What are the ethical and legal considerations when your patient refuses the standard of care?

In medical practice, physicians are sometimes faced with patients who reject the gold-standard treatment for a condition. In this hypothetical clinical scenario, we present the case of a patient who refuses Mohs micrographic surgery for management of infiltrative basal cell carcinoma and instead requests off-label therapy with imiquimod. We discuss the treating dermatologist's options in response to this patient's request and the ethical considerations surrounding the case. We conclude that the physician has the right to refuse to provide treatment that deviates from standard clinical practice but that the physician should counsel the patient on all options, provide thorough informed consent, offer contact information for the patient to pursue a second opinion or a radiation oncology referral, and ensure safe transfer of care should the patient desire treatment with a different provider.

Primary cutaneous Hodgkin-like polymorphic post-transplant lymphoproliferative disorder.

Post-transplant lymphoproliferative disorder (PTLD) is an uncommon complication after solid-organ transplants and hematopoietic stem cell transplants. Isolated involvement of the skin without systemic involvement in PTLD is extremely rare. Primary cutaneous PTLD is generally categorized as either cutaneous T-cell lymphoma or cutaneous B-cell lymphoma, with variable Epstein-Barr virus (EBV) positivity. Herein, we describe an exceedingly uncommon case of a primary cutaneous Hodgkin-like polymorphic PTLD. A man in his 60s, with a history of kidney transplant, presented with a 5-week history of two indurated plaques. Clinical, histologic and immunohistochemical findings were consistent with primary cutaneous Hodgkin-like polymorphic PTLD. Reduction in immunosuppression led to resolution of his lesions. This case highlights a rare case of primary cutaneous Hodgkin-like PTLD and increases awareness of this uncommon post-transplant complication. It also underscores the importance of collaboration between dermatology, hematology, dermatopathology and hematopathology in order to diagnose challenging cases.

A review of N-acetylcysteine in the treatment of grooming disorders.

BACKGROUND: Pathologic grooming disorders can lead to clinically significant distress and functional impairment. Studies on treatment of these disorders with selective serotonin reuptake inhibitors (SSRIs) and anticonvulsants have led to inconsistent findings. N-acetylcysteine (NAC) has shown promise in treatment of obsessive-compulsive and related disorders. The objective of this article is to perform an updated review of NAC in the treatment of grooming disorders. METHODS: PubMed was searched from inception to October 2017 to identify literature on the use of NAC in the management of trichotillomania, onychophagia, and pathological skin picking. Case reports, case series, and randomized controlled trials were included. Data on study design, dosing regimens, comorbidities, concurrent treatment, and side effects were extracted from the included articles. RESULTS: Fifteen articles were included in this review, which consisted of 10 case reports, one case series, and four randomized controlled trials. Dosing of oral NAC ranged from 450 to 2,400 mg per day, and treatment periods lasted from 1 to 8 months. Side effects were uncommon, mild, and usually gastrointestinal in nature, with severe aggression reported in one child. CONCLUSIONS: While there are multiple reports of the safety and efficacy of NAC in the treatment of grooming disorders, there are currently few randomized controlled trials on this topic, and more research is needed to develop a formal treatment algorithm. While current data should be considered very preliminary, case reports have demonstrated mostly positive results and a lack of significant side effects. A trial of NAC may be a viable option for pathologic grooming disorders, especially in patients who have failed prior psychologic or pharmacologic treatment.

Social media in dermatology: clinical relevance, academic value, and trends across platforms.

Social media encompasses multiple sites for online communication that reach a large proportion of the global population. While the increase in number of publications investigating dermatology content on social media has paralleled the growth of social media in the past decade, there has not yet been an extensive review of past literature on this topic. Our objective is to summarize and analyze publications on the role of social media within the field of dermatology. Social media is frequently used by patients to solicit advice related to cutaneous concerns and even when seeking out a dermatologist, but it is underutilized among both clinicians marketing their practices and academicians collaborating with colleagues in the field. Social media has the potential to advance instruction for trainees in dermatology and facilitate new research methods. Patient-centered perspectives and advocacy groups have a larger presence overall on social media platforms than stakeholders in academic dermatology. Furthermore, specific platforms like Facebook, YouTube, and Twitter vary in originators of material, messaging strategies, and reliability of information with regards to certain dermatologic conditions and behaviors.

Management of Complex Arteriovenous Malformations Using a Novel Combination Therapeutic Algorithm.

Importance: Current therapeutic options for patients with extracranial head and neck arteriovenous malformations are limited. Surgical intervention, such as sclerotherapy or resection, often result in rapid recurrence and progression of disease. Objective: To assess the efficacy and tolerability of sirolimus as an adjuvant therapy for endovascular embolization in the management of complicated extracranial head and neck arteriovenous malformations. Design, Setting, and Participants: This case series examined 6 patients with extracranial head and neck arteriovenous malformations treated from January 1, 2013, to December 31, 2017, at a multidisciplinary vascular anomalies clinic within Stanford Hospital and Clinics. Intervention: Initiation of sirolimus at least 1 month prior to endovascular embolization, targeting a trough level of 10 to 15 ng/mL throughout the course of the endovascular embolization series and continued for at least 1 month after the series. Main Outcomes and Measures: Clinical manifestations; disease progression and overall response to treatment were assessed via clinical evaluation and radiographic imaging. Results: All 6 patients (4 male and 2 female patients; mean age, 24.5 years [range, 9-44 years]) responded favorably to the combination of sirolimus therapy followed by endovascular embolization, and 4 patients exhibited a near-complete response. The median duration of follow-up was 19 months (range, 6-40 months). One patient discontinued sirolimus soon after embolization and experienced regrowth of the arteriovenous malformation after 1 year. Sirolimus was resumed, which has stabilized his disease for more than 2 years. Mild adverse effects were noted in 4 patients. The combination therapy was well tolerated in all patients. One patient developed skin ulceration after embolization and required surgical debridement. Another patient developed pulmonary microthrombi after embolization with cyanoacrylate glue that resolved with a brief course of anti-inflammatory therapy. Conclusions and Relevance: Although further prospective trials are needed, this report suggests the benefit of a mammalian target of rapamycin inhibitor as an adjuvant therapy for surgical embolization of complex, extracranial head and neck arteriovenous malformations. The optimal dosing and therapeutic duration of sirolimus treatment before and after embolization remain to be determined.

Infiltrative basal cell carcinoma mimicking tinea corporis.

The diagnosis of infiltrative basal cell carcinoma (BCC) can be delayed owing to its often subtle clinical findings. A 90-year-old woman presented with an asymptomatic annular pink plaque on her left shin that was clinically diagnosed as tinea corporis. After years of not responding to topical anti-fungal therapy, biopsies confirmed a diagnosis of infiltrative BCC. We discuss the differential diagnosis of the case, the difficulties in identifying infiltrative BCC, and the pathologic features of infiltrative BCC.

Sclerotherapy for lymphatic malformations of the head and neck in the pediatric population.

BACKGROUND: Sclerotherapy is one of the most commonly used minimally invasive interventions in the treatment of macrocystic lymphatic malformations (LMs). Several different sclerosing agents and injection protocols have been reported in the literature, each with varying degrees of success. The safety and efficacy of the treatments have not been evaluated comparatively in the pediatric population. METHODS: Chart review of pediatric patients with macrocystic/mixed head and neck LMs who underwent sclerotherapy using OK-432, doxycycline, or ethanolamine oleate at Lucile Packard Children's Hospital at Stanford during 2000-2014. Clinical evaluation and radiographic imaging were reviewed to assess lesion characteristics and response to sclerotherapy following each treatment session. The post-intervention clinical response was categorized as excellent, good, fair, or poor. RESULTS: Among the 41 pediatric cases reviewed, 10 patients were treated with OK-432, 19 patients received doxycycline, and 12 patients received ethanolamine. In univariate analysis, different sclerosants had similar effectiveness after the first injection and final clinical outcome (p=0.5317). In multivariate analysis controlling for disease severity stage as well as disease characteristics (macrocystic vs mixed subtypes), different sclerosants also had similar effectiveness after the first injection (p=0.1192). Radiologic analysis indicated an 84.5% average volume reduction, with similar effectiveness between the different sclerosants (p=0.9910). CONCLUSIONS: In this series of LM cases treated at Stanford, we found that doxycycline, OK-432, and ethanolamine oleate sclerotherapy appear to have a similar safety and efficacy profile in the treatment of macrocystic and mixed LMs of the head and neck in the pediatric population.

Combining biologic and phototherapy treatments for psoriasis: safety, efficacy, and patient acceptability.

BACKGROUND: The efficacy and safety of biologic and phototherapy in treating moderate-to-severe psoriasis is well known. However, some patients may not respond well to biologic agents or phototherapy on their own and may require combination therapy. Skillfully combining a biologic agent and phototherapy may provide an additive improvement without much increase in risks. OBJECTIVE: To summarize the current state of evidence for the efficacy and safety of combining biologics with phototherapy in the treatment of moderate-to-severe plaque psoriasis. METHODS: We conducted an extensive search on Pubmed database for English language literature that evaluated the use of a combination of biologic and phototherapy for the treatment of moderate-to-severe psoriasis through January 2016. The search included the following key-words: psoriasis, etanercept, adalimumab, infliximab, ustekinumab, biologics, phototherapy, and combination therapy. RESULTS: The primary literature included randomized controlled trials, a head-to-head study, open-label controlled and uncontrolled trials, case series, and case reports. Etanercept was used in over half of the reported cases, but other biologic agents used included ustekinumab, adalimumab, and infliximab. The vast majority of phototherapy was narrowband ultraviolet B (NBUVB) radiation. Most cases reported enhanced improvement with combination therapy. Serious adverse events throughout the study duration were reported in <3% of the patients. Long-term adverse events cannot be excluded. CONCLUSION: Combination of biologic and phototherapy appears to be a viable clinical strategy in the treatment of moderate-to-severe psoriasis not responsive to monotherapy, despite limitations in the data available. NBUVB in combination with biologics appears to be especially effective. However, the long-term impact of these combinations is yet to be determined.

Narrowband UV-B Phototherapy for Steroid-Refractory Sclerotic Chronic Cutaneous Graft-vs-Host Disease.

IMPORTANCE: Chronic graft-vs-host disease (GVHD) affects 50% to 70% of patients who receive allogeneic hematopoietic cell transplants (HCTs), and the skin is the most common site of involvement. Chronic cutaneous GVHD can present with sclerotic or nonsclerotic changes of the skin and often requires treatment with systemic immunosuppressants, extracorporeal photopheresis, or phototherapy. We describe the first reported case, to our knowledge, of the effective treatment of sclerotic chronic cutaneous GVHD with narrowband UV-B (NB UV-B) phototherapy. OBSERVATIONS: A woman in her 40s presented with sclerotic chronic GVHD of the skin 6 years after HCT for treatment of chronic myelogenous leukemia. The patient's cutaneous disease progressed despite treatment with prednisone and oral tacrolimus. The patient was initiated on NB UV-B phototherapy 3 times per week, resulting in clinically significant improvement of cutaneous lesions over the first 2 months. The NB UV-B regimen allowed for a reduction of prednisone dose and continued control of cutaneous GVHD over 6 months of therapy. CONCLUSIONS AND RELEVANCE: Our case report describes the successful use of NB UV-B phototherapy for the treatment of sclerotic chronic cutaneous GVHD. Further study should be performed to evaluate the effectiveness of this therapeutic modality for patients with sclerotic chronic cutaneous GVHD.

Treatment of keratitis-ichthyosis- deafness (KID) syndrome in children: a case report and review of the literature.

Keratitis-ichthyosis-deafness (KID) syndrome is a rare hereditary cornification disorder resulting from mutations in connexin 26, a protein important for intercellular communication. In addition to the characteristic clinical triad of congenital bilateral sensorineural hearing loss, keratitis, and erythrokeratoderma, affected individuals also suffer from chronic bacterial and fungal infections and have an increased risk of benign and malignant cutaneous tumors. Treatments with antibiotics, antifungals, and systemic retinoids have been reported with variable response. Ocular and skeletal toxicity from prolonged exposure to systemic retinoids is a major concern especially in children. We report a case of a 7-year-old boy with KID syndrome complicated by frequent infections who responded well to acitretin 0.5-1.0 mg/kg/day. The patient had significant improvement of the hyperkeratosis on the scalp, trunk, and extremities within 4 weeks after initiating treatment. The patient has been on treatment for over a year without notable ocular, skeletal, or laboratory side effects. A review of the literature focusing on therapeutic options for KID syndrome and concerns about safety and tolerability is presented.

Delusions of parasitosis; suggested dialogue between dermatologist and patient.

Delusions of parasitosis (DoP) is a psychocutaneous condition characterized by a fixed false belief that one is infested by skin parasites. Patients afflicted with DoP generally experience sensations of biting, stinging or crawling in the absence of any objective evidence of infestation. The most definitive treatment for DoP is antipsychotic agents. Though the diagnosis and treatment options are rather straightforward, the difficulty lies in the art of building a therapeutic rapport with the patient in order to encourage acceptance of antipsychotic treatment. This article is a practical guide that suggests verbatim how dermatologists might talk to a delusional patient in order to establish a strong therapeutic rapport. Strategies on how to optimize the initial encounter, build rapport and prescribe antipsychotic medications that are likely to be accepted by the patient are discussed.