and safety of mycophenolate mofetil for lichen planopilaris.

Background: Lichen planopilaris (LPP) is a chronic inflammatory disorder that causes permanent scalp hair loss and significant patient discomfort.

Objectives: We sought to determine the efficacy and safety of mycophenolate mofetil (MMF) for treatment of LPP in patients who had failed prior topical, intralesional, or oral anti-inflammatory medications such as hydroxychloroquine or cyclosporine.

Methods: We conducted a retrospective chart review of 16 adult patients with LPP treated with at least 6 months of MMF in an open-label, single-center study from 2003 to 2007.

Hydroxychloroquine and lichen planopilaris: efficacy and introduction of Lichen Planopilaris Activity Index scoring system.

Background: Lichen planopilaris (LPP) and its variant frontal fibrosing alopecia (FFA) are primary lymphocytic cicatricial alopecias for which there is no evidence-based therapy.

Objective: We assessed the efficacy of hydroxychloroquine in active LPP and FFA using the LPP Activity Index (LPPAI), a numeric score that allows quantification of the symptoms and signs of the condition for statistical comparison. In addition, we determined with the LPPAI if any improvement (reduction) in the numeric score pretreatment and posttreatment reached statistical significance.