Hidradenitis Suppurativa: A Novel Model of Care and an Integrative Strategy to Adopt an Orphan Disease.

Unnecessary investigations, inappropriate treatment, worsening disease, and frustration for both patients and health care professionals are the hallmarks of hidradenitis suppurativa (HS) management. In light of a new treatment algorithm and biologic therapies made available to patients, an HS model of care is outlined in this article. The recommendations and management strategy presented here have been developed to help address the currently unmet needs of this patient population. The patient-centred model of care and disease management strategies were developed through the guidance and recommendations of HS medical experts in Newfoundland and Labrador. This article lays the foundation for the resources and steps required to change the status of this orphan disease and firmly embed patients with HS within a coordinated and integrative system of care.

Case report of a lupus patient with a severe flare and miliary tuberculosis: need for proper guidelines for management.

Systemic lupus erythematosus (SLE) is an autoimmune disease that is frequently treated with high doses of corticosteroids and other immunosuppressive drugs. Thus patients with SLE are at increased risk for infections with several pathogens including Mycobacterium tuberculosis. There are no established guidelines available for treatment of tuberculosis in SLE patients with high disease activity due to lack of relevant studies and management based more on physician expertise. We report a case of a young SLE patient with high disease activity index (SLEDAI19) as evidenced by the presence of a vasculitic rash, non-healing ulcer on forearm and proteinuria of >1 g/d along with miliary tuberculosis. She was treated with intravenous methylprednisolone pulse up to 3 g and antituberculous therapy, but the result was a fatal outcome. This case report emphasizes the need for formal guidelines for co-management of active tuberculosis and SLE with high disease activity.

Face and neck swelling in a 16-year-old boy.

Scleredema adultorum is a rare condition characterised by progressive collagen and mucin deposition in the skin. While the aetiology has not been clearly delineated, the condition is often associated with common infections. The current report describes a previously healthy 16-year-old boy who presented with 3 weeks of progressive neck swelling and skin induration. He had evidence of both active streptococcal and Ebstein-Barr virus (EBV) infections. Skin biopsy confirmed the diagnosis of scleredema. The patient was treated for his streptococcal infection, but otherwise managed conservatively. Clear improvement in the signs and symptoms was seen at a 3-month follow-up appointment. Scleredema can be a complication of streptococcal infection but to our knowledge has not been reported in association with EBV. It should be considered in the differential diagnosis of any patient presenting with cutaneous/subcutaneous induration and swelling of the face and/or neck.

Biologic therapy in psoriasis: perspectives on associated risks and patient management.

BACKGROUND: Previous publications have described practical considerations for initiating biologic therapy in psoriasis patients. However, most publications have focused on anti-tumor necrosis factor (TNF) therapy. OBJECTIVE: To create an evidence-based, practical tool that provides guidance on patient management for all biologics currently approved in Canada and the United States. METHODS: Psoriasis publications regarding safety issues in the initiation or monitoring of adalimumab, alefacept, etanercept, infliximab, or ustekinumab therapy were identified through a PubMed search. Phase III trials and open-label extensions (regardless of indication) and relevant guidelines from Health Canada were used to compile this review. RESULTS: Although these biologic agents have demonstrated efficacy in patients with psoriasis and are generally considered safe and well tolerated, rare but serious safety issues (ie, demyelination, infection, tuberculosis, malignancy, lymphoma, cardiovascular outcomes, hepatitis, pregnancy, surgery, and vaccination) have been observed. Attention to specific aspects of patient management (ie, prescreening requirements, symptoms to watch for, appropriate treatment, and referrals) is required to mitigate risk. CONCLUSION: Much of the evidence regarding the long-term safety of these agents has been based on experience in other patient populations. However, it does serve to guide us in understanding the risks that may impact the management of psoriasis patients.