Rachael Cayce

Distinct Autoimmune Syndromes in Morphea: A Review of 245 Adult and Pediatric Cases

OBJECTIVE To determine the prevalence of extracutaneous manifestations and autoimmunity in adult and pediatric patients with morphea. DESIGN A retrospective review of 245 patients with morphea. SETTING University of Texas Southwestern Medical Center-affiliated institutions. Patients Patients with clinical findings consistent with morphea. MAIN OUTCOME MEASURES Prevalence of concomitant autoimmune diseases, prevalence of familial autoimmune disease, prevalence of extracutaneous manifestations, and laboratory evidence of autoimmunity (antinuclear antibody positivity). Secondary outcome measures included demographic features. RESULTS In this group, adults and children were affected nearly equally, and African Americans were affected less frequently than expected. The prevalence of concomitant autoimmunity in the generalized subtype of morphea was statistically significantly greater than that found in all other subtypes combined (P = .01). Frequency of a family history of autoimmune disease showed a trend in favor of generalized and mixed subgroups. The linear subtype showed a significant association with neurologic manifestations, while general systemic manifestations were most common in the generalized subtype. Antinuclear antibody positivity was most frequent in mixed and generalized subtypes. CONCLUSIONS High prevalences of concomitant and familial autoimmune disease, systemic manifestations, and antinuclear antibody positivity in the generalized and possibly mixed subtypes suggest that these are systemic autoimmune syndromes and not skin-only phenomena. This has implications for the management and treatment of patients with morphea.

UVA1 phototherapy is effective in darker skin: A review of 101 patients of Fitzpatrick skin types I-V

Background  Studies suggest ultraviolet (UV) A1 phototherapy is efficacious and safe in treating a variety of skin disorders. However, most reports evaluating the benefits of UVA1 phototherapy have been from Europe, focusing on a predominantly Caucasian population. Darker skin types have been evaluated only sparingly; none the less, it is widely held that these patients respond poorly to UVA1 phototherapy due to increased pigmentation.

The Integrated Skin Exam film: An educational intervention to promote early detection of melanoma by medical students

BACKGROUND Knowledge of the skin cancer examination (SCE) and its practice remain relevant competency gaps among medical students. OBJECTIVE We elaborate on a method of SCE known as the Integrated Skin Exam and discuss the development of an instructional film that illustrates its principles. We assess the tools effect on knowledge, attitudes, and perceptions related to the SCE. METHODS Second-year students among 8 randomized schools viewed the film and completed pre-post questionnaires. RESULTS After viewing The Integrated Skin Exam film, students demonstrated improved melanoma knowledge, including identification of high-risk demographic groups (61% vs 42.9%, P < .001), high-risk anatomic sites in women (88.6% vs 46.5%, P < .001) and men (92.1% vs 34.8%, P < .001), and the ABCDEs of melanoma (98.4% vs 91.2%, P < .001). Students demonstrated increased confidence in the SCE (66.93% vs 16.40%, P < .001) and augmented intentions to practice it (99.05% vs 13.9%, P < .001). A greater proportion (70.4% vs 41.9%, P < .001) of students thought less than 3 minutes were required to integrate SCE into the routine examination. LIMITATIONS Longitudinal impact of the film was not assessed. CONCLUSION The Integrated Skin Exam film introduces an integrated approach to the SCE that addresses knowledge gaps, mitigates perceived barriers, and augments intention related to practice of the SCE.

Metastatic pleomorphic sarcoma to left atrium

Although several thousand patients are diagnosed with sarcoma annually in the United States, metastases to the heart are very uncommon. In this case report, an overall low frequency cancer presents masquerading with common cardiac symptomology. This case illustrates the importance for detailed diagnostic cardiac evaluations and heightened suspicion by physicians to consider metastatic disease to the heart in cancer patients with cardiovascular complications. Also discussed is a review of surgical and chemotherapeutic options for this problem.

A painful eruption in a woman with Darier disease

dyshidrosiform pemphigoid, antibodies to the hemidesmosomes of the basement membrane predominate; in BrunstingePerry cicatricial pemphigoid, the anchoring filament component laminin 332 is also reported. Localized, nonscarring bullous pemphigoid has been described to arise spontaneously and at areas of trauma. Nontraumatic bullae most commonly arise in the pretibial area and either remain localized or precede more generalized bullous pemphigoid. No localizing mechanism has been described, although speculation regarding vascular disease in the lower extremities has been proposed. Localized traumatic bullous pemphigoid has also been described as arising secondary to physical trauma and remaining limited to the site of injury, such as surgical sites, poorly healing wounds, amputation sites, and even as an isomorphic response in patients with chronic pruritus. Treatment of dyshidrosiform pemphigoid, like that of bullous pemphigoid, focuses on immunosuppressive therapy. Topical corticosteroids are regarded as the first-line therapy, with the addition of systemic corticosteroids, dapsone, mycophenolate mofetil, or azathioprine combination therapy as indicated. No clinical, laboratory, or histopathologic findings have been shown to be predictive of either the duration or extent of involvement in patients with localized or generalized bullous pemphigoid. Although half of patients with generalized bullous pemphigoid in 1 hospital series experienced clinical remission after 3 years, no similar studies regarding dyshidrosiform pemphigoid exist. In our patient’s case, a high potency topical corticosteroid applied twice daily was sufficient to obtain a clinical remission after 4 months of therapy. For this series, the recommended choices are: 1, b; 2, a; 3, c; 4, b.