Alain Brassard

Chronic oedema/lymphoedema: under-recognised and under-treated

Even though it is estimated that at least 300 000 people in Canada may be affected by chronic oedema/lymphoedema, recognition of the seriousness of this chronic disease in health care is scarce. Lymphoedema affects up to 70% of breast and prostate cancer patients, substantially increasing their postoperative medical costs. Adding to this problem are the escalating rates of morbid obesity across North America and the fact that 80% of these individuals are thought to suffer with an element of lymphoedema. The costs related to these patient populations and their consumption of health care resources are alarming. Untreated chronic oedema/lymphoedema is progressive and leads to infection, disfigurement, disability and in some cases even death. Thus, prognosis for the patient is far worse and treatment is more costly when the disease is not identified and treated in the earlier stages. Although the number of individuals coping with chronic oedema/lymphoedema continues to increase, the disparity between diagnosis, treatment and funding across Canada endures. The reasons for this include a lack of public awareness of the condition, insufficient education and knowledge among health care providers regarding aetiology and management and limited financial coverage to support appropriate methods and materials.

Pyoderma Gangrenosum: An Update on Pathophysiology, Diagnosis and Treatment.

Pyoderma gangrenosum (PG) is a rare inflammatory neutrophilic disorder with prototypical clinical presentations. Its pathophysiology is complex and not fully explained. Recent information regarding the genetic basis of PG and the role of auto-inflammation provides a better understanding of the disease and new therapeutic targets. PG equally affects patients of both sexes and of any age. Uncontrolled cutaneous neutrophilic inflammation is the cornerstone in a genetically predisposed individual. Multimodality management is often required to reduce inflammation, optimize wound healing, and treat underlying disease. A gold standard for the management of PG does not exist and high-level evidence is limited. Multiple factors must be taken into account when deciding on the optimum treatment for individual patients: location, number and size of lesion/ulceration(s), extracutaneous involvement, presence of associated disease, cost, and side effects of treatment, as well as patient comorbidities and preferences. Refractory and rapidly progressive cases require early initiation of systemic therapy. Newer targeted therapies represent a promising pathway for the management of PG, and the main focus of this review is the management and evidence supporting the role of new targeted therapies in PG.

Fact or Fiction: Does the Non-HIV/AIDS: Immunosuppressed Patient Need Pneumocystis Jiroveci Pneumonia Prophylaxis? An Updated Literature Review

Background: Pneumocystis jiroveci pneumonia (PJP) is a potentially fatal fungal infection occurring in immunocompromised patients. Objective: To determine whether PJP prophylaxis is required in the non-human immunodeficiency virus/acquired immune deficiency syndrome (HIV/AIDS) immunocompromised patient and, if so, the optimal prophylactic therapy. Methods: A thorough literature review, with the appropriate MeSH terms, was conducted using PubMed, Medline, and The Cochrane Database. A number of cases describing PJP in patients with various systemic diseases and immunosuppressive medications, along with a Cochrane review, were highlighted. Results: Although there are a number of case reports in the literature, the only collagen vascular disease with an increased incidence of PJP is Wegener granulomatosis. Oral trimethoprim-sulfamethoxazole continues to be the prophylaxis of choice for PJP. Conclusion: There is currently no evidence to recommend PJP prophylaxis in the non-HIV/AIDS immunocompromised population. If physicians do decide to use prophylaxis, they should always weigh the benefits with the potential risks. Further studies are needed to better quantify the risks of PJP with immunosuppressive medications.

Peripheral neuropathy for dermatologists: what if not diabetic neuropathy?

Background: Patients with cutaneous manifestations associated with peripheral neuropathy often present to the dermatologists office. Objective/Methods: This article outlines a practical approach for obtaining the history, performing a screening physical examination, and ordering initial diagnostic testing to diagnose the cause of nondiabetic neuropathy. When to refer for neurologic consultation and principles of management of neuropathic pain and neuropathy-related ulcers are also discussed. Results: Cutaneous manifestations of peripheral neuropathy may be secondary to a medical condition predisposing the patient to neuropathy or a manifestation of neuropathy itself. In the latter category, skin affected by neuropathy may show characteristics of xerosis, anhidrosis, rubor, edema, callus, ulceration, muscle wasting, and foot deformity. Most often these findings occur in association with diabetic neuropathy; however, many other infectious, inflammatory, metabolic, paraneoplastic, hereditary, and medication- or toxin-related causes should be considered. The treatment of cutaneous manifestations of neuropathy includes pressure downloading, control of edema, and optimal ulcer and neuropathic pain management. Conclusion: It is important for dermatologists to have a basic approach to neuropathy in patients with related skin disease. Referral to Neurology is warranted when basic workup for reversible causes is negative or for any severe, rapidly progressive symptoms.

Relapsing Polychondritis: A Review and Guide for the Dermatologist

Relapsing polychondritis, or RP, is a rare connective tissue disease characterized by relapsing-remitting destructive inflammation of the cartilaginous and other proteoglycan-rich structures in the body. Given the relatively low incidence of RP, a concise clinically relevant guide, focusing on the cutaneous manifestations of this serious disease, is lacking. In this review, we provide the dermatologist with an approach to diagnosing RP and a guide to its initial work-up, and management. We close with an overview of the currently available treatment modalities for RP.

Pyoderma gangrenosum associated with alitretinoin therapy

Pyoderma gangrenosum (PG) is an uncommon ulcerative cutaneous condition, the etiology of which is poorly understood but is thought to be related to improper neutrophil function and chemotaxis. PG is a clinical diagnosis that requires exclusion of other ulcerative and inflammatory cutaneous disorders. A rare side effect of isotretinoin is the development of PG. We report the first case, to our knowledge, of PG associated with alitretinoin therapy (Appendix).

Lyme Borreliosis: An Update for Canadian Dermatologists

Background: Lyme borreliosis is a multisystemic tick-borne spirochetosis, which may result in dermatologic, musculoskeletal, cardiovascular, and neurologic manifestations. Objective: Patients with suspected acute Lyme borreliosis infection may be referred for urgent dermatologic review. Canadian dermatologists should be aware of the latest information regarding the diagnosis and management of Lyme borreliosis. Methods: This review is based on a PubMed database search combining the word “Lyme” with variations of the word “Canada.” Data sources included articles from the fields of ecology, epidemiology, laboratory diagnostics, and clinical management. Conclusion: In this review, the ecological basis of spirochete transmission by tick vectors is described. The latest available Canadian epidemiologic data are summarized. North American clinical manifestations of Lyme borreliosis are contrasted with European presentations. The Canadian Public Health Laboratory Networks diagnostic guidelines are summarized. Finally, treatment recommendations are outlined.

Adalimumab in treatment-resistant hidradenitis suppurativa following recurrence after extensive affected area excision: a review of biologics therapy.

Background: Hidradenitis suppurativa (HS) is characterized by chronic, suppurative abscesses, sinus tracts, and fistulas affecting the axilla, groin, and perianal region resulting from hyperkeratosis and occlusion of the terminal hair follicle. Objective: This report highlights the use of biologic agents for the treatment of recalcitrant HS. Method: We report on a 48-year-old male with a 15-year history of refractory perianal-inguinal-buttock HS who, despite receiving numerous surgical drainages and traditional medical treatment for HS, still had severe pain. After trialing etanercept and infliximab with methotrexate, the patient had marked improvement with adalimumab. A literature review of biologics therapy was also performed. Results: After trialing many traditional therapies, we found that adalimumab appears to be the most effective treatment modality for our patient. A literature search revealed 53 articles on biologics therapy in HS. These articles are summarized. Discussion: Biologic agents have been shown to have variable results in the treatment of refractory HS. Enough low-grade evidence has been accumulated to make the use of these agents suitable in HS. Until more clinical trials are performed on this topic, physicians should use clinical judgment when treating HS with biologic agents and be cautious by watching for significant adverse effects.

Erythema induratum: case series illustrating the utility of the interferon-γ release assay in determining the association with tuberculosis.

Background: The interferon-γ release assay (IGRA) is a novel method for detecting previous sensitization to tuberculosis (TB). Despite having several advantages over the tuberculin skin test (TST), including higher specificity and no influence from past bacille Calmette-Guérin (BCG) exposure, there are a limited number of reports describing its application in patients with erythema induratum (EI)/nodular vasculitis (NV), which is usually but not always related to TB. Objectives: The aim of our case series was to evaluate the usefulness of the IGRA for determining a TB association in patients with EI/NV. Methods: Retrospective chart reviews were conducted on four patients diagnosed with EI/NV at our institution in whom an IGRA had been performed. Results: All four subjects had positive TST results. The IGRA was also positive and therefore supported a link with TB in two cases. One patient responded completely to anti-TB therapy, whereas the second was lost to follow-up. Both cases unrelated to TB, by virtue of negative IGRAs, demonstrated complete response to immunosuppressive therapy (methotrexate), with one individual having failed anti-TB therapy first. Conclusion: Our case series highlights the utility of the IGRA for establishing a TB association in patients with EI/NV. Although limited by a small sample size, we propose adjunctive use of this test at the time of EI/NV diagnosis, especially in the setting of previous BCG exposure, so that management can be tailored according to whether an underlying relationship with TB exists.

Definition of Moderate to Severe Hidradenitis Suppurativa: A Position Paper by the Canadian Hidradenitis Suppurativa Foundation (CHSF).

To the Editor: Hidradenitis suppurativa (HS) is a chronic recurrent debilitating follicular skin disease with significant burden on both patients and the health care system. It commonly presents after puberty with painful deep-seated nodules, abscesses, and subsequent scarring, and sinus formation in apocrine-bearing skin. Despite being an important health problem during an individual’s most productive time of life, the information on patterns and burden of this condition in Canada is very limited. This information is highly relevant because of the publicly funded health care system in Canada, which is covering direct and potentially indirect costs associated with chronic conditions, such as HS. While even one active lesion can be severely disabling, HS severity is commonly categorized as being mild, moderate, or severe. Considering that even a mild HS can be severely incapacitating, the description of moderate to severe disease requires consideration of both patient and disease perspectives. A spectrum of major subtypes and several rare manifestations illustrates the great variety of clinical presentations and clinical course. HS is a personal crisis for people living with the disease and at the same time a financial crisis for our health care system. The delay in diagnosis, fragmented care, and concomitant comorbidities are consuming an ever-larger share of health care budgets and will force an increase in expenditures. HS has a variable clinical course, and multiple instruments have been developed to gauge severity and stages of the disease (Table 1). Table 1 compares different types of HS scoring systems. With regard to HS, clinical assessment tools must account for the inflammatory nature of HS in order to have clinical relevance. Multiple modalities have been used in the management of HS, and adalimumab was recently approved by Health Canada. It would be in our long-term interest to have a definition of severity to apply to all types of HS treatments. As such, the Board of the Canadian Hidradenitis Suppurativa Foundation (CHSF) discussed how to define severity in order to find a common consent to implement a unified guideline for the use of new emerging targeted therapies. Taken in aggregate after the meeting of the CHSF board in February 2016, we propose that these new criteria be used to define moderate to severe HS to provide a sensitive, useful tool that will help clinicians and patients make informed decisions in managing HS. This tool incorporates both objective measures of inflammation as well as qualitative measures of the patient’s quality of life. Moderate to severe HS includes 5 or more inflammatory lesions (nodules and abscesses)—if 1 lesion is located in the genitalia, then only 3 or more inflammatory lesions are required; an involvement of at least 2 anatomic sites; and either Dermatology Life Quality Index score of greater than or equal to 10 or visual analog pain scale score of greater than or equal to 50 mm. The improvement or response to treatment is evaluated based on more than 50% reduction in lesion count (Table 2). The definition of moderate to severe impact for a prototypical disease with multiple phenotypes requires validation. The second part of this project will focus on validation of this definition based on the data from clinical trials.