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Open Access Publications from the University of California

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Dermatology Online Journal is an open-access, refereed publication intended to meet reference and education needs of the international dermatology community since 1995. Dermatology Online Journal is supported by the Department of Dermatology UC Davis, and by the Northern California Veterans Administration.

Volume 21, Issue 10, 2015

Review

Review of digital image security in Dermatology

The inherently visual nature of dermatology naturally lends itself to photography. As technology has evolved, smartphone cameras have become ubiquitous and have the potential to improve education and patient care in dermatology. Although patients and physicians may agree that photography can improve patient care, there are certain risks involved with smartphone photography in the medical field. Perhaps most concerning is the number of dermatologists using smartphones to take unsecured images in their daily practice. A recent study revealed that 22% of surveyed dermatologists used smartphone cameras multiple times per day in their practice. Dermatologists may also overestimate patient comfort with smartphone use in clinical photography. We present a review of the use of smartphones in dermatology and address the potential lack of security and accompanying ethical dilemmas.Keywords: Smartphone, iPhone, digital

Original

Increased severity of itching, pain, and scaling in psoriasis patients is associated with increased disease severity, reduced quality of life, and reduced work productivity

Background: Psoriasis patients report that this disease can impact on their health-related quality of life (HR-QoL) and work productivity. It is important to understand how this is influenced by the clinical characteristics of psoriasis such as symptom severity. Common symptoms include itching, pain, and scaling but the psychosocial impact these features have on patients is not well understood.

Objective: To explore the impact of psoriasis symptoms (itching, pain, and scaling) on HR-QoL and work productivity.

Method: Data were extracted from the Adelphi 2011 and 2013 Psoriasis Disease Specific Programmes – two real world surveys of US dermatologists and their psoriasis patients.  HR-QoL was measured using the Dermatology Life Quality Index (DLQI) and EuroQOL 5-Dimension Health Questionnaire (EQ-5D). Work productivity loss was measured by the Work Productivity and Activity Impairment (WPAI) questionnaire. The impact of symptom severity (none, mild, moderate/severe) for itching, pain, and scaling on DLQI, EQ-5D, and WPAI scores were examined, controlling for differences in demographics and co-morbidities.

Results: Patient mean age was 44 years and 55% were male. Moderate/severe itching, pain, and scaling were experienced by 33%, 13%, and 41% of patients, respectively. Controlling for differences in demographics and co-morbidities, increased symptom severity was associated with reduced HR-QoL. Accordingly, EQ-5D scores decreased with itching severity (moderate/severe vs. none: -0.07; 95% confidence interval [CI] =-0.09, -0.04), whereas DLQI scores increased (moderate/severe versus none: 4.9; CI = 3.9, 5.9) (both p<0.05). WPAI scores increased with itching severity, indicating increased work productivity loss (moderate/severe versus none: 17.6, CI = 11.8, 23.5, p<0.05). The same pattern was observed for pain and scaling.

Conclusions: Among the patients studied, increased severity of psoriasis-related itching, pain, and scaling was associated with reduced health-related QoL and work productivity.

A national survey of residents in combined Internal Medicine and Dermatology residency programs: educational experience and future plans

Importance: In response to a perceived erosion of medical dermatology, combined internal medicine and dermatology programs (med/derm) programs have been developed that aim to train dermatologists who take care of medically complex patients. Despite the investment in these programs, there is currently no data with regards to the potential impact of these trainees on the dermatology workforce.

Objective: To determine the experiences, motivations, and future plans of residents in combined med/derm residency programs.

Design, Setting, and Participants: We surveyed residents at all United States institutions with both categorical and combined training programs in spring of 2012. Respondents used visual analog scales to rate clinical interests, self-assessed competency, career plans, and challenges.

Main Outcomes and Measures: The primary study outcomes were comfort in taking care of patients with complex disease, future practice plans, and experience during residency.

Results: Twenty-eight of 31 med/derm residents (87.5%) and 28 of 91 (31%) categorical residents responded (overall response rate 46%). No significant differences were seen in self-assessed dermatology competency, or comfort in performing inpatient consultations, cosmetic procedures, or prescribing systemic agents. A trend toward less comfort in general dermatology was seen among med/derm residents. Med/derm residents were more likely to indicate career preferences for performing inpatient consultation and taking care of medically complex patients. Categorical residents rated their programs and experiences more highly.

Conclusions and Relevance: Med/derm residents have stronger interests in serving medically complex patients. Categorical residents are more likely to have a positive experience during residency. Future work will be needed to ascertain career choices among graduates once data are available.

Case Report

IgM ocular cicatricial pemphigoid: a unique insight into the immune system

A 42-year-old man with ocular erythema and scarring had a conjunctival biopsy revealing depostion of IgM and C3 without IgG at the epithelial basement membrane zone.  Treatment with doxycyline, dapsone, and mycophenolate mofetil was unsuccessful and treatment with rituximab has led to partial remission of the conjunctival inflammation. He has undergone 4 cycles of rituximab treatment at intervals of 12 to 18 months.

Case Presentation

Scalp necrosis overlying a ventriculoperitoneal shunt: a case report and literature review

Background: The use of ventriculoperitoneal (VP) shunts has become ubiquitous in neurosurgery for the treatment of hydrocephalus. VP shunts work by creating a conduit for cerebrospinal fluid (CSF) to flow from the cerebral ventricles to the peritoneum and thus relieving pressure. Although typically safe, VP shunt complications are extremely common, occurring in up to 29% of adult cases and approximately half of pediatric cases. These complications may require patients to undergo several revisions throughout their lifetime

Purpose: We describe a man who developed scalp necrosis overlying his VP shunt. We also summarize the potential complications of VP shunts. We discuss the presentation and pathogenesis of scalp necrosis in these patients.

Materials and methods: A PubMed search of the following terms was performed and relevant citations were assessed: ventriculoperitoneal shunt, VP shunt, shunt complications, scalp necrosis, skin necrosis, ventriculoperitoneal shunt induced scalp necrosis, ventriculoperitoneal shunt induced skin necrosis.

Results: A 73-year-old man developed VP shunt-induced scalp necrosis leading to loss of skin overlying the shunt valve. The patient was emergently referred to neurosurgery and admitted for revision of the shunt due to the significant risk for infection. The VP shunt was replaced with an external drain and a skin flap was used to repair the defect.

Conclusion: VP shunts are devices that are frequently used for reducing intracranial pressure associated with hydrocephalus. The insertion of the shunt beneath the scalp sets the stage for potential pressure-induced scalp necrosis. The early recognition and diagnosis of VP shunt-induced scalp necrosis is essential to prevent infections and future complications.

A case of Kaposi sarcoma in an immunocompetent, heterosexual Irish man: a discussion of etiology and viral transmission

Four types of Kaposi sarcoma (KS) have been described, all of which are caused by human herpesvirus-8 (HHV-8).  The incidence of KS in the United States is highest among HIV-positive homosexual men and elderly men of Eastern European, Jewish, or Mediterranean descent. However, few reports describe KS in HIV-negative, immunocompetent heterosexual men in the United States. HHV-8 is transmitted largely via saliva and close sexual contact, whereas there are only a handful of reports of transmission via blood and blood products. We report a case of an HIV-negative, immunocompetent heterosexual man who acquired KS via blood transfusion. A 77-year-old immunocompetent, monogamously heterosexual, HIV-negative Irish man presented with a biopsy-proven KS lesion on the right thigh. Past surgical history included a coronary artery bypass graft, during which he received a blood transfusion from an unknown donor source.  His ecchymotic KS lesions progressed while on doxycycline, intralesional vinblastine, and topical anti-angiogenic medications.  The patient eventually achieved stabilization of KS lesions with acitretin. Our case report emphasizes the need to characterize the phenotype and transmission route of HHV-8 in heterosexual, immunocompetent patients in geographic regions with low HHV-8 seroprevalence.

Infantile myofibroma: a firm, round plaque in an infant

Infantile myofibroma is a rare fibromatous tumor that is variable in presentation and is frequently mistaken for hemangioma or rhabdomyosarcoma. We describe a 14-month-old male who presented with multiple, enlarging, firm lesions on the shoulder. Biopsy revealed a proliferation of small spindle cells with myxoid and hyalinized stroma infiltrating into the superficial adipose tissue. We provide a brief review of the clinical presentation, histopathologic features, management and recent advances in our understanding of this rare condition.

C-MYC positive angiosarcoma of skin and breast following MammoSite® treatment

Angiosarcoma of the skin and breast is a known complication of chronic lymphedema following mastectomy or external radiation therapy for breast cancer. We report a 68-year-old woman who presented with a 2.5 cm violaceous plaque on the skin of the right breast and a 3 cm mixed mass of the same breast by ultrasound 9 years after MammoSite® balloon brachytherapy.  Biopsy of the skin lesion and the breast mass showed an infiltrating high grade angiosarcoma.  The tumor cells in the skin and breast showed immunohistochemical reactivity for C-MYC.  A total mastectomy confirmed the presence of high grade angiosarcoma in the skin and parenchyma of the breast and radiation changes in the breast parenchyma.  Surgical margins were considered negative.  The patient had cutaneous recurrence of angiosarcoma three months after the mastectomy.  There have been only two other case reports in the literature of angiosarcoma on the skin following MammoSite® therapy. The c-myc mutation has been shown to be a specific mutation for angiosarcoma following radiation treatment. It is not found in atypical vascular lesions following irradiation or angiosarcoma unrelated to radiation treatment.

Graphite oral tattoo: case report

Pigmented oral lesions compose a large number of pathological entities, including exogenous pigmentat oral tattoos, such as amalgam and graphite tattoos. We report a rare case of a graphite tattoo on the palate of a 62-year-old patient with a history of pencil injury, compare it with amalgam tattoos, and determine the prevalence of oral tattoos in our Oral Pathology Service. We also compare the clinical and histological findings of grafite and amalgam tattoos. Oral tattoos affect women more frequently in the region of the alveolar ridge. Graphite tattoos occur in younger patients when compared with the amalgam type. Histologically, amalgam lesions represent impregnation of the reticular fibers of vessels and nerves with silver, whereas in cases of graphite tattoos, this impregnation is not observed, but it is common to observe a granulomatous inflammatory response, less evident in cases of amalgam tattoos. Both types of lesions require no treatment, but in some cases a biopsy may be done to rule out melanocytic lesions.

Oral manifestations of connective tissue disease and novel therapeutic approaches

Connective tissue diseases such as systemic lupus erythematosus (SLE), systemic sclerosis (SSc), and Sjögren syndrome (SS) have presented many difficulties both in their diagnosis and treatment. Known causes for this difficulty include uncertainty of disease etiology, the multitude of clinical presentations, the unpredictable disease course, and the variable cell types, soluble mediators, and tissue factors that are believed to play a role in the pathogenesis of connective tissue diseases. The characteristic oral findings seen with these specific connective tissue diseases may assist with more swift diagnostic capability. Additionally, the recent use of biologics may redefine the success rate in the treatment and management of the disease. In this review we describe the oral manifestations associated with SLE, SSc, and SS and review the novel biologic drugs used to treat these conditions.

Lichen planus pigmentosus in linear and zosteriform pattern along the lines of Blaschko

Lichen planus pigmentosus (LPP) is a variant of lichen planus (LP) reported in various ethnic groups. It occurs predominantly in the third or fourth decade of life and is characterized by the insidious onset of dark-brown macules in sun exposed areas and flexural folds. Rarely, has it been described in a linear or segmental distribution. Herein we describe a case of LPP with lesions lateralized to right side of body along the lines of Blaschko, in a linear and zosteriform pattern.

Novel mutation in the fumarate hydratase gene in a patient with Reed syndrome

Reed syndrome is an autosomal dominant disorder characterized by cutaneous leiomyomas, uterine leiomyomas, and renal cell carcinoma caused by mutations in the fumarate hydratase gene. Dermatologic evaluation is often the first or only opportunity to discover the diagnosis of Reed syndrome in affected patients, which may prove to be life-saving. We present a 40-year-old woman with history of large uterine leiomyomas who presented with a two-year history of a pruritic papular eruption on the left neck refractory to topical corticosteroids. After histopathologic examination and genetic work-up, the patient was found to have a novel mutation in the fumarate hydratase gene and was subsequently diagnosed with Reed syndrome.

Equestrian perniosis: A case report and review of the literature

Equestrian perniosis is a rare form of perniosis that is predominantly seen in young female equestrians who ride in cold temperatures. Tight fitting clothing, age under 35 years, and smoking can contribute to the disease manifestation. The diagnosis can be determined from a thorough history and physical examination. However, a biopsy can be taken to confirm the diagnosis. Although many cases are self-limiting and resolve within a few days to weeks of diagnosis, nifedipinie can be considered as a therapeutic measure for persistent cases. Measures such as wearing loose fitting clothing and horseback riding in warmer temperatures and for shorter time periods can prevent recurrence.

Photo Vignette

Queratodermia plantar dolorosa y onicodistrofia en mujer joven caucásica

Pachonychia congenita is an uncommon genodermatosis, classified among the disorders of keratinization and caused by mutation of certain keratin genes.  The characteristic clinical triad is onychodystrophy, keratoderma, and plantar pain.  In addition, the patients may present with epidermal cysts, steatocystomas, leukokeratosis, and deciduous teeth.

Juvenile dermatomyositis with joint contractures and calcinosis cutis

A 12-year-old boy presented to our department with firm papules on the fingers of both hands, erythematous scaly plaques on the dorsum of the hands and elbow, and deformities and limitation of motion in the joints of the hands and feet. His parents reported that the eruption started 6 years prior to presentation. He was previously diagnosed with psoriasis by physicians and acitretin treatment was given. However, he did not benefit from the treatment.

LEOPARD Syndrome

LEOPARD syndrome (LS) is an autosomal dominantly inherited or sporadic disorder of variable penetrance and expressivity. The acronym LEOPARD stands for its cardinal clinical features including Lentigines, Electrocardiographic conduction abnormalities, Ocular hypertelorism, Pulmonary stenosis, Abnormalities of genitalia, Retardation of growth, and Deafness. We present herein a patient with LEOPARD syndrome and distinctive features. It was noteworthy that our patient presented with the concern of generalized lentiginosis and subsequent evaluation revealed that the patient had LEOPARD syndrome. In this report we would like to highlight the importance of detailed clinical examination and appropriate imaging in patients with multiple lentigines.

Commentary

Increasing medical student exposure to clinical dermatology through participation in volunteer clinics

Over the previous decade, several innovative teaching methods have been introduced to overcome the decreasing allotment of time dedicated to dermatology in U.S. medical school curricula. We report our experience of increasing medical student exposure to clinical dermatology thorough involvement in an extracurricular, volunteer-driven dermatology clinic. The clinic was well received by students and faculty. Our experience demonstrates that volunteer-driven dermatology clinics may be an effective method of teaching and engendering a culture of community outreach among medical students and faculty.

What are the most important factors that determine the overall quality of dermatology residency training: A resident survey

Currently no broadly accepted and standardized objective ranking system for dermatology residency programs exist. A new ranking system was recently developed in an attempt to address this issue. However, it remains unclear if the algorithm employed is based on factors that have a tangible impact on the quality of training provided by dermatology residency programs [1]. Our authors surveyed all current dermatology residents in order to investigate the factors that they feel are most important in determining the strength of training provided by a residency program.

Letter

Subungual hematoma: clinical appearance of resolution over time

Subungual hemorrhage is usually a benign sign, often linked to a traumatic cause. However, it can also reveal a serious and chronic systemic disease. As we will show, subungual hemorrhage may be an indicator of the severity of diabetic microangiopathy. We report a case of subungual hemorrhage in diabetes.

Terra firma-forme dermatosis: Case Series and dermoscopic features

Terra firma-forme dermatosis (TFFD) is characterized by dirt-like skin lesions that disappear after rubbing with alcohol. We describe the dermoscopic features of TFFD before and after alcohol swabbing in six patients. All patients showed similar dermoscopic appearance with large polygonal plate-like brown scales arranged together giving a mosaic pattern. These features disappear completely after isopropyl alcohol swabbing of the lesions. In conclusion dermoscopy can assist in the evaluation of terra firma-forme dermatosis and the dermoscopic evaluation of other dirty dermatoses is recommended in the future to compare findings with TFFD.