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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 24, Issue 2, 2018

Review

Representation and treatment allocation of racial groups in dermatologic therapy trials: A 2-year review of the literature

Although most investigators would agree that including minority races in clinical trials is important, recruitment and retention may differ among these populations. The objective of this review was to perform an audit of phase III dermatologic therapy trials to determine representation for minority groups and to explore the possibility of racial allocation bias. In this review of 11 dermatology or general medicine journals in 2015-16, we did not find evidence of systemic racial allocation bias. We did however note variation in the proportion of minority races included in studies; whereas some trials had high success in recruiting minorities, many did not. Furthermore, most studies did not provide information on individual racial groups and rather presented an ‘other’ category. This supports findings from previous reviews of dermatologic therapy trials that suggest that most participants are white, race data are not included for many studies, and there is underrepresentation of some racial groups. We conclude that although there is no evidence of racial allocation bias in the previous two years, there remains a need for standardization in the reporting of racial groups and for increased participant diversity in dermatologic therapy trials.

Commentary

An updated assessment of social media usage by dermatology journals and organizations

Despite the increasing prevalence of social media usage, the activity of dermatology journals and professional and patient-centered organizations on top social media platforms has not been investigated since 2012. We investigated a total of 124 dermatology journals, 242 professional organizations, and 78 patient-centered organizations to assess their presence and popularity on social media. Searches were conducted to identify journals and organizations on Facebook and Twitter. Similar searches were done for organizations on LinkedIn. The number of Facebook likes, Twitter followers, and LinkedIn followers of the dermatological entities were quantified. There were 22 (17.7%) dermatology journals active on Facebook and 21 (16.9%) on Twitter. Amongst the professional organizations, 114 (47.1%) were on Facebook, 69 (28.5%) on Twitter, and 50 (20.7%) on LinkedIn. In comparison, 68 (87.2%) patient-centered organizations were on Facebook, 56 (71.8%) on Twitter, and 56 (71.8%) on LinkedIn. Our results demonstrate that the popularity of dermatology journals and professional and patient-centered organizations on top social networking sites has grown markedly since 2012. Although the number of dermatology journals on social media has increased since 2012, their presence continues to trail behind professional and patient-centered dermatological organizations, suggesting underutilization of a valuable resource.

Case Report

A possible role for dupilumab (Dupixent) in the management of idiopathic chronic eczematous eruption of aging

Aging individuals can develop generalized, exquisitely-pruritic, eczematous eruptions of uncertain etiology that can be therapeutically-refractory and life-altering. Limited information exists in the literature to guide clinicians in the diagnosis and management of such patients. It is suggested that in approximately 40% of such patients a known cause for their chronic pruritic eruptions cannot be identified. In this report we will refer to this subgroup of patients as having idiopathic chronic eczematous eruption of aging (CEEA). Idiopathic CEEA must be distinguished from other established eczematous dermatoses. Idiopathic CEEA patients often require long-term systemic immunosuppressive drugs to make living bearable. Elder-onset atopic dermatitis is the most difficult of the known dermatoses to distinguish from idiopathic CEEA. Because of their clinical similarities we questioned whether dupilumab (Dupixent®), the first FDA-approved biologic for atopic dermatitis, might be valuable in the management of idiopathic CEEA. We report the case of an elderly man with idiopathic CEEA of four-years’ duration who had a complete clinical response to the initiation of treatment with dupilumab. This case is presented to stimulate more discussion and systematic study of a possible role for dupilumab in otherwise-refractory idiopathic CEEA patients. We also propose a set of diagnostic criteria for idiopathic CEEA.

Paraneoplastic pemphigus without antibodies to desmoglein 1 and

Paraneoplastic pemphigus is a severe autoimmune blistering disease presenting in the setting of underlying malignancy. Paraneoplastic pemphigus is associated with diffuse painful stomatitis throughout the oral cavity with extension to the lips. The cutaneous findings are varied and have been described as lichenoid, pemphigoid, and targetoid lesions. Herein, we report a patient with paraneoplastic pemphigus whose routine testing led to a diagnosis of pemphigus vulgaris. However, further testing was pursued revealing an antibody profile consistent with paraneoplastic pemphigus. Subsequent neoplastic workup revealed an intra-abdominal mass. Our case represents a subtle, non-classic presentation of paraneoplastic pemphigus and suggests the importance of a comprehensive investigative work-up in atypical cases of pemphigus

Lamellar ichthyosis in a female neonate without a collodion membrane

The term, autosomal recessive congenital ichthyosis (ARCI), describes a group of rare genetic skin diseases of cornification involving hyperkeratotic scaling at birth. The defective skin barrier function may lead to dehydration, body temperature instability, and high susceptibility to infections. In most cases of ARCI, neonates are born with a collodion membrane covering the body, often presenting with ectropion and eclabium. We report a premature female neonate presenting with hyperkeratotic scaling at birth without a collodion membrane. She was managed with placement in a humidified isolette, prophylactic antibiotics, dilute bleach baths, petrolatum ointment, and artificial eye drops. By the fourth week of life, there was marked improvement in her skin with the large, brown, plate-like scales on the trunk and extremities becoming lighter in color and finer in appearance. The ichthyosis genetic panel showed mutations in the ABCA12 gene resulting in the lamellar ichthyosis phenotype of ARCI. Our literature review revealed at least 28 patients with ARCI who were not born as collodion babies. Although collodion babies are a hallmark of most ARCI cases, clinicians should be aware of neonates with ARCI born without a collodion membrane and expedite appropriate workup and treatment.

Case Presentation

Successful management of congenital/infantile fibrosarcoma presenting as large, non-healing buttock ulceration

A two-year-old boy presented with a large, non-healing ulceration on his left buttock, which was originally noted as a brown patch present at birth. Punch skin biopsy was performed and histopathology revealed an atypical, pleomorphic, spindled proliferation in whorled fascicles replacing the dermis and trapping fat in the subcutis, consistent with a diagnosis of congenital/infantile fibrosarcoma. No evidence of metastatic spread was seen on imaging. The tumor was initially deemed unresectable owing to extent of local invasion. Neo-adjuvant chemotherapy caused significant tumor shrinkage and the patient underwent complete resection.

Asymptomatic flesh-colored lobular nodule on the nose

Chondroid syringoma is a rare benign tumor of the skin appendages. Chondroid syringoma is mostly manifested by a slow-growing, painless, well defined subcutaneous or intradermal nodule. A 43-year-old man presented to our clinic with the complaint of an asymptomatic nodule on the dorsum of the nose for 8 months. The lesion was totally excised. Histopathological examination was notable for epithelial islets embedded in the chondroid matrix in the dermis. The patient was diagnosed with chondroid syringoma in light of the clinical and histopathological findings.

A mixed form of intravascular papillary endothelial hyperplasia in an uncommon location: case and literature review

Intravascular papillary endothelial hyperplasia (IPEH) is an unusual benign, non-neoplastic vascular lesion that usually occurs in skin, but is uncommon in the oral cavity. Herein, we review the pertinent literature of oraiiPEH and report a new mixed form. A 61-year­ old man presented with an ulcerated nodule in the lingual portion of the gingiva related to the left mandibular canine. An excisional biopsy was performed presuming the clinical diagnosis of pyogenic granuloma. Histopathological analysis showed areas of granulation tissue consistent with pyogenic  granuloma. But in  addition, there  were thin-wall dilated vessels with papillary projections of endothelial cells producing vascular channels, associated with  an area of  organizing  thrombus. These microscopic findings led to the diagnosis of pyogenic  granuloma  associated with  IPEH. The immunohistochemical  reactions revealed a diffuse positivity of the vascular cells for CD-34 and smooth muscle actin antibodies.In addition,there was partial positivity for podoplanin and negativity for CD-1OS in the IPEH areas. No signs of recurrence were observed after 6 months of follow-up. The most prevalent site of IPEH in the oral region is the lower lip. IPEH is slightly more common in women and exhibits peaks of prevalence between the fourth and sixth decades of life.

Ulcerative cellulitis of the arm: a case of primary cutaneous cryptococcosis

Cutaneous cryptococcosis is usually secondary to the hematogenous dissemination of pulmonary or meningeal Cryptococcus neoformans. Primary cutaneous cryptococcosis (PCC) is a rare form of the infection, typically caused by direct inoculation from trauma to the skin [1]. Most cases of PCC present as a localized cellulitis, abscess, nodule, or ulceration. Herein, we present a case of a rapidly spreading cellulitis characterized by bullae and ulceration, caused by direct inoculation from a fall.

Photo Vignette

An intensely pruritic pebbled presentation

Lichen amyloidosis (LA) is a form of primary localized cutaneous amyloidosis (PLCA) characterized by bilateral intensely itchy domed scaly hyperkeratotic papules. Lichen amyloidosis is rare and affects men more than women. It is uncommonly seen in the western world but more prevalent in Asia. These papules most typically affect the shins and occasionally the arms and torso. Lichen amyloidosis has been reported in association with autoimmune disorders and after prolonged exfoliation and friction of affected skin. We present a 40-year-old woman with LA. In LA, the characteristic histological finding is apple-green birefringence of Congo red-stained preparations observed under polarized light. However, this is not always strongly positive, as in our patient. Other findings may include eosinophilia, periodic acid-Schiff positivity, staining with thioflavin T, and metachromasia after staining with crystal violet or methyl violet. Treatment of LA is difficult and complete clinical remission is seldom achieved. Recent trials revealed beneficial outcomes with topical calcipotriol, phototherapy, acitretin, cyclophosphamide, and laser treatments. A combination of acitretin, antihistamines, topical steroids, and hydrocolloid dressings have been beneficial in our patient with LA.

Letter

A case illustrating successful eradication of recurrent, aggressive basal cell carcinoma located in a scar with vismodegib

Vismodegib is a small molecule inhibitor of the Hedgehog signaling pathway that has shown efficacy in the control of locally advanced or metastatic basal cell carcinoma, although proof of its effectiveness in the elimination of aggressive tumors is lacking. We report a case and provide complete histological evidence of a 69-year-old gentleman who presented with a recurrent, infiltrative, and sclerosing (morpheiform) basal cell carcinoma on his left upper lip that was entirely eradicated with a three-month course of vismodegib 150 mg daily. Complete histologic clearance of a tumor in a recurrent, infiltrative, and sclerosing basal cell carcinoma with vismodegib is uncommon.

Growth of mobile applications in dermatology - 2017 update

Background: More than 80% of households in the US have a smartphone. Growth of mobile applications (apps) has grown in parallel with access to smartphones. Mobile health apps are used in medical fields, including dermatology. These apps allow patients to access information regarding dermatology conditions as well as access physicians via teledermatology.

Purpose: To analyze changes in number of dermatology mobile apps since 2014 and discuss benefits and drawbacks of mobile application growth to dermatology.

Methods: Apple, Android, and Windows were queried for dermatology-related apps. The apps were categorized by purpose and compared to previously published data to assess growth and change in dermatology apps.

Results: A total of 526 dermatology mobile apps were found corresponding to an 80.8% growth in dermatology apps since 2014. The market share of teledermatology increased from 11.0% in 2014 to 20.1% in 2017.

Conclusions: Dermatology apps continue to grow at a comparable pace to general app growth. Teledermatology apps experienced significant growth from 2014 to 2017. This growth has allowed time-efficient and cost-effective access to dermatologists, especially in rural areas. The growth of dermatology apps targeting patients allows for patient autonomy but also can result in access to inaccurate information regarding dermatology conditions.

Wardenburg syndrome type 2 in a woman with no genomic mutation commonly associated with the syndrome

Waardenburg Syndrome (WS) is a condition characterized by pigmentary changes of the hair or skin, hearing loss, heterochromia iridis, and dystopia canthorum. There are four main types of WS, which can be commonly caused by mutations in the PAX3, MITF, EDNRB, EDN3, SNAI2, or SOX10 genes. Herein, we present a patient with Waardenburg Syndrome type 2 with no findings of mutations in the commonly associated genes.

Bullous lichen sclerosus: isolated vulvar involvement

We present a patient with the bullous form of lichen sclerosus of the vulva. She had no lesions in other cutaneous and mucosal areas. We used topical tacrolimus and topical clobetasol propionate. The patient was lesion free at the first-year follow-up.

Fluctuating lesion of the scalp after a journey to the tropics: a case of furunculoid myiasis

Fluctuating lesions or furuncles of the scalp occur frequently in dermatological practice. This clinical condition is often caused by gram positive bacteria (e.g. staphylococcal or streptococcal skin infection) or fungal infection (e.g. Kerion celsi). However, a rare diagnosis such as myiasis might be considered, especially if a journey to an endemic area is reported. Herein, we present a case of furunculoid myiasis of the scalp and review the pathogenesis and therapeutic options to treat this condition.

A report and follow up of a patient with disseminated superficial actinic porokeratosis (DSAP) undergoing novel systemic treatment with palifermin (a keratinocyte growth factor) during chemotherapy

Disseminated superficial actinic porokeratosis (DSAP) is an inherited dermatosis triggered by chronic ultraviolet light exposure. Cosmetic improvement has been noted with topical therapies or laser treatment. Lesions of DSAP are precancerous, having been reported to develop into squamous cell carcinomas (SCCs) or basal cell carcinomas (BCCs) occasionally. We describe a patient with longstanding DSAP who developed a primary CNS lymphoma. Palifermin (a keratinocyte growth factor analogue) was used as an adjuvant for his chemotherapy to minimize mucositis. Our patient noted marked clinical improvement of his DSAP following his chemotherapy for lymphoma. Follow-up has shown a sustained improvement of his DSAP with no development of any SCCs. Palifermin may be safe for use in individuals at high risk of developing SCCs and further research into keratinocyte growth factors and risk of development of SCC is warranted. Systemic chemotherapy appears to improve DSAP lesions.

Comprehensive outreach, prevention education, and skin cancer screening for Utah ski resorts

Outdoor recreation can lead to substantial sun exposure. Employees of outdoor recreation establishments with extended time outdoors have amplified cumulative exposure to ultraviolet (UV) radiation and an increased risk of skin cancer. The “Sun Safe on the Slopes” program was created by Huntsman Cancer Institute at the University of Utah and the Utah Cancer Action Network to address increased UV exposure and skin cancer risk with free skin cancer screenings, outreach, and prevention education to local ski resorts. Herein, we describe the processes and barriers to implementation of a ski resort skin screening and education program and our 5-year report of the experience and screening data. Nine free skin cancer screenings were held at Utah ski resorts between 2011 and 2016, resulting in the presumptive diagnosis of 38 skin cancers (9.6%) in 394 participants. Behavioral data collected from participants indicates suboptimal sun safety practices, including underuse of sunscreen and protective clothing. Ski resort employees who experience sun exposure during peak hours at high altitudes and UV reflection from the snow are at an increased risk of skin cancer. These data indicate a need for emphasis on sun safety education and screening and can serve as a model for future endeavors.

Distance to pharmacy and risk of medication primary nonadherence

Primary nonadherence, a form of prescription nonadherence, is defined as failure to fill and pick up a prescription medication. Little is known about the relationship between distance to pharmacy and primary nonadherence in dermatology. In this study, we investigated the association between primary nonadherence and distance between a patient’s home and pharmacy. We focused on a low-income patient population within the dermatology clinic of a large, urban county hospital system in which patients were enrolled in a pharmacy benefit within a closed-system. Among 678 patients who were prescribed a total of 1156 prescription medications for dermatologic conditions, 11.7% did not pick up any of their prescriptions. After adjusting for patient demographics of race/ethnicity, sex, age, language, and relationship status, there was no association between primary nonadherence and distance traveled between a patient’s home and pharmacy. Results of this study are consistent with other studies in non-dermatologic patients and suggtableest that distance from a pharmacy may not be strongly associated with primary nonadherence for dermatologic medications.

Phytophotodermatitis induced by wild parsnip

Phytophotodermatitis results when skin is exposed to ultraviolet light after previous contact with a phototoxic compound. Wild parsnip (Pastinia sativa), a member of the Umbelliferae family, is an invasive plant species introduced to North America as a root vegetable. Although cultivated less commonly today, the plant is increasingly found growing wild in prairies and roadsides. The stems and leaves contain furocoumarins, which upon activation by UV light interact with oxygen. Resultant reactive oxygen species induce tissue damage manifesting initially as blistering and later as hyperpigmentation. We report the case of a woman who developed phytophoto-dermatitis after encountering wild parsnip on a midwestern prairie.