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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 6, 2018

Review

Cannabinoids in dermatology: a scoping review

The therapeutic applications of cannabis and cannabinoids are an increasingly conspicuous topic as de-criminalization and legalization of these products continues to expand. A limited number of cannabinoid compounds have been approved for a specific set of conditions. However, the current role of cannabinoids for the treatment of dermatologic conditions remains to be defined. We conducted a review of the current literature to determine the applications of cannabinoids for the therapy of various skin diseases. After conducting our analysis, we found that cannabinoid products have the potential to treat a variety of skin conditions, including acne vulgaris, allergic contact dermatitis, asteatotic dermatitis, atopic dermatitis, hidradenitis suppurativa, Kaposi sarcoma, pruritus, psoriasis, skin cancer, and the cutaneous manifestations of systemic sclerosis. However, the majority of available data on these compounds are pre-clinical and there is a corresponding lack of high-quality randomized, controlled trials that evaluate their effects. Cannabinoids have shown some initial promise as therapy for a variety of skin diseases. However, there is a requirement for thorough pre-clinical research and large-scale, randomized, controlled trials before cannabinoids can be considered safe and effective treatments for these conditions.

Original

Expediting patient appointments with dermatology rapid access clinics

Background: Lengthy wait times for dermatology appointments in the U.S. limit care access. The University of Pennsylvania's Department of Dermatology has established an urgent care clinic (UCC) and an intermediate care clinic (ICC) to expedite appointments for higher acuity patients.

Objective: To describe our rapid access clinics' operations, referral patterns, and distributions of diagnoses. Methods: We performed a retrospective review of dermatology consult order and appointment data for UCC, ICC, and routine care to determine the number of orders, consult appointments, and follow-up appointments; appointment wait times; and frequencies of diagnoses in referring provider and consult appointments. Press Ganey patient satisfaction ratings were also analyzed.

Results: The median (interquartile range) wait times for UCC, ICC, and routine care, appointments were 3 (1-8) days, 36 (15-64) days, and 45 (12-97) days, respectively (P<0.001). The proportion of referrals originating from subspecialists varied among UCC (47.6%), ICC (20.2%) and routine care (15.8%), (P<0.001). Distributions of diagnoses differed among UCC, ICC, and routine care. Ratings for most satisfaction metrics were similar across clinic settings.

Conclusions: Dermatology rapid access clinics within an academic medical center can reduce wait times for higher acuity patients while maintaining patient satisfaction.

Characteristics and diagnostic performance of pathologists who enjoy interpreting melanocytic lesions

Diagnostic discrepancy among pathologists interpreting melanocytic skin lesions (MSL) is an ongoing concern for patient care. Given that job satisfaction could impact patient care, this study aimed to characterize which pathologists enjoy interpreting MSL and estimate the association between enjoyment and diagnostic accuracy. Pathologists' demographics, training, and experience were obtained by a cross-sectional survey. Associations between these characteristics and self-reported enjoyment when interpreting MSL were estimated by Pearson's Chi-square tests. Diagnostic accuracy was determined by comparing pathologists' MSL interpretations with reference standard diagnoses. Associations between enjoyment and diagnostic accuracy were evaluated by generalized estimating equations (GEE) models. One hundred and eighty-seven (90%) pathologists completed the study. Seventy percent agreed that interpreting MSL is enjoyable. Pathologists who enjoyed interpreting MSL were more likely to be board certified and/or fellowship trained in dermatopathology (P=0.008), have ?10 years of experience (P=0.010) and have an MSL caseload of ?60 per month (P=<0.001). After adjustment, there was no association between enjoyment and diagnostic accuracy. Our data suggest that job dissatisfaction does not adversely affect diagnostic accuracy in the interpretation of melanocytic lesions, which is of importance given the progressive increase in annual biopsy rates and the attendant work demands imposed on pathologists.

Commentary

Addressing public criticism: a potential HIPAA violation

Although patients are able to speak openly about their healthcare experience in negative reviews, laws protecting the privacy of the patient constrain providers from responding as freely. Unfortunately, violation of this principle occurs when responding to online patient criticism. We describe a case of a physician assistant revealing protected health information of a patient in response to a critical New York Times article. Providers must be wary of violating patients' privacy, even when they are criticized online. Addressing patients' concerns and neutral, caring online responses may be the physician's best options for responding to negative reviews.

Reviewing the use of imiquimod for molluscum contagiosum

We discuss imiquimod associated with non-application site mucosal reactions and two of our own clinical cases. In one of our patients, erosive cheilitis developed in a young boy after using topical imiquimod 5% cream for 5 nights weekly on bilateral cheeks, chin, and near vermillion border for molluscum contagiosum. The case is discussed with concerns for imiquimod use in molluscum contagiosum when used near mucosal surfaces.

Case Presentation

Epidermotropic cutaneous metastases of squamous cell carcinoma from primary esophageal cancer: report of a case and review of the literature

Metastatic squamous cell carcinoma (SCC) to the skin can be distinguished histologically from primary cutaneous squamous cell carcinoma as, unlike the latter, it is typically separated from the normal overlying squamous epithelium. Rare cases have been reported of cutaneous metastases of SCC that demonstrate continuity with the overlying benign squamous epithelium, termed "epidermotropic cutaneous metastases of SCC." We report the first case of epidermotropic cutaneous metastases of SCC originating from primary esophageal SCC with a review of the literature on this rare histological phenomenon.

Striking enhancement at the site of radiation for nivolumab-induced Stevens-Johnson syndrome

Stevens-Johnson syndrome is a rare adverse cutaneous drug reaction characterized by epidermal detachment of <10% body surface area with an average mortality rate of 1-5%. The mechanism of SJS is not fully understood. Nivolumab is a monoclonal antibody directed against programmed cell death-1 protein (PD-1), a receptor with immune checkpoint inhibitory and antineoplastic activities. We present a case of SJS in a patient being treated with anti-PD-1 therapy nivolumab for metastatic squamous cell carcinoma of the oropharynx. This case is unusual because of the severe accentuation with striking enhancement at his prior radiation site and in the cutaneous region with heavier tumor burden from his metastatic disease. This reaction may give insight to the underlying pathophysiology of SJS, suggesting that immune checkpoint inhibitors can activate T-cells to target keratinocytes and that external factors may be involved in creating distinct epitopes for T-cell recognition. We hope this case adds to the body of knowledge in the pathogenesis of Stevens-Johnson syndrome and cutaneous adverse events seen with checkpoint inhibitors.

Golimumab-associated persistent erythema multiforme in a patient with ulcerative colitis in full remission

Erythema multiforme is an immune-mediated cutaneous disorder that is thought to represent a hypersensitivity reaction to infections, drugs, vaccines, malignancies, autoimmune diseases, radiation, and menstruation. Golimumab is a human IgG1-kappa anti-TNF antibody that has been approved for the treatment of rheumatoid arthritis, psoriatic arthritis, ankylosing spondylitis, and ulcerative colitis. We report herein a 41-year-old woman with persistent erythema multiforme, that occurred 18 months after onset of golimumab treatment of her ulcerative colitis; the latter remains in full remission over a period of 36 months.

Giant fibrous hamartoma of infancy: pitfall of CD34 positive dermal mesenchymal tumor

Fibrous hamartoma of infancy (FHI) is a rare benign soft tissue tumor with a triphasic organoid histologic appearance. The authors present a case of a 21-month-old healthy girl with a slowly growing flesh-colored subcutaneous plaque 12cm in size on the lower back, with overlying hypertrichosis. A punch biopsy revealed a proliferation of spindle cells infiltrating the dermis and hypodermis organized in a dense storiform pattern with a strong diffuse positivity for CD34. The diagnosis of congenital dermatofibrosarcoma protuberans (DFSP) was considered and an excision was performed. Histopathologic analysis showed an extensive poorly demarcated mass infiltrating the dermis and hypodermis, composed of different components: a monomorphous fibroblastic/myofibroblastic component, a mature adipose component, and an immature mesenchymal basophilic component. The clinical aspects with the histologic and immunohistochemical features led to the diagnosis of giant fibrous hamartoma of infancy. In our case the strong diffuse CD34 positivity was a diagnostic pitfall leading to an incorrect hypothesis of congenital DFSP. The characteristic triphasic histology of FHI was missed owing to the small size of the punch biopsy. This article highlights the importance of being aware of the CD34+ dermal mesenchymal tumor differential diagnosis and the necessity of appropriate size biopsies to avoid sampling error.

Estrogen receptor positive, progesterone receptor negative, leiomyoma of the areola of a male patient

Leiomyoma of the nipple and areola is a rare subtype of genital leiomyoma. The etiology of the tumor is not well understood. However, sex hormones like estrogen and progesterone have been implicated in the tumorigenesis. Hereby, we report a 47-year-old man with an estrogen receptor positive, progesterone receptor negative, leiomyoma of the areola.

Photo Vignette

Atrophic dermatofibroma in an elderly male - a rarely described variant of a common lesion

Dermatofibroma frequently presents as a red-brown nodule on the extremities of the middle aged. Atrophic dermatofibroma is a rare variant that has been most commonly described as an atrophic depressed, erythematous lesion in females. The correct diagnosis of atrophic dermatofibroma is often hindered by its infrequent presentation. It has a female preponderance with an occurrence ratio of 10:1. We describe a case of an atrophic dermatofibroma on the back of an elderly man. Skin biopsy demonstrated a spindle cell proliferation in a storiform pattern, loss of elastic fibers, and substantial atrophy of both the underlying dermis and subcutaneous tissue. An aggregation of elastic fibers was found in the periphery of the tumor. These histologic features supported the diagnosis of atrophic dermatofibroma. The dermal and adipocyte atrophy was likely responsible for the retracted appearance of the lesion.

Letter

A survey study with assessment of esophageal screening and genetic counseling in patients with Howel-Evans syndrome

Background: It is important to better understand the role that environmental risk factors play on the development of esophageal cancer in Howel-Evans families. Additionally, there is little published about appropriate esophageal cancer screening practices in families genetically confirmed to have this condition.

Methods: Surveys were distributed to 47 addresses of an American family with Howel-Evans syndrome, of which 29 responded and met inclusion criteria. Data was collected about demographics, environmental risk factors, and medical history of participants.

Results : We report characteristics of family members with tylosis, rates of esophageal cancer, rates of genetic counseling, and levels of environmental risk factors. Of the survey respondents, 43% reported features of tylosis, 71.4% were male and 28.6% were female and 28.6% reported leukoplakia. Only 21.4% of tylotic family members smoked, 65% drank alcohol, and 28.6% drank well water. More than half (57.1%) of the tylotic individuals had never had an esophagogastroduodenoscopy (EGD) and no one had been diagnosed with esophageal carcinoma. Only 3.4% of respondents had ever received genetic testing for Howel-Evans syndrome, despite genetic confirmation of their relatives.

Conclusions: We encourage dermatologists to discuss smoking-cessation, genetic counseling, and early EGD with affected families.

A recalcitrant case of Jacquet erosive diaper dermatitis after surgery for Hirschsprung disease in a boy with Waardenburg-Shah syndrome

We herein present a 4 year-old boy with Waardenburg-Shah syndrome who developed Jacquet erosive diaper dermatitis following a total colectomy and ileoanal anastomosis procedure for Hirschsprung disease. The diagnosis was made according to history and typical clinical findings. Complete resolution of the recalcitrant lesions after an ileostomy procedure supported the diagnosis. This case highlights the importance of being familiar with the predisposing factors and clinical presentation of this rare and severe form of chronic irritant dermatitis, since it may easily be misdiagnosed as other diseases in children and may lead to unnecessary diagnostic procedures, treatments, and anxiety due to suspicion of child abuse.

Fixed drug eruption related to sunitinib: a case report

Sunitinib is an oral multi-targeted tyrosine kinase inhibitor. It has been approved for the treatment of gastro-intestinal stromal tumor and advanced renal cell carcinoma. Fixed drug eruption related to sunitinib is a rare cutaneous adverse drug reaction.

Cadaveric simulation for improving surgical training in dermatology

Simulation models are rapidly changing medical education, especially the training of dermatology residents. Various models are available, including cadaveric simulations. Our study evaluates the impact of a cadaveric simulation on the training of dermatology residents. Over a period of three years, cadaveric simulation was shown to increase the surgical knowledge of residents. Residents were more confident in their knowledge of surgical anatomy and also surgical skills. Cadaveric simulation may offer a positive impact on resident training in dermatology.

Eruptive melanocytic nevi in HIV infected patients: report of three cases

The abrupt development of multiple melanocytic nevi has been described in association with many conditions, including human immunodeficiency virus infection. We report three cases of eruptive nevi in men with human immunodeficiency virus type 1 infection. One patient developed this phenomenon during the stage of acquired immunodeficiency syndrome. The other two patients had human immunodeficiency virus infection recently diagnosed and presented to our clinic reporting the development of multiple melanocytic nevi after starting highly active antiretroviral treatment, with improvement of their immunity. To our knowledge, this is the first report of eruptive melanocytic nevi as a possible consequence of the immune reconstitution inflammatory syndrome.

Addressing the average lifespan of skin diseases is critical to good patient care

Dermatology patients routinely ask how long their skin condition may last, yet this critical aspect of their care has not been emphasized in the literature. When a given diagnosis may be self-limited, it is essential that clinicians meet patient expectations by properly discussing the possible time course for resolution. Furthermore, being aware of, and prioritizing the knowledge of the duration of a skin disease can help limit continued exposure to side effects of prescribed treatments once the condition may have self-resolved or remitted.

Crusted scabies masquerading as psoriasis plaques in a patient suffering from burn scars

A 30-year-old woman, presented with erythematous scaling plaques on her trunk with severe pruritus and a burning sensation that began 3 months prior to her visit. She had a history of a thermal burn in that area, three years prior. Topical corticosteroid application for 3 months had no positive effects. Skin biopsy was done and scabies mites were found.

Pheniramine maleate: an apparently safe drug causing bullous fixed drug eruption

Fixed drug eruption is a delayed type hypersensitivity reaction to a drug seen most frequently with antibiotics such as tetracyclines, sulfonamides, and NSAIDs such as naproxen and ibuprofen. Although H1-antihistamines rarely elicit cutaneous adverse effects, there have been a few reports in the literature implicating them in causing fixed drug eruption, particularly the piperazine derivatives (hydroxyzine, cetirizine, levocetirizine), and loratadine. However, cutaneous drug reactions with the alkylamine derivatives like pheniramine maleate are extremely uncommon and fixed drug eruptions have not been reported with any of the alkylamine antihistamines to date. We herein report a case of multifocal bullous fixed drug eruption following ingestion of pheniramine maleate.

Subungual nail bed melanoma masquerading as tinea ungium

Subungual amelanotic melanoma can masquerade as onychomycosis. Recently a man whose amelanotic nail bed melanoma presented as persistent onychodystrophy was reported in the Dermatology Online Journal. The patient had a persistent nail dystrophy; culture and biopsy of the nail demonstrated Candida and dermatophyte infection, respectively. However, he subsequently presented with a nodule that was biopsied and demonstrated melanoma. Similar to that patient, we recently described a 67-year-old woman with a four-year history of persistent nail dystrophy of the left fourth fingernail who had a periodic acid-Schiff staining of the nail plate demonstrating fungal hyphae. Her nail plate subsequently detached, demonstrating a friable nodule; a biopsy of the nodule demonstrated melanoma. In conclusion, in individuals with new morphologic changes to a dystrophic nail or with persistent nail dystrophy despite appropriate therapy, it is important for clinicians to consider performing additional evaluation and possible biopsy to exclude malignancy.

Mycoplasma pneumoniae, more than a lung disease

Mycoplasma pneumoniae-induced rash and mucositis (MIRM) is a recently described clinical entity and should be considered in children who present with oral (94% of patients), ocular (82% of patients), and urogenital lesions (63% of patients). MIRM was first described as a distinct clinical entity from Stevens Johnson syndrome/Toxic epidermal necrolysis (SJS)/(TEN) in 2015 [1]. As a new, uncommon diagnosis it frequently poses a diagnostic and therapeutic challenge for pediatricians and dermatologists. We report a case of MIRM in a previously healthy 15-year-old boy.

Corrigendum

Corrigendum: Leukemia cutis as the presenting symptom of acute myeloid leukemia: report of three cases

The original article was published on July19, 2017 and corrected on June 15, 2018.

The revised version of the article adds appropriate in-text references to Figures 3B, C and 5B, C, and correctly renumbers the list of References. The changes appear in the revised online PDF copy of this article.