Erythromelalgia: A Review of Medical Management Options and Our Approach to Management.

Erythromelalgia (EM) is a rare disorder characterized by episodic, burning pain associated with erythema and warmth of the extremities. The feet and hands are most commonly affected. The pain can be so severe that patients may engage in behaviors, sometimes extreme, to cool the affected areas and change their lifestyle to avoid precipitating factors, such as exercise and increased ambient heat. A literature search was performed with PubMed and MEDLINE with the search term erythromelalgia. Inclusion criteria were studies on EM published after 1985 until January 1, 2022, in the English language and studies that provided information on medical treatment of EM. Studies were excluded if they were duplicates or did not include treatment data. No guidelines exist for the treatment of this complex disorder. Lifestyle modifications and pharmacologic treatments (topical and systemic) are discussed in this article, which provides a comprehensive review of published medical management options for erythromelalgia and a proposed approach to management.

Incidence of squamous cell carcinoma in oral lichen planus: a 25-year population-based study.

BACKGROUND: Oral lichen planus (OLP) is a chronic inflammatory condition of the oral mucosa. Multiple studies have shown that approximately 1% of patients with OLP will develop oral squamous cell carcinoma (OSCC), however, no study has taken a population-based multicenter approach to demonstrate this association. Our main objective was to determine the incidence of OSCC in OLP in a specific population and secondarily to assist physicians regarding appropriate long-term monitoring of patients with OLP. METHODS: We conducted a population-based retrospective cohort study. Patients with OLP from 1986 through 2010 were identified using the Rochester Epidemiology Project (REP) for Olmsted County, Minnesota. For each OLP case (n = 303), we randomly selected two age- and gender-matched referents (n = 606). OLP diagnosis was established based on the World Health Organization (WHO) criteria. Medical records were reviewed for development of OSCCafter the OLP diagnosis (index date). The association between OLP and development of OSCC was assessed. RESULTS: In total, 303 patients with incident OLP were identified; the overall incidence of OLP per 100,000 person-years was 11.4 (95% CI, 10.1-12.7). Among the OLP cohort, 7 had OSCC (incidence of OSCC, 3.1%; 95% CI, 0.6-6.4%) at 20 years after OLP diagnosis. Three OSCC cases were identified among the referents. Patients with OLP were 4.8 times more likely to have OSCC than the matched referents. The median time to OSCC development was 14.7 years earlier for the OLP cohort. CONCLUSIONS: Patients with OLP, particularly the erosive type, have an increased incidence of OSCC development and should be monitored closely.

Prevalence of allergen sensitization detected by patch tests.

BACKGROUND: Existing literature on the prevalence of positive reactions to allergens is largely derived from dermatologists who practice at large academic centers. Data from other providers, including allergists who practice in various other settings, is important to assess a more representative and accurate prevalence of contact allergy. OBJECTIVE: To determine the prevalence of contact allergy among individuals with at least one positive patch test result by comparing data for positive patch test reaction rates of common contact allergens from 3 groups based in different practice settings, 2 of which are academic. METHODS: We retrospectively analyzed patch test results of an academic center (January 1, 2011, to December 4, 2015) and a national contact allergen database (March 1, 2015, to September 1, 2016). Data from a third, academic-based group was obtained separately from the published literature. Logistic regression analysis was used to compare positive reaction rates of the widely available, patch test allergens among the 3 groups. RESULTS: The positive reaction rates for 10 of 36 compared allergens (28%) were significantly higher (p < 0.05) for the national contact allergen database compared with both the academic groups. CONCLUSION: Positive reaction rates to common allergens used in patch testing may be underreported in the literature. Limitations of our study included the retrospective nature of the study, different date ranges among the three groups, and the absence of all allergens tested by the national contact allergen database.

Clinical Features, Etiologic Factors, Associated Disorders, and Treatment of Palmoplantar Pustulosis: The Mayo Clinic Experience, 1996-2013.

OBJECTIVE: To further characterize clinical characteristics, etiologic factors, associated disorders, and treatment of palmoplantar pustulosis (PPP). PATIENTS AND METHODS: We conducted a retrospective review of patients with PPP at Mayo Clinic between January 1, 1996, and December 31, 2013. RESULTS: Of 215 patients with PPP identified, 179 (83%) were female, and the mean age at onset was 45.3 years. Most patients (n=165, 77%) were current or former smokers. At diagnosis, 15 patients (7%) had an anxiety diagnosis and 9 (4%) had an infection. Nineteen cases (9%) were drug induced. Comorbid conditions included thyroid disease in 18 patients (8%), gluten sensitivity in 3 (1%), and type 2 diabetes mellitus in 21 (10%). In all, 194 patients (90%) received topical corticosteroids, 55 (26%) received phototherapy, and 54 (25%) received systemic agents. CONCLUSION: More than three-fourths of the patients in this study had a history of smoking, which is considered a triggering or aggravating factor for PPP. Regarding comorbid conditions, gluten sensitivity and thyroid disease were found less frequently than previously reported in the literature. Treatment regimens and responses in this cohort varied considerably.

What's new with common genetic skin disorders?

Familiar genetic disorders such as neurofibromatosis type I (NF1), tuberous sclerosis complex (TSC), oculocutaneous albinism (OCA), basal cell nevus syndrome (BCNS), incontinentia pigmenti, ichthyosis, and epidermolysis bullosa (EB) have prominent, cutaneous manifestations. This review describes recent advances in knowledge concerning the pathophysiology, diagnosis, and treatment of these skin features. Specifically, clinical diagnostic criteria for incontinentia pigmenti, ichthyosis, and tuberous sclerosis have been updated. Additionally, there have been considerable advancements in the technology used in the molecular diagnoses of these conditions. In the case of TSC, the discovery that a portion of TSC mutations are missed due to mosaicism is driving the development of new diagnostic methods. Also, scientists are also seeking minimally invasive methods of genetic diagnosis, as in the case of using hair follicles to diagnose autosomal recessive congenital ichythosis (ARCI). Finally, there are innovative targeted medical therapies being developed that serve as promising tools in the care of patients afflicted with conditions including ichthyosis and EB.

Dermatomyofibromas Arising in Children: Report of Two New Cases and Review of the Literature.

Dermatomyofibroma is a rare, benign mesenchymal proliferation not commonly reported in children. Two patients with biopsy-proven dermatomyofibroma were identified (one female, one male) at our institution, both with rather atypical clinical presentations. The clinical and histopathologic findings and a review of the literature are presented here. Features of dermatomyofibroma should be recognized and differentiated from those of similar-appearing entities since the prognosis of this benign tumor is favorable after complete excision.

Increasing Incidence of Melanoma in the Elderly: An Epidemiological Study in Olmsted County, Minnesota.

OBJECTIVE: To estimate the age- and sex-adjusted incidence of melanoma in adults 61 years or older in Olmsted County, Minnesota, from 1970 through 2009. PATIENTS AND METHODS: Using Rochester Epidemiology Project resources, 397 patients were identified who were 61 years or older and who received a first lifetime diagnosis of melanoma from January 1, 1970, through December 31, 2009, in Olmsted County. The incidence of melanoma and the overall and disease-specific survival rates were compared by age, sex, year of diagnosis, and stage of disease. RESULTS: From 1970 through 2009, age- and sex-adjusted incidence increased significantly (P<.001) from 17.0 (95% CI, 8.6-25.4) to 124.6 (95% CI, 108.9-140.3) per 100,000 person-years, with a 4-fold increase in women and a more than 11-fold increase in men. In men, incidence rates increased with age (P<.001) and over time (P<.001). In women, incidence rates increased over time (P<.001) but were constant across all age groups studied (P=.90). The dramatic increase in the incidence of melanoma was observed mainly for stages 0 and I tumors in both men and women (>55-fold increase). Disease-specific survival increased across the decades (P<.001); when year of diagnosis was compared to mortality, hazard ratios were 0.94 (95% CI, 0.90-0.99; P=.010) and 0.93 (95% CI, 0.89-0.98; P=.006) for men and women, respectively. CONCLUSION: The incidence of melanoma in older men and women increased significantly over the past 4 decades in Olmsted County, with men experiencing higher rates of increase than did women.

Green tea, black tea, and oolong tea polyphenols reduce visceral fat and inflammation in mice fed high-fat, high-sucrose obesogenic diets.

Green tea (GT) and caffeine in combination were shown to increase energy expenditure and fat oxidation, but less is known about the effects of black tea (BT) and oolong tea (OT). This study investigated whether decaffeinated polyphenol extracts from GT, BT, and OT decrease body fat and inflammation in male C57BL/6J mice fed high-fat/high-sucrose [HF/HS (32% energy from fat, 25% energy from sucrose)] diets. Mice were fed either an HF/HS diet with 0.25% of polyphenol from GT, OT, or BT or a low-fat/high-sucrose [LF/HS (10.6% energy from fat, 25% energy from sucrose)] diet for 20 wk. Monomeric tea polyphenols were found in the liver and adipose tissue of mice fed the HF/HS diet with GT polyphenols (GTPs) and OT polyphenols (OTPs) but not BT polyphenols (BTPs). Treatment with GTPs, OTPs, BTPs, and an LF/HS diet led to significantly lower body weight, total visceral fat volume by MRI, and liver lipid weight compared with mice in the HF/HS control group. Only GTPs reduced food intake significantly by ∼10%. GTP, BTP, and LF/HS-diet treatments significantly reduced serum monocyte chemotactic protein-1 (MCP-1) compared with HF/HS controls. In mesenteric fat, monocyte chemotactic protein-1 (Mcp1) gene expression was significantly decreased by treatment with GTPs, BTPs, OTPs, and an LF/HS diet and in liver tissue by GTP and BTP treatments. Mcp1 gene expression in epididymal fat was significantly decreased by the BTP and LF/HS diet interventions. In epididymal fat, consistent with an anti-inflammatory effect, adiponectin gene expression was significantly increased by GTPs and OTPs. Angiogenesis during adipose tissue expansion is anti-inflammatory by maintaining adipocyte perfusion. We observed significantly increased gene expression of vascular endothelial growth factor A by GTPs and vascular endothelial growth factor receptor 2 by BTPs and the LF/HS diet and a decrease in pigment epithelium-derived factor gene expression by OTPs and BTPs. In summary, all 3 tea polyphenol extracts induced weight loss and anti-inflammatory and angiogenic effects, although the tissue content of polyphenols differed significantly.

Merkel cell carcinoma in immunosuppressed patients.

Merkel cell carcinoma (MCC) is a rare and aggressive cutaneous malignancy. The infectivity of Merkel cell polyomavirus (MCPyV), an apparent agent in MCC development, may be exacerbated with impaired immune responses. This paper reviews relevant data regarding the role of immunosuppression in the development of MCC and describes modes of immunodeficient states. Because of the inherently low incidence rate of MCC, several case studies and series are also briefly mentioned to provide a more comprehensive summary of MCC in the setting of immunosuppression. We describe immunosuppressed patients who have experienced excessive UV radiation, organ transplantation, human immunodeficiency virus infection/AIDS, autoimmune diseases, and lymphoproliferative disorders. Iatrogenic forms of immunosuppression are also highlighted. Studies that quantify risks consistently report that individuals with a history of solid organ transplantation, autoimmune diseases, AIDS, and/or lymphoproliferative diseases have a significantly elevated risk of developing MCC. Overall, immunocompromised patients also appear to have an early onset and more aggressive course of MCC, with poorer outcomes. Recommendations for multidisciplinary approaches are proposed to effectively prevent and manage MCC in these patients.