A fatal case of hemophagocytic lymphohistiocytosis due to neonatal lupus erythematosus.

Neonatal lupus erythematosus (NLE) is an autoimmune disease caused by the passive transfer of autoantibodies from mother to child during pregnancy. A rare complication of NLE is hemophagocytic lymphohistiocytosis (HLH), a potentially life-threatening hyperinflammatory state more commonly associated with other rheumatologic disorders. Herein, we describe a fatal case of NLE-associated HLH.

Staphylococcal phosphatidylglycerol antigens activate human T cells via CD1a.

Expressed on epidermal Langerhans cells, CD1a presents a range of self-lipid antigens found within the skin; however, the extent to which CD1a presents microbial ligands from bacteria colonizing the skin is unclear. Here we identified CD1a-dependent T cell responses to phosphatidylglycerol (PG), a ubiquitous bacterial membrane phospholipid, as well as to lysylPG, a modified PG, present in several Gram-positive bacteria and highly abundant in Staphylococcus aureus. The crystal structure of the CD1a-PG complex showed that the acyl chains were buried within the A'- and F'-pockets of CD1a, while the phosphoglycerol headgroup remained solvent exposed in the F'-portal and was available for T cell receptor contact. Using lysylPG and PG-loaded CD1a tetramers, we identified T cells in peripheral blood and in skin that respond to these lipids in a dose-dependent manner. Tetramer+CD4+ T cell lines secreted type 2 helper T cell cytokines in response to phosphatidylglycerols as well as to co-cultures of CD1a+ dendritic cells and Staphylococcus bacteria. The expansion in patients with atopic dermatitis of CD4+ CD1a-(lysyl)PG tetramer+ T cells suggests a response to lipids made by bacteria associated with atopic dermatitis and provides a link supporting involvement of PG-based lipid-activated T cells in atopic dermatitis pathogenesis.

Bitemporal Scalp Hair Loss: Differential Diagnosis of Nonscarring and Scarring Conditions.

BACKGROUND: Bitemporal hair loss can be a diagnostic challenge because several entities may affect this region of the scalp, including both scarring and nonscarring conditions. Although traction alopecia is the most common cause of bitemporal hair loss, no studies to date have outlined all of the potential causes. OBJECTIVE: We sought to review nonscarring and scarring conditions that have a clinical presentation of bitemporal hair loss, including traction alopecia, telogen effluvium, female pattern hair loss, frontal fibrosing alopecia, central centrifugal cicatricial alopecia, and seborrheic dermatitis. METHODS: A Google Scholar and PubMed literature search were conducted for this review. The keywords used in the search included the following: "traction alopecia", "telogen effluvium", "androgenic alopecia", "androgenetic alopecia", "female pattern hair loss", "alopecia areata", "frontal fibrosing alopecia", "central centrifugal cicatricial alopecia", and "seborrheic dermatitis". The scope of our search included all research articles published from 1957 to February 2019. In total, 94 articles regarding non-scarring and scarring hair loss were selected and included according to topic relevance. Exclusion criteria included articles that did not address the epidemiology and/or clinicopathologic or dermatoscopic findings of non-scarring and scarring forms of alopecia. Inclusion criteria included articles that addressed a clinical presentation of bitemporal hair loss; or addressed epidemiology, clinical presentation, dermatoscopic findings, and/or treatment. RESULTS: Bitemporal hair loss is a common and often distressing condition with a broad differential. CONCLUSION: Clinicians must be aware of the potential causes of bitemporal hair loss. Prompt diagnosis is essential to prevent further hair loss, especially in scarring conditions.

Periorbital hypopigmentation and telangiectasias: Clues to diagnosing neonatal lupus in skin of color.

Neonatal lupus erythematosus (NLE) is an autoimmune disease characterized by a periorbital erythematous rash. Although post-inflammatory hypopigmentation and telangiectasias are known possible sequelae, these features may be particularly noticeable in skin of color. Herein, we describe two infants with skin of color in whom periorbital hypopigmentation and telangiectasias were clues to the diagnosis of NLE.

Sweat and Hair: Online Interventions for Improving Physical Activity in African-American Women.

OBJECTIVE: To evaluate the efficacy of an informational website in improving patient knowledge on hair care and exercise. DESIGN: Pre- and post-exposure surveys, focusing on exercise and hair care practices, were administered to subjects after 1 h of browsing a dermatologist-designed hair care and exercise website. SETTING: Winston Salem State University, a historically Black university in North Carolina, USA. PARTICIPANTS: 22 African-American women (AAW) aged between 18 and 54 years. MEASUREMENTS: The outcome measures of the study include patient demographics, hair care practices, knowledge of hair/care disorders, exercise habits, and website feedback. RESULTS: There were 22 women enrolled in the study with 95% identifying as African-American. The average age was 28 ± 12 years. A total of 80% (16/20) of subjects reported exercising less than 150 min per week, and 36% of these patients reported modifying their hairstyle to accommodate physical activity. 85% learned new information about hair and scalp disorders from the educational material on the website, and 81% had increased understanding of their hair health. 91% reported that they would return to the website. CONCLUSION: An informational website can be an effective tool to educate AAW on hair and scalp disease, as well as appropriate hair care practices and fitness goals.

Dermatosis Papulosa Nigra: A Quality of Life Survey Study.

Background: Dermatosis papulosa nigra (DPN) is a skin condition characterized by pigmented sessile and pedunculated papules on the body, particularly on the face and other sun-exposed areas, that can cause cosmetic disfigurement and varying degrees of distress and psychosocial concern among patients. Objective: We sought to evaluate the effect of DPN on quality of life (QoL). Methods: A 39-item questionnaire was administered to 50 African-American (AA) adults, 48 women and two men, with an average age of 51.7 years and visible DPN lesions at a dermatology clinic. We collected demographic information, family history, prior treatment, as well as type(s), frequency, duration of lesions, and attitudes about lesions. Dermatologic QoL (DLQI) survey scores were used to quantify the effect that DPN had on QoL. RESULTS: Most subjects reported lesions on the face (86%); 49 percent reported between 10 to 30 lesions on the body. The average age of diagnosis was 44.7 years (±15.51 years). Further, 84 percent of subjects reported having a firstdegree relative with DPN. Most patients reported little to no symptoms from their DPN (82%), while 36 percent had previously visited a physician due to their DPN. Electrocautery and cryosurgery were the most common methods of DPN removal. All subjects who had their DPN lesions removed reported improvement in the appearance of their skin. The average (standard deviation) DLQI score was six points (±5.42 points), which indicates that DPN has a moderate effect on QoL. Conclusion: Our study results reveal the high likelihood of a family history of DPN in subjects with the condition. Overall, most subjects reported few symptoms of DPN and their QoL was only moderately affected by DPN. Still, patients might benefit from the removal of DPN lesions.

Intralesional Triamcinolone Acetonide in the Treatment of Traction Alopecia

Traction alopecia (TA) is a form of hair loss caused by continuous and prolonged tension to the hair, most commonly seen in Black/African American women and children who wear hairstyles that pull excessively at the frontotemporal hairline. Dermatologists have recommended the use of intralesional triamcinolone acetonide injections (ILK) to decrease the inflammatory process, however, evidence-based proof is lacking in the literature. In this case series, we evaluate the effectiveness and safety of ILK in the TA management of 6 African American women. A retrospective chart review was done of patients with a diagnosis of TA, who were treated with ILK at an academic dermatology clinic, yielding 6 patients. Management of TA was assessed by comparing the photographs for changes in hair density along the frontotemporal hairline. ILK with a concentration of 5 mg/mL, was administered in areas of low hair density along the frontotemporal hairline at 6 to 8-week intervals, for 3 successive visits. All subjects demonstrated visible increase in hair density along the frontotemporal hairline following their first or second treatment, and no severe adverse effects were observed or reported. The use of ILK is currently an effective and safe method of treating TA, particularly in the early to mid-stages. Common adverse effects are pain, and subsequent transient atrophy at the injection site. The transient atrophy is not an indication to stop treatment. Avoidance of treating dented areas is sufficient to allow it to revert. Patient education is pivotal in the prevention and management of TA. It is imperative that dermatologists caution against grooming practices that exert tension on the hairline. J Drugs Dermatol. 2020;19(2)128-130. doi:10.36849/JDD.2020.4635

Variant PADI3 in Central Centrifugal Cicatricial Alopecia.

BACKGROUND: Central centrifugal cicatricial alopecia (CCCA) is the most common form of scarring alopecia among women of African ancestry. The disease is occasionally observed to affect women in families in a manner that suggests an autosomal dominant trait and usually manifests clinically after intense hair grooming. We sought to determine whether there exists a genetic basis of CCCA and, if so, what it is. METHODS: We used exome sequencing in a group of women with alopecia (discovery set), compared the results with those in a public repository, and applied other filtering criteria to identify candidate genes. We then performed direct sequencing to identify disease-associated DNA variations and RNA sequencing, protein modeling, immunofluorescence staining, immunoblotting, and an enzymatic assay to evaluate the consequences of potential etiologic mutations. We used a replication set that consisted of women with CCCA to confirm the data obtained with the discovery set. RESULTS: In the discovery set, which included 16 patients, we identified one splice site and three heterozygous missense mutations in PADI3 in 5 patients (31%). (The approximate prevalence of the disease is up to 5.6%.) PADI3 encodes peptidyl arginine deiminase, type III (PADI3), an enzyme that post-translationally modifies other proteins that are essential to hair-shaft formation. All three CCCA-associated missense mutations in PADI3 affect highly conserved residues and are predicted to be pathogenic; protein modeling suggests that they result in protein misfolding. These mutations were found to result in reduced PADI3 expression, abnormal intracellular localization of the protein, and decreased enzymatic activity - findings that support their pathogenicity. Immunofluorescence staining showed decreased expression of PADI3 in biopsy samples of scalp skin obtained from patients with CCCA. We then directly sequenced PADI3 in an additional 42 patients (replication set) and observed genetic variants in 9 of them. A post hoc analysis of the combined data sets showed that the prevalence of PADI3 mutation was higher among patients with CCCA than in a control cohort of women of African ancestry (P = 0.002 by the chi-square test; P = 0.006 by Fisher's exact test; and after adjustment for relatedness of persons, P = 0.03 and P = 0.04, respectively). CONCLUSIONS: Mutations in PADI3, which encodes a protein that is essential to proper hair-shaft formation, were associated with CCCA. (Funded by the Ram Family Foundation and others.).

No sulfates, no parabens, and the "no-poo" method: a new patient perspective on common shampoo ingredients.

The ingredients in shampoos and other cosmetic products have become scrutinized by the general public and the Internet has contributed to misinformation about certain shampoos. Dermatologists must be prepared to acknowledge the concerns that their patients have about common shampoo ingredients to dispel the myths that may misinform patient decision-making. This article reviews the controversy surrounding the use of sulfates and parabens in shampoos, as well as commonly used shampoo alternatives, often called the "no-poo" method.

Coping Mechanisms and Resources for Patients Suffering from Rosacea.

Rosacea has significant quality of life impact. The authors review the literature and Internet sources pertaining to rosacea to identify coping mechanisms and resources available to rosacea patients. MEDLINE and PsycINFO databases were searched to identify pertinent articles. The term "rosacea" was searched in combination with "patient resources," "coping," "dealing with," "blog," "forum," "support," "nonpharmacologic," and "psychological." There are several social and educational coping resources available to rosacea patients. These may optimize quality of life and psychosocial outcomes in patients with rosacea.

Frontal Fibrosing Alopecia and Concomitant Lichen Planus Pigmentosus: A Case Series of Seven African American Women.

The association of frontal fibrosing alopecia (FFA) and lichen planus pigmentosus (LPPigm) is rare. Prior reports suggest that FFA and LPPigm are on the same spectrum of disease, and a diagnosis of LPPigm may predict the future development of FFA. We aim to further characterize the association between FFA and LPPigm by reviewing the clinical cases of seven African American women. Seven patients with FFA were diagnosed clinically by recession of frontotemporal hairline and confirmed by histopathologic examination showing lymphocyte-mediated cicatricial alopecia. LPPigm was diagnosed by clinical evaluation alone based on the characteristic morphology, color, and distribution of the lesions. It is difficult to distinguish whether halted progression of FFA was due to the success of the treatment regimen or spontaneous stabilization of disease over time. Our case series supports the theory that FFA and LPPigm likely exist on the same spectrum of disease. Our observations demonstrate a likely positive correlation between FFA and LPPigm.

J Drugs Dermatol. 2018;17(4):397-400.

Obesity in Women: The Clinical Impact on Gastrointestinal and Reproductive Health and Disease Management.

Approximately 36% of adult women in the United States are obese. Although obesity affects women similarly to men in terms of prevalence, there seem to be gender-specific differences in the pathophysiology, clinical manifestations, and treatment of obesity. Obesity is linked to comorbid diseases involving multiple organ systems, including the gastrointestinal tract, like gastroesophageal reflux disease, fatty liver disease, and gallstones. This article focuses on obesity in women, specifically the impact of obesity on gastrointestinal diseases and reproductive health, as well as the treatment of obesity in women.