Clinicopathologic Characteristics of Melanoma in Patients with Parkinson Disease.

Patients with Parkinson disease (PD) are at high risk for developing melanoma, although current literature lacks details on the associated clinicopathologic characteristics. Our retrospective case-control study aimed to guide skin cancer surveillance recommendations for patients with PD, focusing on tumor sites. Our study included 70 adults with concurrent diagnoses of PD and melanoma from January 1, 2007 to January 1, 2020 at Duke University and 102 age-, sex-, and race-matched controls. The head/neck region accounted for 39.5% of invasive melanomas in the case group compared with 25.3% in the control group as well as 48.7% of noninvasive melanomas in the case group compared with 39.1% in the control group. Of note, 50% of metastatic melanomas in patients with PD originated on the head and neck (n = 3). Logistic regression showed 2.09 times higher odds of having a head/neck melanoma in our case group compared with that in the control group (OR = 2.09, 95% confidence interval = 1.13‒3.86; P = 0.020). Our study is limited by small sample size, and our case cohort lacked diversity regarding race, ethnicity, sex, and geography. Validation of the reported trends could provide more robust guidance for melanoma surveillance in patients with PD.

Meta-analysis of number needed to treat for diagnosis of melanoma by clinical setting.

OBJECTIVE: To provide a formal statistical comparison of the efficacy of melanoma detection among different clinical settings. METHODS: A systematic review and meta-analysis of all relevant observational studies on number needed to treat (NNT) in relation to melanoma was performed in MEDLINE. We performed a random-effects model meta-analysis and reported NNTs with 95% confidence intervals (CIs). The subgroup analysis was related to clinical setting. RESULTS: In all, 29 articles including a total of 398,549 biopsies/excisions were analyzed. The overall NNT was 9.71 (95% CI, 7.72-12.29): 22.62 (95% CI, 12.95-40.10) for primary care, 9.60 (95% CI, 6.97-13.41) for dermatology, and 5.85 (95% CI, 4.24-8.27) for pigmented lesion specialists. LIMITATIONS: There is heterogeneity in data reporting and the possibility of missing studies. In addition, the incidence of melanoma varies among clinical settings, which could affect NNT calculations. CONCLUSION: Pigmented lesion specialists have the lowest NNT, followed by dermatologists, suggesting that involving specialists in the diagnosis and treatment of pigmented skin lesions can likely improve patient outcomes.

A trichogenic tumor with aggressive features initially diagnosed as basal cell carcinoma.

Trichoblastic carcinoma is a rare carcinoma often arising in a pre-existing trichoblastoma. It may resemble basal cell carcinoma, posing a diagnostic challenge. Trichoblastic carcinoma is divided into low-grade and high-grade tumors. Low-grade tumors resemble basal cell carcinomas and are therefore synonymous in some classifications. High-grade tumors, which commonly present on the scalp in older individuals or in patients with Brooke-Spiegler syndrome, have been associated with a higher potential for distant metastasis and death. We present a case in which a 73-year-old female had a long-standing scalp nodule for over 30 years that rapidly increased in size. The patient's lesion was initially diagnosed as basal cell carcinoma on shave biopsy, but upon excision, revealed features concerning for trichoblastic carcinoma such as brisk mitotic activity and comedo-like necrosis. Sudden change in an atypical scalp lesion that has been present for many years should increase suspicion for an atypical trichogenic tumor, such as trichoblastic carcinoma.

Multiple Clustered Dermatofibromas Associated With Pulmonary Arterial Hypertension.

BACKGROUND: Dermatofibromas (DFs) are common, benign, fibrohistiocytic tumors of the skin. Clinically, if they present in a rapid succession, they are termed multiple eruptive DFs. When they arise in a localized distribution, they are termed multiple clustered dermatofibromas (MCDFs). These DF variants are rare, and the subtype of multiple eruptive DFs has been associated with autoimmune diseases, hypertriglyceridemia, pregnancy, HIV, and primary pulmonary hypertension. Conversely, there is a paucity of published clinical associations with MCDF. METHODS: A 23-year-old white woman with pulmonary arterial hypertension presented with a 7-year history of multiple, asymptomatic, firm, brown, and violaceous papules clustered on the right hip. RESULTS: Histopathologic examination of the biopsies demonstrated acanthotic epidermis, proliferation of cytologically bland fibrohistiocytic spindle cells in a fascicular pattern in the dermis, and collagen trapping on the periphery. Immunohistochemistry was focally positive for factor XIIIa and negative for CD34, thus supporting the clinical diagnosis of MCDF. CONCLUSIONS: A case of MCDF associated with pulmonary arterial hypertension is presented. To date, there have been greater than 25 reported cases of MCDF, but only one other publication has described an associated systemic comorbidity.

Congenital candidiasis: an uncommon skin eruption presenting at birth.

We present the case of a preterm neonate who was born with respiratory distress and a papulovesicular rash that was diagnosed as congenital candidiasis (CC). The mother was asymptomatic. The cutaneous eruption and respiratory distress improved following treatment with systemic antifungals. Congenital candidiasis ranges in presentation from isolated cutaneous involvement to severe multisystem disease. Given its rarity among neonatal skin eruptions, heightened suspicion is required for prompt diagnosis and treatment.

Isotretinoin-induced skin fragility in a teenaged athlete: a case report.

Isotretinoin is approved by the US Food and Drug Administration for the treatment of severe recalcitrant nodular acne. Mucocutaneous toxicity is the most commonly observed side effect of isotretinoin use. Because atrophy and skin fragility may occur while taking isotretinoin, most warnings recommend avoidance of cosmetic procedures, such as dermabrasion, laser treatments, waxing, and chemical peels. We report a case of isotretinoin-induced skin fragility in a 16-year-old adolescent boy who presented with an unusual amount of skin erosions and excoriations on his face during wrestling season. We propose that it may be prudent to advise athletes who are involved in contact sports that skin fragility and increased erosions may occur during or after their course of treatment with isotretinoin.

Resource utilization and quality of life associated with congenital ichthyoses.

We explored resource utilization (ResUtil) and quality of life (QOL) associated with congenital ichthyoses (CI). Subjects completed an online survey related to clinical severity, demographics, ResUtil, and QOL as measured according to the Dermatology Life Quality Index (DLQI). Validated Likert scales were used to evaluate severity of hyperkeratosis, erythema, and alopecia. ResUtil was determined according to time spent daily treating CI symptoms (TimeTx) and number of ichthyosis-related dermatology visits (DermVisits) per year. We used linear regression to investigate predictors of a transformed DLQI (sqrtDLQI) and logistic regression for ResUtil. Of 235 subjects, 60.2% were female, 83.8% were Caucasian, 42.3% had a family history (FamHx) of CI, and the mean age was 28.7 years (SD 20.3). Predictors for worse QOL were hyperkeratosis severity (ß = 0.27, p < 0.01), erythema (ß = 0.27, p < 0.01), TimeTx (ß = 0.21, p < 0.01), ichthyosis type (ß = 0.09, p < 0.01), and age (ß = 0.01, p = 0.02). Predictors for DermVisits were hyperkeratosis severity (odds ratio [OR] = 1.38, 95% confidence limit [CL] = 1.01, 1.87), FamHx (OR = 0.28, 95% CL = 0.09, 0.85), age (OR = 0.97, 95% CI = 0.96, 0.99), and alopecia severity (OR = 1.43, 95% CL = 1.12, 1.82). Predictors for treatment duration were erythema (OR = 1.35, 95% CL = 1.02, 1.78), age (OR = 0.98, 95% CL = 0.96, 0.99), and DLQI (OR = 1.09, 95% CL = 1.03, 1.15). Increased hyperkeratosis severity and erythema negatively impact QOL in the CI. Furthermore, increased disease severity predicted greater ResUtil, whereas increased age and FamHx predicted less ResUtil. Our findings suggest that better therapies and increased patient education may improve QOL and decrease ResUtil.

The public's perception of dermatologists as surgeons.

BACKGROUND: Dermatologists perform more cutaneous surgical procedures than any other medical specialists, including plastic surgeons, especially for treating skin cancers, but anecdotal evidence suggests that the public may not identify dermatologists as surgeons. OBJECTIVE: Our study was designed to assess the public's perception of expertise in surgery of the skin of three medical specialties: dermatology, plastic surgery, and general surgery. We also investigated whether the physician's specialty biases people when they assess the cosmetic appearance of a surgical scar. MATERIALS AND METHODS: We administered an institutional review board-approved survey to individuals at the Emory Student Center and the Emory Dermatology Clinic. Participants rated the perceived skills and training of the different medical specialties and scored the cosmetic appearance of 16 surgical scars created by a fellowship-trained Mohs surgeon labeled as the work of different specialists. RESULTS: Results from 467 participants were overwhelmingly in favor of plastic surgeons (p<.001). The physician's specialty did not bias participants in assessing the cosmetic appearance of surgical scars. CONCLUSION: The study population had greater confidence in the surgical skills of plastic surgeons than in those of dermatologists, although participants were objective in rating the cosmesis of surgical scars, regardless of the purported surgeon's specialty. Although dermatologic surgeons must continually refine our surgical expertise, we must also educate the public about the breadth and depth of our work. The authors have indicated no significant interest with commercial supporters.

Quality of life of cutaneous disease in the ectodermal dysplasias.

The ectodermal dysplasias are a complex, heritable group of syndromes that affect derivatives of ectoderm. The dermatologist plays an important role in ectodermal dysplasias as the visible defects of skin, hair, and nails are often recognized first. Our objective was to examine how quality of life relates to the degree of skin involvement in ectodermal dysplasias. Subjects (n = 42) with ectodermal dysplasias were surveyed at national and regional conferences hosted by National Foundation for Ectodermal Dysplasias on July 26 to 28, 2007 and November 17, 2007. Severity of hypohidrosis, alopecia, and fingernail involvement were measured using validated Likert scales. The quality of life was measured using the Children's Dermatology Life Quality Index in children, and the Skindex-29 and RAND Short Form-36 in adults. The severity for all subjects (n = 42) was greatest for hypohidrosis, which clinically translated into "little ability to sweat." We found that the greatest impact on quality of life was related to the actual symptoms of ectodermal dysplasias, which is not surprising as almost all participants reported skin involvement (93%), most likely related to hypohidrosis, atopic dermatitis, skin erosions and infections. The symptoms of ectodermal dysplasias may also be related to the nail involvement, as manifested by dystrophic or fragmented nails. Alopecia did not play a significant role in the quality of life burden. The challenge of providing proper skin care emphasizes the benefit of dermatologic involvement, and the need for increased recognition and enhanced awareness of the cutaneous burden in ectodermal dysplasias.

Reliable methods to evaluate the clinical severity of ichthyosis.

A reliable method for assessing ichthyosis severity has not been uniformly agreed upon. The objective of our study was to develop and validate a tool to measure the clinical severity of the congenital ichthyoses, the Congenital Ichthyoses Severity Index. A prospective study was performed to determine reliability of three Likert scales to evaluate clinical severity of ichthyosis. Thirty-eight subjects recruited from the Foundation for Ichthyosis and Related Skin Types National Conference were evaluated separately by two blinded investigators using the Likert scales. Subjects were then asked to evaluate themselves using these scales. Inter-rater reliability was determined between ratings provided by all three raters. Test-retest validation was conducted with 21 subjects who completed follow-up surveys 4 weeks later. Our severity scales had excellent inter-rater and test-retest reliability as determined by intraclass correlation coefficients (ICC >0.7), with the exception of our hyperkeratosis scale, which demonstrated moderate test-retest reliability (ICC = 0.4). This pilot study provides a promising method for evaluating clinical severity of the congenital ichthyoses, one easily employed by both physicians and patients. Future epidemiologic studies may benefit from use of this instrument, as well as studies evaluating emerging therapies for ichthyosis.

Annual direct and indirect health costs of the congenital ichthyoses.

The aim of this study is to estimate annual, per patient, health care costs for congenital ichthyoses (CI). We conducted a cost analysis through an online survey posted on the Foundation for Ichthyosis and Related Skin Types Website. We assessed cutaneous disease severity, via the previously validated Congenital Ichthyosis Severity Index (CISI), demographics, and CI type. We estimated direct health care costs: prescription and over-the-counter medications, outpatient visits, and emergency department and hospital visit costs; and indirect costs: earnings lost owing to absences from work because of CI-related illness. The CI subjects of our study (n=224) consumed a mean (SD) of $3,192 ($7,915) annually. Direct costs accounted for 90%, whereas indirect costs accounted for 10%. These costs resulted in an estimated annual cost of $37 MM/year (excluding ichthyosis vulgaris) of which $17 MM is borne out-of-pocket by patients. Depending on the CI diagnosis, patients were responsible for 30-51 cents of every dollar of mean annual medical care costs. Our estimated annual CI costs are comparable to cutaneous lymphoma. More effective treatments for CI would help minimize this burden. Traditional insurance products do not appear to substantially alleviate the financial burden of disease, as a significant amount is from out-of-pocket expenses.

Dysregulation of the mTOR pathway secondary to mutations or a hostile microenvironment contributes to cancer and poor wound healing.

Either heredity mutations or adverse microenvironment conditions may result in dysregulation of the mammalian target of the rapamycin (mTOR) pathway. The former lead to clinical syndromes such as tuberous sclerosis, Peutz-Jeghers syndrome, and Cowden's disease, which are characterized by hamartomatous growth or cancer. The latter can be associated with poor wound healing as described by Goren et al. (2009, this issue).

l-DOPA improves odor discrimination learning in rats.

Using a forced-choice odor discrimination task in rats, we show here that intraperitoneal injections of l-DOPA improve rats' odor discrimination performance in comparison to control rats injected with saline. Although the exact mechanism of l-DOPA and potential action on dopamine receptors remains unclear, the present results indicate that rats injected with the 20-50 mg/kg of l-DOPA performed significantly better in an odor discrimination task than did control rats. In addition, we observed a significant correlation between the concentration of l-DOPA and odor discrimination performance.

Opposing effects of D1 and D2 receptor activation on odor discrimination learning.

Dopaminergic modulation of cortical activity has been implicated in the formation of reward associations. There is abundant evidence for dopaminergic effects on olfactory processing. Using an olfactory discrimination task, the authors show that D1 and D2 dopamine receptors can regulate rats' olfactory discrimination capacities and that the effects of receptor activation functionally oppose one another. Injection of either the D1 agonist SKF 38393 (10 mg/kg) or the D2 antagonist spiperone (0.62 mg/kg) facilitated the discrimination of similar odorants but had no effect on the discrimination of dissimilar odorants, whereas both the D, antagonist SCH 23390 (0.025 mg/kg) and the D2 agonist quinpirole (0.2 mg/kg) significantly impaired rats' ability to discriminate similar and dissimilar odorants.