Perceptions of skincare ingredients and product labels in pediatric eczema care.

Educating pediatric eczema patients and caregivers on appropriate product selection and avoidance of common irritants or allergens is a crucial aspect of eczema management. This study surveyed 80 pediatric caregivers in an academic pediatric dermatology clinic to assess influential factors in caregivers' selection of pediatric eczema-care products and identify ways to improve patient counseling on appropriate product selection and avoidance of common irritants or allergens. Caregivers frequently reported positive perceptions of commonly recommended ingredients for eczema but had inconsistent perceptions of fragrant plant oils and extracts, regardless of previous counseling on fragrance avoidance. These findings demonstrate uncertainty and misperceptions perpetuated by product labeling and a need for improved counseling strategies for avoiding fragrance and excessive product costs.

The ocular and periocular involvement of psoriasis.

Ocular manifestations of psoriasis are generally underrepresented in the literature and can often be missed by clinicians. Appreciation of eye involvement in psoriasis is especially important for dermatologists and ophthalmologists who may be closely involved with treating afflicted patients. Depending on the involved site and severity of the condition, signs and symptoms of ocular psoriasis can vary from asymptomatic erythema to debilitating scarring and vision loss. Treatment is critical to prevent disease progression and to improve the quality of life for these patients. We discuss the various clinical manifestations and treatment options for ocular complications in patients with psoriasis, including specific circumstances that should trigger immediate referral to our ophthalmology colleagues.

Atypical Wells syndrome successfully treated with dupilumab.

Wells syndrome, also referred to as eosinophilic cellulitis, is a rare and often sporadic inflammatory skin condition whose aetiology remains uncertain. Clinically, this condition presents as a collection of erythematous, oedematous, and tender skin lesions most often affecting the extremities and trunk that can mimic cellulitis. Histologically, Wells syndrome is characterised by inflammatory changes and eosinophilic infiltration of the dermis with the absence of underlying infection, thereby distinguishing it from cellulitis. Due to the rarity of this syndrome and its ambiguous presentation, there remains to be a definitive strategy for treatment. Recent case reports have documented varying success and recurrence with the use of oral and topical corticosteroids, antifungals, antibiotics, immunosuppressants and antihistamines. Here, we report a unique case of progressively worsening neutrophilic-rich Wells syndrome on the vertex of the scalp that was successfully treated with a combination of dupilumab and oral corticosteroids.

Emerging therapeutics in the management of connective tissue disease. Part I. Lupus erythematosus and Sjögren syndrome.

The management of connective tissue diseases is dramatically evolving with the advent of biologics and novel oral systemic therapeutics. Despite involvement in the care of these complex patients, there is a knowledge gap in the field of dermatology regarding these emerging agents. The first article in this continuing medical education series discusses new and emerging therapeutics for lupus erythematosus and Sjögren syndrome that target cells, intracellular signaling pathways, and cytokines.

Large Necrotic Mass in the Small Bowel.

Melanoma of unknown primary (MUP) is a melanoma found in sites other than the skin, mucosa, or eye. It is most commonly present in lymph nodes and least frequently in visceral organs. Diagnosis is difficult given its internal presentation and is often late stage with poor prognosis. We report an 89-year-old man who presented with weakness, a 27-kg weight loss, and gastrointestinal bleeding. He was found to have a large necrotic mass in the small bowel consistent with melanoma. Although he had extensive skin, anogenital, and ocular examinations, no cutaneous lesion was identified, therefore leading to a diagnosis of MUP.

Facial lipogranulomas due to self-injection of vitamin A oil.

Lipogranulomas represent foreign body reactions to exogenous lipid or oil-like substances introduced into the skin. These lesions characteristically have round-to-ovoid, vacuole-like cavities of varying sizes in the dermis, which results in a Swiss cheese-like appearance. We present the case of a 51-year-old Hispanic woman with an onset of painful, swollen, subcutaneous nodules on the face, most prominently on the right lower lip and both cheeks, after multiple self-injections of vitamin A oil. Histopathology test results of the lower lip showed a superficial-to-deep, nodular and interstitial, polymorphous inflammatory infiltrate of predominantly histiocytes with necrobiotic-type granulomatous changes, lymphocytes, and plasma cells. The cheek revealed deep dermal and subcutaneous small collections of foamy/vacuolated histiocytes, without significant numbers of other inflammatory cells. Given the patient's history of injecting oil extracted from vitamin A capsules into her skin, the light microscopic features are consistent with lipogranulomatous changes that are secondary to a local injection of foreign material. Clinicians and pathologists should be aware of the granulomatous immune reaction generated by the injection of unregulated products into the face and other areas of the body.

Muir-Torre Syndrome: A Case Report in a Woman Without Personal Cancer History.

We report a case of a 68-year-old white woman presenting with 5 sebaceous neoplasms, ranging from sebaceous adenoma to sebaceoma on histopathology. Despite the lack of a personal cancer history, her multiple sebaceous neoplasms and a paternal history of colon cancer prompted testing her sebaceous adenomas for microsatellite instability (MSI) by immunohistochemistry. The results showed retained nuclear expressions of MLH1 and PMS2 while MSH2 and MSH6 proteins were absent. The tumor infiltrating lymphocytes expressed both MSH2 and MSH6, providing reliable internal positive controls. Having a high probability for MSI, she was found to be heterozygous for a germline point mutation in MSH2 gene, where a pathologic variant, c.1165C > T (p.Arg389*), determined by sequencing confirmed Muir-Torre syndrome (MTS). On further genetic counseling recommendations, one of her 2 sons was found to have colon cancer in the context of his MTS. In this article, we highlight and review the implications of MSI testing by both immunohistochemistry and sequencing as they relate to confirming the diagnosis of a suspected case of MTS.

Cutaneous features and diagnosis of primary Sjögren syndrome: An update and review.

Sjögren syndrome (SS) is an autoimmune connective tissue disorder (CTD) that principally affects the lacrimal and salivary glands. Although SS is 1 of the 3 most common autoimmune CTDs alongside systemic lupus erythematosus and progressive systemic sclerosis, it is the least researched CTD overall. SS poses a particular diagnostic challenge because it shares multiple clinical and immunologic features with other CTDs. However, there are some characteristic cutaneous clinical features that can precede the well-known sicca symptoms by years. By familiarizing themselves with these clinical features and having a high suspicion for SS, dermatologists can play an important role in the early diagnosis and treatment of this disease.

Molecular testing practices and perceptions among dermatopathologists.

BACKGROUND: We evaluated how dermatopathologists are employing molecular testing in the setting of neoplastic skin diseases, and assessed their opinions of the broader role and utility of molecular technologies in clinical practice. METHODS: A 15-question online survey was sent to Fellows of the American Society of Dermatopathology in April 2017. RESULTS: One hundred and thirty-six dermatopathologists completed the survey (response rate = 16%). A majority (94%) of respondents reported experience with one or more molecular testing strategies. Sixty-two percent of dermatopathologists order 12 or more molecular tests per year, while 5% of respondents order 2 or fewer assays per year. More frequent utilization of molecular testing is associated with relevant instruction during residency training (P = .009), primary board certification in pathology (P = .008), academic medical center affiliation (P = <.0001), higher volume clinical practice (P = .0004), presence of on-site clinical molecular pathology/cytogenetics laboratory (P = .007), and greater physician confidence incorporating test results into histopathological assessments (P = <.0001). CONCLUSIONS: Wider adoption of molecular testing in dermatopathology may be limited by factors such as physician training, test costs/insurance coverage, logistical issues and lack of evidence-based clinical practice guidelines. Dermatopathologists have concerns regarding clinical validity/utility and inappropriate/overuse of some molecular tests. The importance of longitudinal education in molecular technologies and their applications for trainee and practicing physicians is highlighted.

Educational Gaps in Molecular Diagnostics, Genomics, and Personalized Medicine in Dermatopathology Training: A Survey of U.S. Dermatopathology Fellowship Program Directors.

BACKGROUND: Molecular technologies offer clinicians the tools to provide high-quality, cost-effective patient care. We evaluated education focused on molecular diagnostics, genomics, and personalized medicine in dermatopathology fellowship training. DESIGN: A 20-question online survey was emailed to all (n = 53) Accreditation Council for Graduate Medical Education (ACGME)-accredited dermatopathology training programs in the United States. RESULTS: Thirty-one of 53 program directors responded (response rate = 58%). Molecular training is undertaken in 74% of responding dermatopathology fellowships, with levels of instruction varying among dermatology-based and pathology-based programs. Education differed for dermatology- and pathology-trained fellows in approximately one-fifth (19%) of programs. Almost half (48%) of responding program directors believe that fellows are not currently receiving adequate molecular education, although the majority (97%) expect to incorporate additional instruction in the next 2-5 years. Factors influencing the incorporation of relevant education include perceived clinical utility and Accreditation Council for Graduate Medical Education/residency review committee (RRC) requirements. Potential benefits of molecular education include increased medical knowledge, improved patient care, and promotion of effective communication with other healthcare professionals. More than two-thirds (68%) of responding program directors believe that instruction in molecular technologies should be required in dermatopathology fellowship training. CONCLUSIONS: Although all responding dermatopathology fellowship program directors agreed that molecular education is important, only a little over half of survey participants believe that their fellows receive adequate instruction. This represents an important educational gap. Discussion among those who oversee fellow education is necessary to best integrate and evaluate teaching of molecular dermatopathology.