Toxic Epidermal Necrolysis Spectrum Management at Sunnybrook Health Sciences Centre: Our Multidisciplinary Approach After Review of the Current Evidence.

Toxic epidermal necrolysis spectrum (TENS) is a rare yet severe adverse drug reaction associated with a high mortality rate. Beyond supportive care, there is still no established therapy for TENS, although recent meta-analyses and UK guideline recommendations have attempted to offer a review of relevant literature on this difficult topic. As most directed treatments lack clear consensual evidence, care centres often resort to establishing their own strategies. As Canada's largest adult burn centre and the provincial reference centre for most burn patients in Ontario, our team at the Ross Tilley Burn Centre, in collaboration with the Department of Dermatology at Sunnybrook Health Sciences Centre, Toronto, Canada, has managed over 60 confirmed cases of TENS over the past 2 decades. We would like to share our management, experience, and present our treatment protocol that we recently established by a collaborative multidisciplinary team approach to help guide treatment of these complex patients not only in Canada but worldwide.

Multiple Pyoderma Gangrenosum Overlying AV Fistula Treated With Colchicine: A Case Report.

Rationale: Pyoderma gangrenosum (PG) is a rare neutrophilic dermatosis which gives rise to painful ulcers. Pyoderma gangrenosum can be triggered by trauma, a phenomenon called pathergy. Here, we report the first case of PG arising from pathergy due to needle insertion overlying an arteriovenous fistula (AVF). This case report seeks to inform nephrologists about PG, this yet unreported presentation, and management in the context of hemodialysis. Presenting concerns: A 69-year-old woman presented to dermatology clinic for erythemato-violaceous plaques with central ulceration at the site of needle insertion overlying her AVF. The patient was known for chronic renal insufficiency secondary to C3 glomerulonephritis, for which she received hemodialysis. After an accidental burn which lead to appearance of a painful ulcer, following each needle insertion for hemodialysis, she would develop an erythematous papule that progressed to a painful ulcer with erythematous-violaceous borders. Diagnosis: Pyoderma gangrenosum was clinically diagnosed and both clinical and paraclinical evaluation did not reveal any secondary cause of PG. Intervention: Dialysis via AVF was suspended due to the risk of triggering more PG and was temporarily pursued by central venous catheter. The patient was initially treated with prednisone and topical corticosteroids. Furthermore, owing to the high recurrence rate of PG, colchicine was initiated in prevention to avoid resorting to immunosuppressive or long-term corticotherapy. Outcomes: The patient's lesions improved on prednisone, which was then tapered over 1 month. Following prednisone taper and continuing improvement of PG on colchicine and topical corticosteroids alone, the decision was taken to recommence dialysis via AVF after performing a negative pathergy test. Topical corticosteroids were ceased due to the risk of cutaneous atrophy and were replaced by pimecrolimus ointment. The patient has continued dialysis via AVF ever since, without recurrence. Novel Finding: This is the first case reported of PG arising from pathergy due to needle insertion overlying an AVF. Colchicine may be a safe and effective therapy for long-term treatment of PG in the context of hemodialysis.

Contexte: Le Pyoderma gangrenosum (PG) est une dermatose neutrophilique rare qui provoque des ulcères douloureux. Le PG peut être déclenché par un traumatisme, un phénomène appelé pathergie. Nous rapportons ici le premier cas de PG résultant d'une pathergie au site d'insertion de l'aiguille recouvrant une fistule artérioveineuse. Ce rapport de cas vise à informer les néphrologues sur le PG, sur cette présentation non encore rapportée et sur sa prise en charge dans le contexte de l'hémodialyse. Présentation du cas: Une femme âgée de 69 ans s'étant présentée à une clinique de dermatologie pour soigner des plaques érythémateuses violacées avec ulcération centrale au site d'insertion de l'aiguille recouvrant sa fistule artérioveineuse (FAV). La patiente était connue pour une insuffisance rénale chronique secondaire à une glomérulonéphrite à C3, pour laquelle elle recevait des traitements d'hémodialyse. À la suite d'une brûlure accidentelle ayant entraîné l'apparition d'un ulcère douloureux, la patiente développait une papule érythémateuse qui évoluait vers un ulcère douloureux aux bords érythémateux violacés après chaque insertion d'aiguille pour l'hémodialyse. Diagnostic: Le Pyoderma gangrenosum a été diagnostiqué cliniquement et l'évaluation, tant clinique que paraclinique, n'a révélé aucune cause secondaire de PG. Intervention: En raison du risque accru de déclenchement de PG, la dialyse par la FAV a été suspendue et remplacée temporairement par la dialyse par cathéter veineux central. La patiente a d'abord reçu un traitement de prednisone et de corticostéroïdes topiques. En raison du taux élevé de récidive de PG, un traitement préventif à la colchicine a été initié pour éviter le recours à une corticothérapie immunosuppressive ou à long terme. Résultats: Les lésions de la patiente se sont améliorées avec le traitement à la prednisone, celui-ci a ensuite été réduit progressivement sur une période d'un mois. Après la réduction progressive de la prednisone et en raison de l'amélioration continue du PG avec le traitement par colchicine et corticostéroïdes topiques seulement, la décision a été prise de recommencer la dialyse par la FAV après un test de pathergie négatif. Un risque d'atrophie cutanée a justifié l'arrêt des corticostéroïdes topiques qui ont été remplacés par une pommade de pimecrolimus. Depuis, la patiente a poursuivi sa dialyse par FAV, sans récidive. Principales observations: Il s'agit du premier cas signalé de PG résultant d'une pathergie au site d'insertion de l'aiguille recouvrant une fistule artérioveineuse. La colchicine peut s'avérer un traitement sûr et efficace pour le traitement à long terme du PG dans le contexte de l'hémodialyse.

Ixazomib-induced Sweet's syndrome: A case report.

Ixazomib, a proteasome inhibitor commonly used for the treatment of multiple myeloma, is a rare cause of Sweet's syndrome. We present a 62-year-old man who developed drug-induced Sweet's syndrome during his fifth cycle of ixazomib for treatment of refractory multiple myeloma. Monthly rechallenge led to the recurrence of symptoms. The patient was successfully treated with addition of weekly corticosteroids and resumed his cancer treatment.

Morphea, Eosinophilic Fasciitis and Cancer: A Scoping Review.

Morphea is an autoimmune fibrotic skin disease. Eosinophilic fasciitis (EF) is considered to belong to the severe spectrum of morphea. We conducted a scoping review assessing the risk of secondary cancer among morphea/EF patients, paraneoplastic morphea/EF and morphea/EF developing secondary to cancer therapy. The search was conducted using MEDLINE, Embase, Cochrane databases for articles published from inception to September 2022 following the Preferred Reporting Items for Systematic reviews and Meta-Analyses for Scoping Reviews (PRISMA-ScR) guidelines with no language or date restrictions. Two hundred and one studies were included. Of these, 32 studies reported on secondary cancer in morphea/EF patients, 45 on paraneoplastic morphea/EF and 125 on cancer-treatment-induced morphea/EF. While the current evidence remains limited, data suggest an increased risk of secondary cutaneous and possibly pancreatic malignancy in morphea patients, particularly the generalized subtype. There were insufficient data for EF. On the other hand, paraneoplastic morphea was anecdotal, whereas several observational studies suggested that ~10% of EF cases may be paraneoplastic, primarily in the context of hematologic malignancies. Radiotherapy-induced morphea is rare, seen in ~0.2% of treated patients and is usually localized to the treatment site, except in patients with pre-existing autoimmunity. While chemotherapy-induced cases are reported, immunotherapy morphea/EF cases are emerging and are preferentially seen with PD-1 and not CTLA-4 inhibitors. This study is limited by the type of articles included (case reports, case series and observational studies), and hence, additional research on this important topic is needed.

Rare case of primary cutaneous adenoid cystic carcinoma of the abdomen: A case report.

Adenoid cystic carcinoma is predominantly a tumor of the parotid glands and can sometimes be found in other glands. In most cases, skin location is usually a metastatic presentation and rarely a primary tumor. We describe the case of a 59-year-old female patient presenting with a 5-mm skin-colored nodule on the abdomen histologically compatible with a primary cutaneous adenoid cystic carcinoma. Extensive workup revealed no other primary source, nor evidence of metastatic disease; therefore, wide local excision was the preferred treatment given the low potential of recurrence. As this adnexal carcinoma is rare and its morphology non-specific clinically, we wanted to raise awareness of this entity and its management.

Annular bullous pemphigoid: A case report and review of literature.

Bullous pemphigoid is an autoimmune blistering disease that primarily affects the geriatric population. It often presents as urticarial erythematous plaques, which evolve into subepidermal blisters accompanied by pruritus. Although rare, clinical variants of bullous pemphigoid have been documented. We present a rare case of annular bullous pemphigoid in a 50-year-old male and offer a brief review of the literature. Only five other case reports, including three in adults, have described this unusual presentation, which can mimic other autoimmune blistering diseases, including linear IgA bullous dermatosis and pemphigus herpetiformis. Therefore, histopathology and immunologic studies were essential in properly diagnosing this patient. Our case supports that annular blistering lesions can be a clinical variant of bullous pemphigoid.

Verrucous plane xanthomas secondary to lipoprotein X dyslipidemia in the context of cholestatic fulminant hepatitis: A case report.

Cutaneous xanthomas are the result of dermal deposition of lipid, mostly caused by disorders of lipid metabolism. Less commonly, they occur in the setting of cholestatic liver disease, leading to accumulation of lipoprotein X, a rare form of dyslipidemia that does not respond well to conventional treatments. We describe an atypical presentation of sudden diffuse xanthomas secondary to lipoprotein X dyslipidemia in the context of cholestatic fulminant hepatitis caused by trimethoprim-sulfamethoxazole hypersensitivity. Histopathology was also atypical and showed an unusual verrucous appearance consisting of overlying epidermal hyperplasia with hyperkeratosis. Our patient had significant improvement, after normalization of her lipid panel under cholestyramine and 13 sessions of apheresis, with topical corticosteroids offering some relief. This rare case shows the importance of recognizing atypical presentations of xanthomas, particularly when they do not respond to conventional dyslipidemia treatments.

Influence of the phenotype on mycosis fungoides prognosis, a retrospective cohort study of 160 patients.

BACKGROUND: Mycosis fungoides (MF) typically has a CD4+ CD8- T-cell phenotype. Rare cases of CD4- CD8+ , CD4- CD8- , or CD4+ CD8+ immunophenotypes have been described. Little is known about the impact of MF immunophenotypes on disease behavior. METHODS: We conducted a retrospective cohort study to review all cases of MF from 2007 to 2017 from Sunnybrook Health Sciences Centre, Toronto, Canada. CD4+ CD8- (Group 1) was compared to the three less common subtypes (Group 2) with respect to stage at diagnosis, progression, and transformation. Potential confounding factors (demographic, clinical, and laboratory parameters) were assessed. RESULTS: A total of 160 patients with confirmed MF were analyzed, including 126 CD4+ CD8- MF (79%), 26 CD4- CD8+ MF (16%), six CD4+ CD8+ MF (4%), and two CD4- CD8- MF (1%). Both groups were similar with respect to demographics and laboratory parameters at the time of diagnosis. There was no difference between patients with late stage disease (10% vs. 9%) for groups 1 and 2, respectively (P = 0.901). There was no statistically significant difference either in 5-year progression (27.7% vs. 23.5%, P = 0.283) or transformation (16.2% vs. 17.3%, P = 0.350) estimates. We did find that atypical immunophenotypes presented with different clinical morphologies and were less likely to require systemic therapy. CONCLUSION: Our large cohort study indicates that atypical MF immunophenotypes do not seem to influence prognosis. Hypopigmented MF was more frequent in the CD4- CD8+ group while folliculotropic MF was exclusively seen in the CD4+ CD8- group. We believe that cases of CD8+ MF with aggressive behavior described in the literature represent misclassified primary cutaneous aggressive epidermotropic CD8+ T-cell lymphoma. The small number of patients included in the study is a limiting factor.

Eosinophils in the skin-a red herring masking lymphoma: a case series.

Eosinophilia, both peripheral and in cutaneous tissue, is not a typical finding in mycosis fungoides; in fact, when faced with a lymphoeosinophilic infiltrate, mycosis fungoides is often not part of initial differential considerations. However, eosinophilia has been described in certain subtypes of mycosis fungoides, namely, in folliculotropic mycosis fungoides. We describe three challenging cases of folliculotropic mycosis fungoides presenting with varied clinical morphologies and a dense lymphoeosinophilic infiltrate and/or severe hypereosinophilia that obscured the final diagnosis for years. Only after treatment of the eosinophilia were the underlying atypical lymphocytes more apparent on histology and a correct diagnosis made. Thus, when characteristic features of mycosis fungoides are subtle, eosinophils can act as a red herring in terms of clinico-pathologic correlation and may prevent early and accurate diagnosis of mycosis fungoides. We suggest that further studies are needed to evaluate whether treatments to reduce eosinophilia, once other causes have been excluded, may help clear the confounding reactive inflammatory infiltrate and facilitate the diagnosis of mycosis fungoides.