Linear scleroderma "en coup de sabre" with extensive brain involvement-Clinicopathologic correlations and response to anti-Interleukin-6 therapy.

Linear scleroderma "en coup de sabre" (LSES) variant is a cephalic subtype of localized scleroderma that can be associated with extracutaneous stigmata, such as epilepsy, dementia syndromes, as well as focal central nervous system neurologic deficits. While the pathophysiology of cutaneous linear scleroderma includes endothelial cell injury and up regulation of pro-fibrogenic pathways, the basis of LSES-associated neurologic complications is largely unknown. We report a patient with a history of LSES who developed intractable epilepsy and cognitive decline. Magnetic resonance imaging (MRI) of the brain exhibited numerous persistently enhancing brain lesions. Due to progressive neurologic deterioration over a period of 7 years, despite interventional therapy, a brain biopsy was performed. Neuropathologic analysis exhibited acute and chronic cortical ischemia associated with a small vessel lymphocytic vasculitis. Direct immunofluorescent studies showed C5b-9 and IgG deposition on endothelium while indirect immunofluorescent studies demonstrated reactivity of the patient's serum with the microvasculature of the patient's own brain tissue and generic human umbilical vein endothelial cells indicative of anti-endothelial cell antibodies. Therapy focusing on damaged endothelium was implemented. The interleukin-6 (IL-6) receptor inhibitor tocilizumab was used and the patient improved dramatically, likely reflecting the drug's effect on the replenishment of endothelial progenitor cells.

Pediatric Onychophagia: A Survey-Based Study of Prevalence, Etiologies, and Co-Morbidities.

BACKGROUND: Onychophagia, defined as habitual nail biting, is a common disorder affecting 6-45% of the population and is more prevalent in children. OBJECTIVES: Our primary objective was to determine the prevalence of nail biting in the pediatric population. Secondary objectives were to assess the presence of psychiatric co-morbidities associated with nail biting, and the effect of treatment on nail biting. METHODS: An anonymous voluntary survey was administered to participants at an outpatient academic pediatric clinic at Weill Cornell Medicine. Age, sex, psychiatric diagnosis, treatment sought, family history, and frequency of nail biting were analyzed across 282 enrolled patients aged 3-21 years. One patient was subsequently excluded due to incomplete data. RESULTS: Of 281 patients, 101 (37%) reported past or present nail biting lasting more than a month. Median age of onset was 5 years old (range 1-13 years). A significantly higher percentage of biters (18%; 19/104) than non-biters (6%; 11/177) were diagnosed with a psychiatric disorder (p < 0.01). Amongst biters, concurrent fingernail and toenail involvement was much less common (12%; 12/104) than that of fingernails alone (88%; 92/104). However, the ratio of fingernail and toenail biters to fingernail biters alone was greater in those with psychiatric diagnosis (0.36) than without (0.09) (p = 0.07). CONCLUSIONS: Our study highlights behavioral patterns as well as familial, psychiatric, and other factors associated with pediatric nail biting. Familiarity with such factors, the clinical presentation of onychophagia, and available treatment options may aid in reducing its severity in affected patients and overall prevalence.

Onychotillomania: Diagnosis and Management.

Onychotillomania, defined as self-induced trauma to the nail unit, either by picking or pulling at the nails, affects 0.9% of the population. It may lead to severe irreversible nail dystrophy, melanonychia, or infections. Although no large clinical trials have assessed the efficacy of treatments, cognitive-behavioral therapy, physical barrier methods, and pharmacological treatments have shown some benefits in case reports. The objective of this article is to review the prevalence, diagnostic criteria, etiology, historical and physical examination findings, pathological features, and current treatment methods. Onychotillomania remains a clinical challenge to dermatologists, pediatricians, internists, and psychiatrists in practice, as there are no evidence-based treatment methods.

Blastomycosis during pregnancy: a case report and review of the literature.

The diagnosis of blastomycosis during pregnancy is rare, but can carry significant clinical questions for both the infected mother and developing fetus. Furthermore, given its rarity, providers have little available data to help manage and counsel patients in this uncommon, yet serious, scenario. We present a case of blastomycosis in a woman at 38weeks' gestation and review all published cases of blastomycosis during pregnancy. It is our hope to provide a multidisciplinary understanding of the current knowledge surrounding the presentation, diagnosis, management, and outcome of this unusual infection.

A retrospective study on rapid plasma reagin testing in patients with Pityriasis rosea.

Pityriasis rosea (PR) is an acute self-limited exanthem characterized by oval erythematous patches with scale and may be difficult to differentiate from secondary syphilis. A rapid plasma reagin (RPR) test can be used to rule in secondary syphilis with high sensitivity and specificity. A retrospective study was performed on patients at Weill Cornell Medicine, who were diagnosed with PR from 2000 to 2016 and also received RPR testing at the time of diagnosis. The objective was to assess the frequency of secondary syphilis when the initial clinical impression was PR. Only 2/142 patients (1.4%) had a reactive RPR test. Based on our results, we advocate that careful social and sexual histories be taken in all patients presenting with atypical PR and syphilis screening performed if risk factors are present.

Onychopapilloma Presenting as Leukonychia: Case Report and Review of the Literature.

Onychopapilloma is a benign tumor of the nail bed and distal matrix and is the most common cause of localized longitudinal erythronychia. Here, we describe a case of onychopapilloma presenting as longitudinal leukonychia of the left 4th fingernail in a 71-year-old female. To date, this is only the ninth described case of onychopapilloma presenting as longitudinal leukonychia. We review the literature on the reported cases and provide evidence that longitudinal leukonychia as the presenting sign for onychopapilloma is becoming increasingly recognized in clinical practice.

Assessment of Patient Knowledge of Longitudinal Melanonychia: A Survey Study of Patients in Outpatient Clinics.

IMPORTANCE: Subungual melanoma (SM) is a rare subtype of cutaneous melanoma but carries a worse prognosis than similarly staged cutaneous melanomas. Assessing patient knowledge of melanonychia is integral to early diagnosis of SM. OBJECTIVES: The aim of this paper is to determine patient knowledge of longitudinal melanonychia (LM) and warning signs for SM, frequency of nail self-examinations, and satisfaction of patients with their physician's nail examinations. DESIGN SETTING AND PARTICIPANTS: We conducted a survey-based study of 363 random patients at Weill Cornell Medicine in New York, USA, performed at 3 different clinics: a general dermatology clinic (n = 167), a nail specialty clinic (n = 44), and a primary care clinic (n = 152). MAIN OUTCOMES AND MEASURES: Knowledge of the ABCDEF mnemonic for SM was compared to the ABCD mnemonic for cutaneous melanoma. Analyses were performed for patient behavior regarding suspicious nail changes as well as satisfaction with nail counseling and examination. RESULTS: Only 5% (18/363) of the patients in our study had heard of the ABCDEF mnemonic. In contrast, 9.9% (36/363) of the patients had heard of the ABCD mnemonic for cutaneous melanoma. In total, 37/363 (10.2%) patients reported having LM, 32.4% (12/37) of the patients noted changes in color or width of the band, and 10.8% (4/37) presented with pain or bleeding of the nail, with only 45.9% (17/37) seeking medical attention. Only 11.8% (43/363) of the patients stated that their physician asked them about nail changes, and 1.4% (5/363) of the patients stated that they were counseled about the ABCDEF mnemonic. In comparison, 13.8% (50/363) of the patients were advised on the ABCD mnemonic for the cutaneous melanoma mnemonic. While 70.2% (255/363) of the patients stated that they used sunscreen or wore sun-protective clothing, only 31.4% (114/363) assessed their nails for color changes, with 54.9% (128/233) of the patients categorizing themselves as "very unsatisfied" with the evaluation of their nails by their dermatologist. CONCLUSIONS AND RELEVANCE: Our data shows that there is a lack of patient knowledge of LM and warning signs for SM. Further testing is needed to determine whether educating patients about LM, warning signs for SM, and nail self-examinations would improve patient outcomes.

Onychophagia: A nail-biting conundrum for physicians.

Onychophagia, defined as habitual nail biting, is a common disorder affecting 20-30% of the population and all age groups. It may lead to significant psychosocial problems, have a negative impact on quality of life, and cause complications involving both the nail unit and the oral cavity. The objective of this paper is to review the prevalence, etiology, history, physical examination, complications and management of nail biting. Since onychophagia is a challenging disorder to treat, a multi-disciplinary approach should be taken involving dermatologists, internists, pediatricians, psychiatrists and dentists.

Over-the-counter and natural remedies for onychomycosis: do they really work?

Onychomycosis is a fungal infection of the nail unit that may lead to dystrophy and disfigurement over time. It accounts for up to 50% of all nail conditions, with toenails affected more commonly than fingernails. Onychomycosis may affect quality of life and increase the prevalence and severity of foot ulcers in patients with diabetes. Available oral agents approved by the US Food and Drug Administration (FDA) for the treatment of onychomycosis include terbinafine and itraconazole, which have demonstrated good efficacy but are associated with the risk of systemic side effects and drug-drug interactions. Topical medications that are FDA approved for onychomycosis include ciclopirox, efinaconazole, and tavaborole. These therapies generally have incomplete efficacy compared to systemic agents as well as long treatment courses and possible local side effects such as erythema and/or blisters. Given the need for safe, effective, and cost-effective options for onychomycosis therapy, there has been a renewed interest in natural and over-the-counter (OTC) alternatives. This review will synthesize the laboratory data, known antifungal mechanisms, and clinical studies assessing the efficacy of OTC and natural products for onychomycosis treatment.

Subungual pleomorphic fibroma: a case report and review of the literature.

We describe an interesting case of pleomorphic fibroma of the subungual region in a middle aged woman who presented with a recurrence of thickening, lateral ridging, and a midline split of the right third fingernail, 20 years after initial excision. Histology of the specimen demonstrated hyperchromatic stellate cells within the superficial corneum, which were CD34 positive, consistent with a pleomorphic fibroma. Pleomorphic fibroma is a rare mesenchymal neoplasm characterized by atypical spindled cells amidst a collagenous stroma. It is a benign, slow growing tumor that has only been reported in the subungual region twice previously. Although histology may show cellular atypia, the slow-growing benign nature of the tumor, reassuring features histologically i.e low cellularity, and lack of myxomatous change in the stroma, make a conservative approach to therapy reasonable.