bluuebunny/crossref_metadata_2025_split huggingface dataset:
Pyoderma Vegetans
Authors: Papadopoulos Anthony J. , Schwartz Robert A. , Kapila Rajendra , Samady Joseph A. , Ruszczak Zbigniew , Rao Babar K. , Lambert W. Clark 
Published: July 2017

Background: Pyoderma vegetans is a rare condition that is clinically characterized by large verrucous plaques with elevated borders and multiple pustules. The etiology of this disorder remains unknown. Objectives: We describe a 24-year-old woman with rapidly evolving pyoderma vegetans. Our patient had the unique additional findings of a highly elevated serum IgE level and a history of hidradenitis suppurativa. Conclusions: Pyoderma vegetans is diagnosed on clinical and histological criteria. Differentiation must be made from disorders such as pyoderma gangrenosum, Sweet's syndrome, and deep fungal infections. We illustrate a case of pyoderma vegetans and review the literature on this rare disorder. Clinical and histological criteria for diagnosis are presented, as well as differentiation from some mimicking disorders.
Dermatological Manifestations of Down's Syndrome
Authors: Barankin Benjamin , Guenther Lyn 
Published: July 2017

Background: Down's syndrome (DS) is an extensively researched congenital condition characterized by mental retardation and distinct physical features. The cutaneous manifestations of DS are numerous, yet they seldom receive appropriate attention. Objective: To review the dermatological conditions associated with DS. Methods: A review of the medical literature. Results: DS is associated with an increased incidence of numerous dermatological conditions, some of which may be related to an immunological deficiency. Conclusion: Along with the importance of understanding the physical and psychosocial aspects of DS, an appreciation of the dermatological manifestations of this condition is integral to comprehensive medical care.
Betamethasone Valerate in Foam Vehicle is Effective with Both Daily and Twice a Day Dosing: A Single-Blind, Open-Label Study in the Treatment of Scalp Psoriasis
Authors: Feldman Steven R. , Ravis Scott M. , Fleischer Alan B. , McMichael Amy , Jones Evelyn , Kaplan Robert , Shavin Joel , Weiss Jonathan , Bartruff J. Kent , Levin Donald L. , Del Rosso James , Kpea Nomate 
Published: July 2017

Background: Psoriasis is a chronic relapsing skin disorder that affects about 2% of the U.S. population and involves the scalp approximately 50% of the time. Topical corticosteroids, including betamethasone valerate, have been used effectively in the treatment of corticosteroid-responsive dermatoses of the skin and scalp. Betamethasone valerate (BMV) in foam vehicle (Luxiq) is designed to improve patient compliance with topical therapy. Superior efficacy over a BMV lotion preparation has been demonstrated with twice-daily use. Even greater compliance would be expected if the drug is effective with once-daily application. Purpose: To compare the efficacy of the betamethasone valerate foam (Luxiq) in the treatment of scalp psoriasis following once-daily versus twice-daily dosing. Methods: Seventy-nine patients with moderate to severe scalp psoriasis from seven centers were enrolled and treated with BMV foam either once a day or twice a day for four weeks. The physician-grader was blinded to the treatment regimen, and the subjects were randomly assigned to either once-daily or twice-daily dosing in a 1:1 ratio. Results: The signs of psoriasis (plaque thickness, scaling, and erythema) were assessed before and after treatment. The investigator's and the patients' global assessments were also evaluated. The composite score improved from 7.7±2.1 to 3.0±2.2 with twice-a-day use and from 8.1 ± 2.2 to 3.9 ± 2.8 with once-daily use ( p > 0.05 for the difference between groups). Discussion: BMV foam is effective for scalp psoriasis with both once-a-day and twice-a-day use. This feature of the BMV foam is encouraging for expected improvement in clinical use.
Pseudoporphyria: An Atypical Variant Resembling Toxic Epidermal Necrolysis
Authors: Papadopoulos Anthony J. , Schwartz Robert A. , Fekete Zoltan , Kihiczak George , Samady Joseph A. , Atkin Suzanne H. , Lambert W. Clark 
Published: July 2017

Background: Pseudoporphyria has been attributed to both medication usage and chronic hemodialysis. Histologically, it is identical to porphyria cutanea tarda. It is most commonly seen as localized bullae on sun-exposed skin, often on the dorsum of the hands and fingers. Objectives: We describe a 31-year-old man with rapidly evolving bullae which became denuded, clinically suggestive of toxic epidermal necrolysis. Pseudoporphyria was proven histologically. However, our patient's eruption was not localized as small bullae but was widespread, with large bullae evolving into large, cutaneous, denuded erosions. Conclusions: We describe a previously unreported, generalized variant of pseudoporphyria that resembles toxic epidermal necrolysis. We provide a review of pseudoporphyria and compare our variant to toxic epidermal necrolysis and mimicking disorders.
Self-Healing Congenital Langerhans Cell Histiocytosis Presenting as Neonatal Papulovesicular Eruption
Authors: Morgan Kelli W. , Callen Jeffrey P. 
Published: July 2017

Background: Congenital self-healing Langerhans cell histiocytosis (CSHLCH) is a rare condition which may present at birth or during the neonatal period. It is usually characterized by the eruption of multiple, disseminated, red-brown papules and nodules which may increase in size and number during the first few weeks of life. Systemic signs are usually absent except for occasional mild hepatomegaly. Objective: We present a 3.5-kg male infant who presented at birth with numerous diffuse, erythematous, crusted erosions. He was presumed to have congenital herpes simplex virus (HSV) and was started on IV acyclovir. Histopathologic examination revealed a mixed inflammatory infiltrate with numerous histiocytes which were S-100 and peanut agglutin positive consistent with CSHLCH. Further workup did not reveal any signs of systemic involvement. Conclusion: CSHLCH has rarely been reported to present as a papulovesicular eruption at birth. In these cases, a viral etiology is commonly entertained in the differential diagnosis. Despite the spontaneous regression of skin lesions in CSHLCH, close followup is required to evaluate for systemic signs and symptoms associated with latent Letterer–Siwe disease.
Peripheral T Cell Lymphoma Presenting as Dermatomyositis-like Eruption
Authors: Rencic Adrienne , Laman Susan , Nousari Hossein C. 
Published: July 2017

Background: There are several conditions reported to mimic the cutaneous manifestations of dermatomyositis, including lymphoproliferative disorders. Objective: This case report presents an unusual case of peripheral T cell lymphoma mimicking dermatomyositis and discusses the clinical and pathologic features that distinguish it from dermatomyositis. Methods and Results: A 62-year-old woman presented with a two-month history of a progressive painful cutaneous eruption and interstitial infiltrates on chest x-ray. Skin biopsy revealed peripheral T cell lymphoma. The diagnosis was confirmed by lung biopsy. Conclusions: Although rare, a lymphoproliferative disorder must be included in the differential diagnosis of a cutaneous dermatomyositis-like eruption.
Progression of Cutaneous B-Cell Pseudolymphoma to Cutaneous B-Cell Lymphoma
Authors: Kulow Brittain F. , Cualing Hernani , Steele Paul , VanHorn Judi , Breneman John C. , Mutasim Diya F. , Breneman Debra L. 
Published: July 2017

Background: Debates regarding nosology and clonality surround the entity known as cutaneous pseudolymphoma and its questionable transformation to frank cutaneous lymphoma. The relevance of these arguments is important, not only from a diagnostic standpoint, but also for making inferences based upon behavior, prognosis, and treatment. Objective: Our goal was to demonstrate further evidence of progression from cutaneous pseudolymphoma to malignant lymphoma while at the same time advocating a comprehensive plan for evaluation, treatment, and followup of these patients. Methods: A retrospective review was conducted of four patients initially considered to have cutaneous B-cell pseudolymphoma (CBPL) and who were later treated for primary cutaneous B-cell lymphoma (CBCL). A review of the literature of cases suggesting progression to malignant lymphoma from precursor lesions was also performed. Results: Four patients initially diagnosed with CBPL by a combination of histologic, immunophenotypic, and gene rearrangement criteria had a progressive clinical course that, over a range of 17–51 months, evolved into CBCL. All patients had a comprehensive systemic workup to rule out the possibility of extracutaneous disease and were treated with local radiation therapy and close followup. There has been no evidence of extracutaneous disease with an average followup of 14 months. Conclusion: The potential for certain cutaneous pseudolymphomas to progress to CBCL is real. The combination of histologic and immunophenotypic criteria, along with the clinical picture, remains the best way to judge the aggressiveness of the lesion. Gene rearrangement studies, whether performed by Southern blot or polymerase chain reaction (PCR), are of limited value and should be used to support the overall clinicopathologic picture. Radiation therapy of these patients should be thought of early in the management plan and is a very successful form of treatment when combined with close followup.
6-Thioguanine in the Treatment of Psoriasis: A Case Report and Literature Review
Authors: Sherer Daniel W. , Lebwohl Mark G. 
Published: July 2017

Background: 6-Thioguanine is a purine analog primarily indicated for acute myelogenous leukemia. Its use in psoriasis was first investigated in the 1950s and current interest in serious treatments for psoriasis, such as cyclosporine, has renewed interest in this medication. Newer therapies, such as PUVA and retinoids, have side effects as do some older treatments, such as methotrexate, which fell into favor at the same time 6-thioguanine was being investigated. Reexamining older treatments can open up new therapeutic options in difficult cases. Objective: The goal of this article is to provide a comprehensive review of the literature regarding the use of 6-thioguanine in the treatment of psoriasis as well as an illustrative case report demonstrating the use and side effects of 6-thioguanine in a patient with pustular psoriasis. Topics reviewed include efficacy, side effects, laboratory monitoring, different dosing regimens, and proposed mechanisms of action. Conclusions: 6-Thioguanine has been highly effective in treating and maintaining improvement in patients with psoriasis. A study comparing efficacies with methotrexate would be extremely useful. Because of the possibility of severe bone marrow depression, 6-thioguanine is not a first-line treatment. However, with new dosing regimens, proper laboratory monitoring, and further studies, use of 6-Thioguanine could expand in the future.
Use of Transcutaneous Nerve Stimulation Wristband to Treat Methotrexate-Induced Nausea
Authors: Wilson Jill K. , Phelps Kathy C. , Feldman Steven R. 
Published: July 2017

Background: Although methotrexate is a highly effective treatment for psoriasis, nausea and vomiting may be dose-limiting side effects. Various approaches have been used to improve the tolerability of methotrexate therapy, including changing the dose or form of administration of the methotrexate or concomitant administration of folic acid or an antiemetic. Objective: The objective of this case report is to present the use of a novel, nonpharmacologic approach for controlling the nausea associated with methotrexate therapy. Results: A 47-year-old patient with long-standing psoriasis was treated with methotrexate. She developed nausea refractory to standard antiemetic measures. She noted almost immediate relief of her nausea when using a transcutaneous nerve stimulation wristband. Conclusions: Nerve stimulation may be a valid alternative to other first-line antiemetic therapies.
Modulation of the Contact Hypersensitivity Response by Æ-941 (Neovastat), a Novel Antiangiogenic Agent
Authors: Dupont É. , Wang B. , Mamelak A. J. , Howell B. G. , Shivji G. , Zhuang L. , Dimitriadou V. , Falardeau P. , Sauder D. N. 
Published: July 2017

Background: Æ-941 (Neovastat) is an angiogenesis inhibitor noted to have antiinflammatory properties. Objective: We tested Neovastat in a contact hypersensitivity (CHS) model to determine the mechanism of action of its antiinflammatory effects. Methods: Neovastat was orally administered (200 mg/kg/day) during the sensitization and challenge phases of a murine CHS assay and inflammatory responses were measured. Subsequent assays were performed on mice treated with Neovastat or Cortisone (120 mg/kg/day, IP) and differential mRNA expression of several pro- and antiinflammatory cytokines was quantified using RT-PCR. Results: Neovastat decreased inflammation by 39% when administered during sensitization but did not alter the CHS response when given during the challenge phase. Neovastat significantly induced IL-10 expression in skin and skin-draining lymph nodes (49% and 45%, respectively) and decreased IFNγ expression in the lymph nodes (35%). Conclusion: Antiinflammatory effects of Neovastat observed in CHS could be linked to modulation of cytokines early in the sensitization phase.
Role of Extracellular Adenosine Triphosphate in Human Skin
Authors: Holzer Aton M. , Granstein Richard D. 
Published: July 2017

Background: The nucleotide adenosine triphosphate (ATP) has long been known to drive and participate in countless intracellular processes. Extracellular ATP and its metabolite adenosine have also been shown to exert a variety of effects on nearly every cell type in human skin. Knowledge of the sources and effects of extracellular ATP in human skin may help shape new therapies for skin injury, inflammation, and numerous other cutaneous disorders. Objective: The objective of this review is to introduce the reader to current knowledge regarding the sources and effects of extracellular ATP in human skin and to outline areas in which further research is necessary to clarify the nature and mechanism of these effects. Conclusion: Extracellular ATP seems to play a direct role in triggering skin inflammatory, regenerative, and fibrotic responses to mechanical injury, an indirect role in melanocyte proliferation and apoptosis, and a complex role in Langerhans cell-directed adaptive immunity.
Melasma: A Review
Authors: Victor Frank C. , Gelber Jeremy , Rao Babar 
Published: July 2017

Objective: To better understand melasma, a review of its etiologic factors, classification, pathogenesis, and treatment was undertaken. Methods: Articles discussing the above aspects of melasma were used to demonstrate what is currently known about the disease and how to treat it. Results: Melasma is associated with many etiologic factors, most importantly, sun exposure. It occurs in three distributions and has four reported patterns of pigmentation. Among the many differences between melasma and normal skin, melasma skin contains increased melanin, melanocytes, and melanosomes, as well as increased synthesis of tyrosinase. Its pathogenesis remains largely unknown. Treatment consists of phenolic and nonphenolic depigmenting agents, chemical peels, lasers, and dermabrasion. Conclusion: Melasma is a common skin disorder. Although melasma has been studied, its pathogenesis remains largely unknown and its treatment is still met with difficulty. Randomized controlled trials involving larger numbers of patients and comparing treatments, as well as studying combination therapies, would be beneficial.
Multiple Keratoacanthomas Arising Post-UVB Therapy
Authors: Craddock Kenneth J. , Rao Jaggi , Lauzon Gilles J. , Tron Victor A. 
Published: July 2017

Background: Ultraviolet (UV) radiation is known to be an important etiologic agent in the development of skin cancer. Keratoacanthoma is an unusual, well-described cutaneous neoplasm that resembes squamous cell carcinoma but spontaneously resolves. Rarely, multiple keratoacanthomas may develop. Objective We present a case of multiple keratoacanthomas in a patient with psoriasis who had received UVB phototherapy. These lesions were hyperkeratotic papules, many of which spontaneously resolved and demonstrated the histologic characteristics of keratoacanthoma. Conclusion: We believe that UV radiation is the most likely etiologic factor in this patient's development of multiple keratoacanthomas. We wish to bring to the attention of clinicians this unusual adverse effect of UV treatment.
Infantile Myofibromatosis
Authors: Levy Elie , Raymond Giles P. 
Published: July 2017

Background: Infantile myofibromatosis is a rare condition that usually presents in infancy. Objective: The purpose of this article is to report a new case of late-onset infantile myofibromatosis and to review the literature regarding similar cases. Methods: A Medline search was performed, and a new case is reported. Results: The solitary tumour presented by the 9-year-old boy is histologically proven to be compatible with the diagnosis of infantile myofibromatosis. Conclusion: Although infantile myofibromatosis usually presents in infancy, onset may occur in childhood.
Pharmacoeconomics for Dermatologists: An Introduction
Authors: Gupta Aditya K. , Oh Paul I. , Shear Neil H. 
Published: July 2017

Background: The budgets available for health care are becoming constrained and health care decision makers have increasingly begun to scrutinize cost along with efficacy, tolerability, and cost of the different treatment options for each disease state. In keeping with the above, there has been a marked increase in the number of pharmacoeconomic evaluations published in the medical literature, including dermatology journals. Methods: Comprehensive economic evaluations systematically consider the following: statement of question, defining relevant costs, perspective and time-horizon, synthesis of data on efficacy and effectiveness, and selection of the appropriate analytic type and framework. The conclusions should be tested through extensive sensitivity analyses. Conclusions: Economic evaluations are becoming more prevalent in the field of dermatology. A well-constructed analysis may be an aid to more rational therapeutic decision-making.
Melanocytic Nevi in Malignant Melanomas
Authors: Wasti Qasim H. , Toussaint Sonia , Kopf Alfred W. , Kamino Hideko , Provost Nathalie , Bart Robert S. 
Published: July 2017

Background: A substantial proportion of malignant melanomas arise in preexisting melanocytic nevi. However, the clinical and dermoscopic features of such combination lesions have not been well defined in the literature. Objective: To determine the ability to recognize in thin malignant melanomas the presence or absence of melanocytic nevi based on clinical and/or dermoscopic observations. Methods: Thirty malignant melanomas, less than 1 mm in Breslow thickness, were studied clinically, dermoscopically, and histologically for the presence or absence of features of a melanocytic nevus. Results: The ability to recognize melanocytic nevi within thin malignant melanomas was poor by clinical and dermoscopic examinations, with 30% false negatives and 23% false positives. Conclusion: One cannot depend on clinical and/or dermoscopic observations to rule out a melanocytic nevus within a thin malignant melanoma. Histopathologic study of a small portion of a combined melanocytic neoplasm could lead to the mistaken conclusion that the lesion is entirely a melanocytic nevus or entirely a malignant melanoma. Therefore, when feasible, lesions suspected of being malignant melanomas should be totally excised and step-sectioned throughout.
Assessing Scientific Data: The Case-Control Study as it Applies to Dermatology Part 2: Interpreting the Results
Authors: Heacock Helen J. , Rivers Jason K. 
Published: July 2017

Background: In order to interpret the results of case-control studies correctly and to assign causality to an exposure-disease association, one must be able to assess elements in the design and/or analysis that may lead to inaccurate conclusions. Objective: The purpose of this article is to describe factors that may invalidate the results of a case-control study. Topics discussed include bias, confounding, and chance. Criteria used to judge causality between exposure and disease are highlighted. A brief checklist that can be used as a tool in the design and interpretation of case-control studies is provided. Conclusion: Before one concludes that an exposure causes a disease, alternative explanations for the observations must be considered. Assuming that these threats to the validity of the study have been minimized, criteria for judging causality can be examined. In this way, it becomes possible to link a specific exposure with a specific outcome.
Monitoring Isotretinoin Therapy in Acne: Rational Use of Laboratory Tests
Authors: Willoughby Joanne S. , Rosenthal Donald 
Published: July 2017

Background: Several recent long-term follow-up studies confirm the relative safety of isotretinoin in acne. Yet, conscientious dermatologists may order more laboratory tests than is strictly necessary. Objective: Our goals were to determine which laboratory tests used in monitoring isotretinoin therapy predict significant abnormalities, when such abnormalities are likely to occur, and to establish guidelines for practitioners in ordering these tests. Methods: In the Hamilton, Ontario region, 27 dermatologists were surveyed to determine the tests and their frequency requested by practicing dermatologists. We also critically reviewed the literature to establish the frequency and magnitude of laboratory tests in acne patients on isotretinoin and assessed the rationale and cost-effectiveness of the suggested tests. Results: Seventeen dermatologists (63%) responded. A wide range of tests were ordered at variable intervals. The literature review suggests that adverse laboratory events are uncommon and that many are clinically insignificant. Conclusion: Dermatologists may be requesting more laboratory tests for monitoring isotretinoin therapy than are warranted or cost-effective. We propose a rational laboratory program for monitoring patients on isotretinoin.
Calciphylaxis: A Cause of Necrotic Ulcers in Renal Failure
Authors: Kalaaji Amer N. , Douglass Margaret C. , Chaffins Marsha , Lowe Lori 
Published: July 2017

Background: Calciphylaxis is a rare and life-threatening condition occurring in patients with end-stage renal disease on dialysis. The diagnosis is frequently delayed or missed and therapy is often unsuccessful. Objective: The clinical and pathological components of calciphylaxis are emphasized in a patient that may facilitate early diagnosis and initiation of therapy. Results: Early recognition of the clinical signs and symptoms of calciphylaxis is crucial. The tissue diagnosis requires large elliptical biopsies because histological findings are segmental and often missed. Conclusion: Early diagnosis and intervention in calciphylaxis is critical. Parathyroidectomy should be considered in all cases.