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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 23, Issue 9, 2017

Original

Future growth of physicians and non-physician providers within the U.S. Dermatology workforce

Trends in the training, supply, availability, career decisions, and retirement of US dermatology physicians are not well delineated. The current study evaluates whether growth in the dermatology workforce will keep pace with population expansion in the United States. A dermatologist supply model was projected to 2030 drawing on data from the American Academy of Dermatology, American Medical Association, Bureau of Labor Statistics, American Association of Medical Colleges, and other associations. The clinically active dermatologist workforce in 2015 was 36 per capita (1,000,000); entry following postgraduate training was age 30 with career separation at age 65 on average. Added to the provider model are physician assistants and nurse practitioners in dermatology practices. A linear regression micro simulation model based on age cohorts produced a per capita supply of dermatology providers of 61 (±3) per 1,000,000 by 2030, up from 47 in 2016. The dermatology workforce is growing faster than population expansion. Workforce estimates could be affected by changing trends in retirement and training of dermatology providers. Investments in training of nurse practitioners and physician assistants, in addition to training more doctors, may be an effective strategy for increasing access to care in populations with low dermatologist density.

Video-based education about systemic corticosteroids enhances patient knowledge more than verbal education: A randomized controlled trial

Background: Video-based patient education about long-term systemic corticosteroid treatment has not been assessed.

Objective: To compare video-based versus verbal education in patient knowledge gained and satisfaction.

Methods: English-speaking adults (≥18 years) were recruited from March-August 2013 from medical dermatology clinics. Study provider clinics were the unit of randomization. Verbal subjects heard a script based on the standard discussions of two top systemic corticosteroid prescribers at Emory. Video subjects viewed a video developed by the investigators. A 12-item survey created by the the investigators assessed baseline and post-education knowledge (immediate, one-, three-, and six-month).

Results: Baseline knowledge scores averaged 7.2±2.2 correct answers with no between-group differences. Post-education, the video group’s (N=39) mean paired score difference was 0.9±2.0 higher than the verbal group’s (p<0.04). After 1 month, most scores maintained gains with no between-group differences. 97% of patients in each group were satisfied (none were unsatisfied) with their education.

Limitations: Our cohort was more literate than the general public, and a minority of subjects completed long-term follow up assessments.

Conclusion: Video education enhanced near-term patient knowledge more than verbal education and maintained patient satisfaction.

Risky business: Behaviors associated with indoor tanning in US high school students

Background: Understanding of associations between indoor tanning and risky health related behaviors such as sexual activity and substance abuse among high school students across the United States is incomplete.

Objective: To identify risky health related behaviors among high school students utilizing indoor tanning and analyze differences between state specific data.

Methods: Results from the Youth Risk Behavior Surveillance System (YRBSS) 2013 in 14 different states were analyzed. Participants were 90,414 high school students. Responses to questions assessing indoor tanning habits, sexual activity, and use of substances were analyzed.

Results: Sexual activity was associated with indoor tanning in 10 of 14 states, with Nebraska having the strongest association (adjusted odds ratio, 3.8; 95% CI, 2.4-6.2; p<0.001). Indoor tanning was also associated with use of alcohol, marijuana, ecstasy, cocaine, prescription medications, and cigarettes.

Limitations: Only 15 states asked students about their personal history of indoor tanning use, and Minnesota was excluded from our analysis as they administered a non-YRBS questionnaire. Additionally, our study only analyzed results from the 2013 YRBS. Lastly, our data was analyzed in 14 individual data sets, giving a high likelihood of Type 1 error.

Conclusions: High school students utilizing indoor tanning are more likely to engage in sexual activity and substance abuse as compared to students who do not utilize indoor tanning.

Commentary

Assessment of risk and use of prophylaxis for glucocorticoidinduced-osteoporosis among dermatologists in the Pacific Northwest: a survey study

Objective: Exposure to even physiologic doses of glucocorticoids can reduce one's bone mass and increase risk for osteoporotic fracture. There currently exists a wide variation in clinician approach to the assessment and management of glucocorticoid-induced osteoporosis (GIO). Our objectives were to characterize Pacific Northwest dermatology providers' general practices, assessment of risk for GIO, and preferred GIO prophylaxis measures by way of survey. To identify whether knowledge deficits exist with respect to preventing and managing GIO in dermatology patients.

Design: A self-administered, 22-question survey was sent electronically to respondent population. Surveyed population composed of 392 dermatology providers of the Washington State Dermatology Association and Oregon Dermatology Society registries. Survey responses were collected anonymously via Catalyst WebQ.

Results: Respondents over-estimated fracture risk and reported they would prescribe antiresorptive medications at a less-than-adequate rate. When given clinical scenarios and asked to assess risk of major osteoporotic fracture, respondents frequently overestimated risk compared to that estimated by the FRAX tool (67%-71%). When asked directly if one would prescribe bisphosphonates as GIO prophylaxis for a high-risk patient, only 49% responded always/almost always.

Conclusions: This study suggests that a knowledge deficit exists within dermatology with respect to prevention and screening of GIO. The resultant practice gap is likely contributing to morbidity and mortality for dermatology patients requiring chronic glucocorticoid use for dermatologic disorders. Provider variability in practices suggests that dermatology could benefit from additional education in assessment and treatment of GIO, as well as a clear set of guidelines for GIO management.

Case Report

Associated conditions in patients with multiple dermatofibromas: Case reports and literature review

Dermatofibromas are benign, fibrohistiocytic, dermal tumors. Solitary dermatofibromas may be incidental findings, whereas multiple dermatofibromas may be associated with systemic conditions or previous therapies. Two women and one man with multiple dermatofibromas and an associated systemic condition, immunosuppression, or both, are described. Nine dermatofibromas developed in a woman with hypothyroidism, optic neuritis, and Arnold Chiari I malformation. Five dermatofibromas developed in a woman with breast cancer who had received several systemic antineoplastic therapies. Eleven dermatofibromas developed in a man with HIV whose systemic therapies included acyclovir, darunavir/cobicistat, dolutegravir, etravirine, and ritonavir. Conditions associated with multiple dermatofibromas include autoimmune diseases, cancer, chromosomal abnormalities, immunodeficiencies, metabolic disturbances, and altered physiologic states such as pregnancy. Medications received by patients with multiple dermatofibromas included immunosuppressive agents, psoriasis therapies, and antineoplastic drugs. Multiple dermatofibromas can be observed in patients with associated medical conditions, systemic therapies, or both. Therefore, in individuals presenting with multiple dermatofibromas, not only evaluation for associated disorders, but also review of prior and current drug therapies, should be considered.

Familial pseudoxanthoma elasticum associated with multiple comedones

Pseudoxanthoma elasticum (PXE) is an autosomal recessive disorder characterized by atypical elastic fibers that causes connective tissue abnormalities of the skin, eyes, and heart, among other organs. The disorder is rare, with a classic presentation of yellow-orange cobblestone-like papules on flexural areas, lax skin, ocular degeneration, and moribund vasculature in multiple organs. There is wide variability in the presentation of the affected organs [1]. We present two sisters with classic cutaneous findings of PXE with the additional unusual findings of numerous open comedones on the neck. To our knowledge, this is the first report of numerous open comedones in familial PXE.

Case Presentation

Eccrine squamous syringometaplasia in an allogenic stem cell transplant patient undergoing chemotherapy

Eccrine squamous syringometaplasia (ESS) is a rare finding defined as metaplastic change of the cuboidal epithelial cells of eccrine glands into two or more layers of squamous epithelial cells. We present a patient who developed ESS after induction of CLAG chemotherapy [2-Chlorodeoxyadenosine (2-CdA) with cytarabine (Ara-C) and (granulocyte-colony stimulating factor) G-CSF] for management of the blast crisis of his chronic myelogenous leukemia (CML). Our patient’s ESS eruption presented with a variety of morphologies, thus multiple skin biopsies were taken to determine the possible diagnosis(es). All skin biopsies showed ESS and the eruption resolved with topical corticosteroids after CLAG therapy was finished.

Simultaneous flagellate erythema in a husband and wife secondary to shiitake mushroom ingestion

Flagellate erythema secondary to shiitake mushroom (Lentinus edodes) ingestion is a condition that was first documented in 1977 by Nakamura and has been reported in Japan, Europe, and the United States. Herein, we present two cases of flagellate erythema after a couple ate a meal containing shiitake mushrooms at a chain restaurant. We hypothesize that this condition may not be as rare or as dependent on volume of exposure as previously suggested, considering that two genetically unrelated individuals simultaneously developed the eruption after minimal exposure.

Erythema multiforme major in a patient with metastatic melanoma treated with nivolumab

Nivolumab, a relatively novel immune checkpoint inhibitor with FDA approval in 2014, is gaining greater utilization due to its efficacy in treating metastatic melanoma. Many of the cutaneous immune-related adverse events (irAEs) being catalogued do not necessitate discontinuation of immunotherapy and are managed with supportive therapy. We present a case of erythema multiforme major secondary to nivolumab requiring hospitalization and discontinuation of treatment. This is only the second reported case of nivolumab-induced erythema multiforme in the literature we are aware of, and emphasizes the importance of oncologists working in conjunction with dermatologists for prompt diagnosis and management.

Lupus and scleroderma overlap features in a 28-year-old man with anti-PL-12 anti-synthetase syndrome

A 28-year-old man with clinically and laboratory diagnosed anti-PL-12 anti-synthetase syndrome (AS) in 2009 developed cutaneous lupus lesions, discoid lupus lesions, and sclerodacytly with finger-tip ulcerations four years following his AS diagnosis. Laboratory tests including +ANA, +anti-dsDNA antibody, +anti-Smith antibody, and +anti-RNP antibody in 2014 confirmed the diagnosis of progression to an overlap syndrome including systemic lupus erythematosus. The patient now also has clinical findings (sclerodacytly, Raynaud phenomenon, finger-tip ulcerations) consistent with scleroderma overlap. In each stage of his evolving connective tissue disease, cutaneous findings have been central to the recognition and monitoring of his overlap syndromes.

Acquired elastoma in a subungual location

Elastomas are connective tissue nevi or hamartomas. They may occur in isolation or can be associated with familial syndromes such as Buschke-Ollendorff syndrome. Elastomas typically present in childhood as small ivory papules or firm skin-colored nodules that can coalesce into larger yellow plaques. These lesions are typically distributed over the extremities, abdomen, and back. Herein, we report an unusual case of a renal transplant recipient who presented with an acquired subungual papule with associated koilonychia and distal nail plate dystrophy. Histopathologic findings were consistent with subungual elastoma.

Mycobacterium haemophilum infection in a renal transplant patient with inflammatory bowel disease

A 61-year-old immunosuppressed renal transplant patient with inflammatory bowel disease presented with tender pink nodules on the trunk and extremities. An initial biopsy was suggestive of metastatic Crohn disease, but after disease persistence, a second biopsy revealed disseminated Mycobacterium haemophilum. Atypical mycobacterial infections should be considered in immunosuppressed patients. This case highlights the complexities of diagnosing such infections in patients with an underlying granulomatous condition and the particular growth requirements of M. haemophilum.

Oral lesions as an important marker for HIV progression

Background: Oral hairy leukoplakia (OHL) is a benign lesion caused by Epstein-Barr virus (EBV) replication in the oral epithelium affecting the borders of the tongue. It is strongly associated with immunosuppression, especially in HIV+ adults but is uncommon in pediatric population. The aim of the study is to show the importance of the correct diagnosis of OHL and its influence on HIV treatment.

We report two cases of HIV+ adolescent patients that presented with leukoplakic lesions on the border of the tongue, suggestive of OHL. OHL diagnosis was confirmed in only one case through EBV in situ hybridization. After confirmation of the diagnosis, the patient with OHL was referred to an infectious disease specialist with the decision to start antiretroviral therapy. Conclusion: OHL definitive diagnosis can help clinical management of pediatric HIV+ patients.

Histiocytoid autoimmunity-related neutrophilic dermatosis in a patient with rheumatoid arthritis

Autoimmunity-associated neutrophilic dermatoses are a recently recognized manifestation of connective tissue diseases, in particular, lupus erythematosus. These entities are clinically and sometimes histopathologically distinct from classic neutrophilic dermatoses. We describe a case of an autoimmunity-related neutrophilic dermatosis in a patient with rheumatoid arthritis. In addition to this uncommon association, there was an absence of mature neutrophils and a population of immature histiocytoid granulocytes. This unusual case expands the concept of histiocytoid neutrophilic dermatoses to include those seen in association with autoimmune connective tissue diseases.

Psoriasiform mycosis fungoides: a rare form of the disease with review of the literature

Background: Mycosis fungoides (MF) typically presents as erythematous scaly patches or plaques that may progress to cutaneous tumors. Although MF may be presented like other dermatoses, initial presentation as psoriasiform plaques simulating psoriasis is rare. Differentiating MF from psoriasis is important because systemic therapies used for psoriasis can worsen MF. We describe a case of psoriasiform MF and we also review the clinicopathological features of similar cases in the literature.

Case: A 46-year-old woman was referred to our clinic with a history of psoriasiform plaques for 13 years. She had multiple, generalized, indurated plaques with thick psoriasiform scales that were unresponsive to topical treatments. The histopathology showed marked psoriasiform epidermal hyperplasia with epidermotropic atypical lymphocytes compatible with MF. Immunohistochemical (IHC) staining showed that atypical lymphocytes were positive for CD3, CD4, CD8, and CD5. Of note, upper dermal and intraepidermal large atypical lymphocytes were CD30 positive. The review of similar psoriasiform MF cases revealed that they had all been treated as psoriasis for many years and finally diagnosed as MF especially after deterioration induced by immunosuppressive therapies.

Conclusions: In presumed cases of psoriasis that are unresponsive to treatment, progressive, or ulcerative, biopsy should be considered to rule out MF, particularly before starting a potent immunosuppressive agent.

Photo Vignette

Painful subcutaneous nodules on the thigh

Osteoma cutis is the presence of bone within the dermis or subcutaneous tissue. This condition may occur sporadically or secondary to other dermatologic or genetic conditions. We present a 12-year-old girl with pseudohypoparathyroidism type-Ia who developed osteoma cutis on the right thigh.

Generalized eruptive syringomas

Eruptive syringoma is a rare variant of syringoma, benign neoplasms of the eccrine sweat ducts that appear on the face, neck, chest, and axillae of predominately Asian and African American women before or during puberty [1, 2]. Lesions appear as small skin-colored or slightly pigmented, flat-topped papules [2]. The condition can be cosmetically disfiguring and difficult to treat, especially in dark-skinned patients. The investigators report a 52-year old Guyanese woman who presented with widespread, chronic, non-pruritic and nontender, skin-colored papules that arose approximately 20 years earlier. A punch biopsy of affected skin was obtained and the histological diagnosis was eruptive syringoma. The patient pursued no further treatment, after discussion of costs and risks.

A pedunculated lesion on the foot: acquired fibrokeratoma

Acquired fibrokeratomas are benign and uncommon lesions consisting of collagenous papules and nodules covered by hyperkeratotic epidermis. These tumors occur mainly on the fingers and toes and infrequently on the palms and soles. They may possibly be triggered by a reaction to a trauma and presents as small and solitary dome-shaped lesions with a collarete of slightly raised skin at the base. Several case reports have been published of this rare lesion, with only a few investigators describing lesions of the feet.

A giant apocrine hidrocystoma of the trunk

Hidrocystomas are benign cysts that typically present as translucent, bluish dermal nodules on the face and are rarely > 1 cm in size. They are classically categorized as eccrine or apocrine based on histologic features. We present a rare case of a giant apocrine hidrocystoma of the trunk, demonstrating that, although a rare variant, apocrine hidrocystomas can present both off the head and neck, and can be significantly larger in size than previously reported.

Letter

Local skin reactions following the administration of topical ingenol mebutate for actinic keratosis

Actinic keratoses are cutaneous lesions that appear as the result of the proliferation of atypical keratinocytes. These lesions are considered pre-malignant and they can progress to squamous cell carcinoma. Ingenol mebutate has been approved as an effective treatment for AK on the face and trunk. We studied the local skin reactions to this therapy. Data about local skin reactions were collected in a series of 5 patients with photographic documentation, a visual analog scale, and a ranking of satisfaction of the patient. Moderate to severe reactions were reported in most of patients, but only one stopped treatment early. The short duration of treatment contributes to high adherence to the therapy.

Mycobacterium fortuitum infection after acupuncture treatment

Actinic keratoses are cutaneous lesions that appear as the result of the proliferation of atypical keratinocytes. These lesions are considered pre-malignant and they can progress to squamous cell carcinoma. Ingenol mebutate has been approved as an effective treatment for AK on the face and trunk. We studied the local skin reactions to this therapy. Data about local skin reactions were collected in a series of 5 patients with photographic documentation, a visual analog scale, and a ranking of satisfaction of the patient. Moderate to severe reactions were reported in most of patients, but only one stopped treatment early. The short duration of treatment contributes to high adherence to the therapy.

Nasolabial flap – alternative uses for a classic but versatile technique

The nasolabial flap is one of the most ancient techniques used in orofacial surgery. The authors report two cases of patients with skin cancer treated surgically with variations of the classic nasolabial flap by transposition (bilateral and folded) that highlight the broad applicability of this technique.

Clinical resolution of pemphigus vulgaris on rituximab

Although significant progress has been made for the treatment of pemphigus vulgaris (PV) with rituximab (RTX), a consensus remains to be determined for standard treatment protocol, regarding optimal dosing, infusion regimen, and use of concomitant immunotherapy to achieve safe, effective, and rapid clinical response. We describe a patient with pemphigus vulgaris treated with high dose rituximab with the rheumatoid arthritis protocol along with intravenous immunoglobulin therapy. This case provides evidence towards the growing body of research needed to modify and improve treatment for pemphigus using rituximab.