Search

Background Pyoderma gangrenosum (PG) is a rare neutrophilic dermatosis characterized by painful and ulcerating lesions on the skin. It rarely involves the face and is often difficult to diagnose. There are few cases reported in the literature of PG involving the face. Aim To share our experience with 5 patients in whom the final diagnosis was PG involving the face, and to review the literature. Methods We report a series of 5 patients with a final diagnosis of PG involving the face and reviewed relevant literature. We searched through PubMed and EMBASE using keywords such as “face” and “pyoderma gangrenosum,” “blastomycosis-like pyoderma gangrenosum, vegetative pyoderma gangrenosum and granulomatous pyoderma gangrenosum.” Results We report 5 patients (4 females) with pyoderma gangrenosum involving the face. All 5 had a final diagnosis of superficial granulomatous PG. All cases presented with nonhealing facial ulcer most commonly on cheeks and a common histopathology of mixed inflammatory infiltrates, multinucleated giant cells, and plasma cells with some granulomatous inflammation. Conclusions PG can involve the face, and all 5 of our patients had the superficial granulomatous as the most common form.

Background: Mycosis fungoides (MF) and Sezary syndrome (SS) are the most common subtypes of cutaneous T-cell lymphoma (CTCL). There is currently no cure for CTCL, and treatment is aimed at limiting disease progression. This study evaluated the efficacy and tolerability of alitretinoin in CTCL management. Methods: A retrospective, multicenter study was conducted on CTCL patients treated with alitretinoin as a primary agent or in combination with standard therapies. Results: Forty-eight patients with MF ( n = 40) and SS ( n = 8) with a median age of 59.7 years (±14.3) were eligible for study inclusion. Treatment response data were evaluated in 40 patients and safety in 42 patients. 40.0% of the patients had early-stage, 43.8% had advanced-stage CTCL, and in 16.7% of patients there was insufficient information for staging. 40.0% (16/40) of the patients achieved a complete or partial response, whereas 47.5% (19/40) achieved stable disease, 12.5% (5/40) had progressive disease, and there were no cases of disease relapses in responders. Both early and advanced stages of CTCL were responsive to alitretinoin as a primary or combined modality. Alitretinoin was well tolerated, and 64.3% (27/42) of patients did not report any side effects. The most commonly observed side effect was hypertriglyceridemia. Conclusions: This retrospective analysis supports the efficacy and safety of alitretinoin in clearing skin disease and preventing disease progression in CTCL as a monotherapy or in combination with standard therapies.

A man aged 51 years was referred to dermatology for hand dermatitis. The dorsal hands and fingers had superficial erosions with pale pink shallow scars and milia suggestive of porphyria cutanea tarda (PCT). Urine and fecal studies were typical of PCT. The patient had daily alcohol use and was found to have elevated serum ferritin, aspartate aminotransferase, and alanine transaminase. Genetic testing for common hemochromatosis genetic variants (HFE C282Y and H63D) was normal. He underwent next-generation sequencing analysis using the 16-gene hyperferritinemia gene panel for genes known to be associated with hereditary hyperferritinemia, iron overload, or both and was discovered to have a genetic variant in bone morphogenetic 6 (BMP6, c.287T> C, p.Leu96Pro). The skin lesions improved with phlebotomy therapy.

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.

Background:Hidradenitis suppurativa (HS) is a chronic, recurrent inflammatory disease of the pilosebaceous follicle that severely affects patients’ quality of life and can be challenging for clinicians to manage. A few case series have reported on the efficacy of acitretin monotherapy in the treatment of intractable HS.Objective:To assess the efficacy of acitretin for the treatment of intractable HS.Methods:A retrospective review was conducted involving all patients with HS who received acitretin between January 2011 and January 2015 in a tertiary academic medical center. All patients with HS who received acitretin, either as a monotherapy or as an adjuvant to other systemic medications, were included, and clinical response was assessed using the physician global scale.Results:Fourteen patients with HS (mean age 48 years [range 32-64 years]; 9 [64%] were men) received acitretin. Most patients (86%) had Hurley stage II or III disease. All patients had failed other standard systemic medications prior to initiating acitretin. Six patients (43%) received acitretin monotherapy, and 8 patients (57%) received acitretin as an adjuvant to other standard systemic medications. None of the patients who received acitretin monotherapy exhibited clinical improvements. Clinical improvements were observed in 7 of the 8 (87.5%) patients who received acitretin as an adjuvant to other systemic medications, with 3 patients (37.5%) exhibiting partial response and 4 patients (50%) exhibiting good response.Conclusions and Relevance:Acitretin monotherapy was ineffective for the treatment of intractable HS. Acitretin may be effective when used as an adjuvant to other systemic medications.

Background: Major advances have been made in the understanding of the pathophysiology of psoriasis. Objectives: The authors review the role of interleukin (IL) 17 in the pathogenesis of psoriasis and provide updates on approved and investigational therapies targeting IL-17 and the IL-17 receptor. Methods: A PubMed search was performed for relevant literature. Conclusion: The IL-23/Th17 signaling pathway (including IL-17) plays a central role in the pathogenesis of psoriasis. Biologic agents that block IL-17 (secukinumab and ixekizumab) or its receptor (brodalumab) are effective and safe for the treatment of psoriasis.

Background: Despite the complexity of psoriasis treatment using biologic therapy, there does not exist a standardized synoptic reporting form for the initiation of this population. The purpose of this study was to use a modified Delphi approach to develop a standard checklist for the standardized documentation of patients receiving systemic biologic therapy for psoriasis. Methods: A modified Delphi survey was conducted over 3 rounds (February 2017 through January 2018). An expert panel generated a 51-item checklist that was proposed to participants. Items were rated on an anchored 1-7 Likert scale. Consensus was defined apriori as ≥ 70% agreement by respondents. Results: A total of 58, 17, and 18 dermatologists participated in 3 consecutive Delphi rounds, respectively. Only half of the dermatologists surveyed reported using a checklist for the management of psoriasis. The final checklist comprised 19, 5, 6, and 9 items pertaining to patient history; physical exam and history of systemic therapy; vaccinations; and lab investigations and bloodwork, respectively. Conclusions: Given the increasing availability and complexity of biologic agents for psoriasis treatment, there is a need to promote standardized documentation for this population. The Checklist for the Systemic Treatment of Psoriasis presents 38 items that should be considered when initiating patients with psoriasis on biologic therapy.

Lymphomatoid papulosis (LyP) is a benign chronic often relapsing skin condition that belongs to the CD30-positive cutaneous lymphoproliferative disorders. LyP typically presents as crops of lesions with a tendency to self-resolve, and morphology can range from solitary to agminated or diffuse papules and plaques to nodules or tumours. The clinical-histological spectrum can range from borderline cases to overlap with primary cutaneous anaplastic cell lymphoma (pcALCL). Histology and immunophenotype commonly show overlap with other CD30-positive disorders and sometimes may be identical to pcALCL, making its diagnosis more difficult. Patients with LyP have an increased risk of developing a second neoplasm such as mycosis fungoides, pcALCL and/or Hodgkin lymphoma. Clinical correlation allows its proper classification and diagnosis, which is fundamental for treatment and prognosis. This review focuses on the clinical appearance, histopathological features, diagnosis, differential diagnosis and management of LyP.

Background: Atopic dermatitis is a common inflammatory condition of the skin. Moderate to severe cases not responding to topical treatments and lifestyle changes may need second-line therapy. Methotrexate has been suggested as an effective treatment in such cases. Objective: This study was done to determine the efficacy, adverse effects, and safety profile of methotrexate therapy in patients with atopic dermatitis. Materials/Methods: All adult patients with moderate to severe atopic dermatitis seen in the dermatology clinic at this tertiary hospital from January 2015 to December 2015 who were treated with methotrexate were reviewed in a retrospective chart review. Results: Forty-one patients (19 female, 22 male, mean age 45 years, range 19-90 years) were enrolled. Of these, 29% were naive to any systemic treatments in the past, including systemic corticosteroids. Methotrexate treatment resulted in excellent improvement (>75%) in 93% of patients, good (50%-75% improvement) in 5%, and partial (25%-50% improvement) in 2%. Median duration of therapy was 26 months, and 80% of patients were still on treatment at last review. Transient nonsignificant elevation of transaminases was the most common adverse effect noted in 20%, followed by nausea in 12% and fatigue in 7%. A fibroscan was done in 10 patients at cumulative doses ranging from 2 to 11 g methotrexate. No liver fibrosis was seen in these patients. Conclusion: Methotrexate is an effective treatment for moderate to severe atopic dermatitis with an acceptable safety profile. A low dose can be used to control the disease for prolonged periods without significant risk.

Background: Little is known about quality of life and work productivity in autoimmune bullous dermatoses (AIBDs). Objective: To determine the impact of AIBDs on quality of life and work productivity. Methods: An observational cross-sectional study took place between February and May 2013 at an AIBD tertiary referral centre. Ninety-four patients were included. All participants completed the Dermatology Life Quality Index and the Work Productivity and Activity Impairment–Specific Health Problem questionnaires. Results: Responders to treatment had less impairment ( P < .001) than nonresponders. Patients with severe AIBD had significantly more impairment that those with mild ( P < .001) and moderate ( P = .002) AIBD. Greater impairment was associated with higher percentage of work missed. Those with a higher Dermatology Life Quality Index score had greater work impairment and overall activity impairment ( P = .041, P = .024). Nonresponders had increased impairment while working ( P < .001), overall work impairment ( P < .001), and activity impairment ( P < .001). Severely affected patients had worse impairment in all Work Productivity and Activity Impairment Questionnaire domains. Conclusions: AIBD has the potential to be a large burden on ability to work and quality of life. Larger studies are needed to clarify how these domains change over time and whether or not they improve with treatment.

Background: Apremilast is a new oral drug for the treatment of moderate to severe plaque psoriasis that reduces inflammation by inhibiting phosphodiesterase 4. Its efficacy and safety data are limited; hence, real-world outcomes are important for elucidating the full spectrum of its adverse events (AEs) and expanding generalizability of clinical trial findings. Objective: Assess the efficacy and safety of apremilast monotherapy in real-world practice. Methods: A retrospective chart review was conducted in 2 academic dermatology practices. Efficacy was measured as the proportion of patients achieving a ≥75% reduction from baseline Psoriasis Area and Severity Index score (PASI-75) or a Psoriasis Global Assessment (PGA) score of 0 (clear) or 1 (almost clear) at 16 weeks. Safety was measured as the proportion of patients reporting ≥1 AE at 16 weeks. Results: Thirty-four patients were included. Efficacy: 19 patients (55.9%) achieved PASI-75 or PGA 0/1. Safety: 23 patients (67.6%) experienced ≥1 AEs. Five patients (14.7%) withdrew treatment prior to week 16 due to AEs. One patient withdrew treatment due to mood lability and depression. Common AEs included headache (32.4%), nausea (20.6%), diarrhoea (14.7%), weight loss (8.8%), and loose stool (8.8%). Conclusion: Apremilast monotherapy had higher efficacy with similar safety outcomes in the real world compared to clinical trials. There were higher proportions of reported headaches compared to clinical trials. This study supports the apremilast monotherapy clinical trial findings, suggesting that it has an acceptable safety profile and significantly reduces the severity of moderate to severe plaque psoriasis. Limitations include the retrospective nature of the study.

Background Rituximab (RTX) is increasingly used for the treatment of pemphigus and pemphigoid disorders. The high cost of RTX frequently limits its use and access. Objective To determine the health system resources and costs associated with RTX treatment of pemphigus and pemphigoid. Methods Health system resources and costs attributed to a convenience sample of 89 patients with either pemphigus or pemphigoid were identified, quantified, and valued 6 months prior to and following RTX initiation between May 2006 and August 2012. Overall cohort costs and costs per patient were calculated (2013 Can$). Results The overall cohort cost for 6 months pre-RTX was $3.8 million and for 6 months post-RTX was $2.6 million. The average cost per patient decreased from $42,231 to $29,423 (30.3% decrease). The main cost driver was intravenous immunoglobulin. Conclusions Our findings suggest that RTX is effective in reducing health system resources and the costs associated with the treatment of pemphigus and pemphigoid.

Background/Objectives The clinical and histopathologic findings of a rare simultaneous occurrence of papulonecrotic tuberculid and nodular tuberclid in a patient with active but asymptomatic pulmonary tuberculosis are presented. Papulonecrotic tuberculid was observed at a very early stage, presenting as molluscum-like lesions. This has been described once in the literature. This was observed in conjunction with lesions compatible with the rare clinicopathologic variant of nodular tuberculid. Critical to the diagnosis of active pulmonary tuberculosis was the use of induced sputum testing, which confirmed the diagnosis despite the lack of a cough and a chest x-ray negative for active tuberculosis. Methods/Results A 40-year-old male presented with a 2-week history of fever and a skin eruption consisting of molluscum-like papules on the ears, arms, and abdomen and nodules on his legs. Biopsies from both lesions were consistent with papulonecrotic and nodular tuberculid, respectively. Despite the lack of any respiratory symptoms, induced sputum grew Mycobacterium tuberculosis, and the lesions resolved on antituberculous therapy. Conclusions and Relevance Tuberculids are rare in Western countries but must be considered in the differential diagnosis of eruptions in patients from endemic countries. An active tuberculous focus must be sought out.

Introduction Epidermolysis bullosa pruriginosa (EBP) is a clinical variant of dystrophic epidermolysis bullosa (DEB), characterized by intense pruritus and hypertrophic, lichenified, prurigo-like papules, plaques, and nodules secondary to scratching. These clinical findings have been attributed to various mutations in the COL7A1 gene. Previous reports have yielded inconsistent findings regarding a possible genotype–phenotype relationship in EBP.

Epidermolysis bullosa (EB) pruriginosa is a rare clinical subtype of dystrophic epidermolysis bullosa (DEB) that is characterized by intense pruritus resulting in hypertrophic, lichenified, prurigo-like plaques and nodules secondary to scratching. The variability in the age of onset, rarity of intact bullae, histologic ambiguities, and close resemblance to other conditions such as acquired inflammatory dermatoses may make diagnosis difficult for this unusual condition, for which fewer than 100 cases have been documented. In this report, we describe 3 cases of EB pruriginosa and review the current literature.

Paradoxical development of psoriasis in patients on anti-TNF agents has been increasingly reported. The aim was to characterize the prevalence and clinical characteristics of anti-TNF-associated psoriasis in a large cohort of inflammatory bowel disease patients. Medical records of patients with Crohn’s disease or ulcerative colitis treated with anti-TNF therapy at a single, tertiary IBD center were identified between 2004 and 2016. Patients identified as having developed psoriasis while on anti-TNF underwent detailed retrospective review of dermatologic features and changes in IBD treatment prompted by the development of psoriasis. Among 676 patients treated with anti-TNF (infliximab or adalimumab), the incidence of psoriasis was 10.7% (N = 72). Female gender (OR 1.88 [95% CI 1.12–3.17], p = 0.017), stricturing or fistulizing Crohn’s disease (OR 1.83 [95% CI 1.04–3.21], p = 0.036) and upper GI Crohn’s disease (OR 3.03 [95% CI 1.06–8.33], p = 0.039) were associated with psoriasis development. The median time to psoriasis onset was 569 days from initiation of anti-TNF, with onset occurring earlier in patients who developed psoriasis on adalimumab versus infliximab (457 vs. 790.5 days, p = 0.008). Overall, in 15/72 (20.8%), cases, cessation of the anti-TNF was required as a result of psoriasis. Plaque psoriasis was the most common type of psoriatic lesion (75%). Topical corticosteroids were the most common treatment for psoriasis. We report a high incidence of anti-TNF-associated psoriasis that was associated with female gender, foregut disease location, and fistulizing and stricturing disease behavior. More prospective studies and genetic analyses evaluating possible pathophysiologic underpinnings of this problem are needed.

Background: Lymphomatoid papulosis is one of the primary cutaneous CD30+ T-cell lymphoproliferative disorders. Although considered a benign disease, lymphomatoid papulosis has been associated potentially with an increased risk of secondary hematolymphoid malignancies. Objective: The aim of this study was to assess the clinical characteristics and histologic subtypes of lymphomatoid papulosis, identify the prevalence and types of secondary hematolymphoid malignancies, and determine the potential risk factors for development of these hematolymphoid malignancies. Methods and Materials: A retrospective chart review was performed for all histologically confirmed cases of lymphomatoid papulosis between 1991 and 2016. Results: Seventy patients with lymphomatoid papulosis were identified. Thirty patients (43%) experienced a secondary hematolymphoid malignancy. Twenty-four (80%) of the hematolymphoid malignancies occurred after the onset of lymphomatoid papulosis. Older age at diagnosis of lymphomatoid papulosis, male sex, histology type B, and the presence of T-cell receptor gene rearrangement are associated with higher risk of developing hematolymphoid malignancy. Conclusion: Lymphomatoid papulosis is associated with increased risk of developing secondary hematolymphoid malignancies, particularly mycosis fungoides and cutaneous anaplastic large cell lymphoma.

Importance: Dermatologists are using an increasing range of immunomodulatory therapies to treat an expanding number of skin diseases. Complications of therapy are broad and include infection. Cytomegalovirus (CMV) retinitis has not been reported in association with dermatologic disease. Observations: We report two cases of CMV retinitis associated with immunosuppression for eczema and pemphigus vulgaris. In both cases, patients were receiving corticosteroid and a second-line immunosuppressive agent (cyclosporine or mycophenolate mofetil). Disease presented in both patients with painless visual loss. Conclusions and Relevance: Patients receiving immunosuppressive therapy should be monitored for blurred vision, floaters, or visual loss and referred for urgent assessment to ensure accurate diagnosis and prompt treatment of possible CMV retinitis.

Background Phototherapy is used frequently to treat early-stage mycosis fungoides (MF). The effectiveness of bath psoralen-ultraviolet A (bath PUVA) and narrowband ultraviolet B (nbUVB) in MF is well established. However, evidence is limited comparing the effectiveness of the 2 modalities for early-stage MF. The objective of the present study was to compare the responses between the study participants receiving bath PUVA versus nbUVB phototherapy. Patients and Methods The study included a retrospective cohort of stage 1A and 1B MF patients treated with bath PUVA or nbUVB at their initial presentation. The primary outcome was the response to treatment. The secondary outcome was disease-free survival. Results The cohort included 267 patients (158 treated with bath PUVA and 109 treated with nbUVB) with a mean age of 45.22 years. The mean follow-up period was 59.58 months for bath PUVA and 22.27 months for nbUVB. Overall, 88.61% of the bath PUVA group and 88.07% of the nbUVB group had complete responses ( P = .89). The median number of treatments to achieve a complete response was 55 for bath PUVA and 55.5 for nbUVB ( P = .63). The median disease-free survival was 43.25 months for bath PUVA and 14.9 months for nbUVB ( P Conclusion Bath PUVA and nbUVB are both effective treatments. The use of bath PUVA resulted in significantly greater disease-free survival.

Background: Internal medicine trainees receive limited teaching and training in dermatology and may feel inadequately prepared to assess and manage patients with dermatologic complaints. No study to date has assessed the needs of internal medicine trainees in Canada with regard to dermatology teaching. Objective: To determine internal medicine residents' comfort in assessing and managing dermatologic issues and their educational needs in dermatology. Methods: An electronic survey was conducted of first-, second-, and third-year internal medicine residents at the University of Toronto. Results: Fifty-four of 186 internal medicine trainees responded to our survey (response rate = 29%). Each respondent did not answer every question. Residents were generally uncomfortable or very uncomfortable assessing and managing dermatologic issues in the emergency department (40 of 47, 85%), ward or intensive care unit (39 of 47, 83%), and ambulatory clinic (40 of 47, 85%). Residents thought that various clinical and didactic dermatology exposures would be useful to their training as internists. Case-based teaching and ambulatory clinical rotations were felt to be particularly valuable. Additionally, 38 of 46 (83%) respondents wanted to learn how to perform punch biopsies. Conclusions: An effort should be made to increase the availability of relevant dermatology teaching and clinical exposures for internal medicine residents.

Background: Erosive oral lichen planus and desquamative gingivitis are uncommon but severe debilitating variants of oral lichen planus. Treatment of these presentations is difficult and challenging. Main observation: A 44-year-old woman was referred to the dermatology clinic with chronic painful lichen planus-related gingivitis and buccal erosions. She has failed multiple treatments including topical clobetasol and tacrolimus, intralesional corticosteroids and several systemic and immunosuppressive agents. Following completion of three months of treatment with oral apremilast at a dose of 30 mg twice daily, significant improvement was noted in her disease activity. Conclusion: Oral apremilast may be a safe and effective treatment for erosive oral lichen planus. ( J Dermatol Case Rep . 2016; 10(3): 56-57)

This study aimed to highlight the importance of routine screening for hyperglycemia and to develop a standardized, evidence-based approach for the management of pemphigus patients on prolonged systemic corticosteroid (CS) therapy. A cross-sectional study was conducted in two university-affiliated teaching hospitals using a referred sample of 200 patients with a confirmed diagnosis of pemphigus vulgaris, pemphigus foliaceus, or mucous membrane pemphigoid. All patients were receiving systemic CS therapy. A total of 150 patients responded to the survey. Six participants were excluded and 144 were included. The main outcome measure was blood glucose level to detect hyperglycemia. New-onset hyperglycemia was identified in 40% of patients who received CS therapy. None of the expected variables, including age, body mass index, family history of diabetes, corticosteroid dose, and duration of corticosteroid therapy, were independently associated with new-onset hyperglycemia. These findings indicate that the prevalence of CS-induced hyperglycemia in pemphigus patients is 40% and that in patients with pemphigus or MMP, CS therapy is associated with a markedly increased risk for hyperglycemia (odds ratio = 10.7, 95% confidence interval 1.38-83.50) compared with that of patients with the same diseases who do not receive CS therapy.

Background/Objectives: There are limited data on the clinical presentation and progression of pediatric cutaneous lymphoma. This study focuses on the clinical characteristics of pediatric patients with mycosis fungoides (MF). Materials and Methods: This descriptive study presents clinical characteristics of 22 pediatric patients with MF, enrolled in the international Childhood Registry for Cutaneous Lymphomas (CRCL). Results: The mean ages at onset and at diagnosis were 7.5 (SD 3.8 years) years and 9.9 (SD 3.4) years, respectively. The most common MF presentation was patch stage (68%), followed by hypopigmentation (59%) and plaque stage disease (50%). Epidermotropism and lymphocytic atypia were the most common pathologic features, found in 89% and 85%, respectively. Cerebriform nuclei were noted in 42%, and Pautrier microabscesses were seen in 16% of cases. A cytotoxic pattern was more commonly seen (67% vs 33%), and clonality was detected in 21% (3 of 14) of patients. All patients presented with early-stage disease and received skin-directed therapy (topical steroids, 73%; light therapy, 54%; or combination therapy, 35%). Conclusions: Pediatric patients with MF present in the first decade of life, with early-stage disease and unusual forms such as hypopigmented variant. Further patient enrolment will provide information regarding natural history, treatment response, and overall prognosis of pediatric cutaneous T-cell lymphoma (CTCL).

Background: Oral lichenoid lesions (OLLs) resemble oral lichen planus (OLP) but develop secondary to various underlying causes. The role of contact allergy in precipitating and/or perpetuating OLL is well documented but remains controversial. Objective: To help elucidate the association of contact allergy and OLL, we reviewed patch-test readings in patients diagnosed with OLP-like lesions. Methods: We retrospectively reviewed patients diagnosed with OLP-like lesions who had patch tests performed between January 1, 2006, and December 31, 2007. Results: Patch tests were performed on 24 patients with a histopathologic and/or clinical diagnosis of OLP. Of these, 16 (67%) had positive patch-test readings. At least eight (50%) of these patients had clinically relevant reactions. Ten of the 16 patients (63%) had reactions to metals. In most of these patients, troublesome areas tended to localize adjacent to metallic dental restorations. Of the nine patients (56%) who had reactions to fragrances, flavorings, gallates, and/or diallyl disulfide, the majority improved after avoiding these allergens. Conclusion: Our findings support the notion that contact allergy may underlie the pathogenesis of OLL and that allergen avoidance may result in amelioration of disease.

Background: Apremilast is an oral phosphodiesterase 4 inhibitor that has been approved as monotherapy for the treatment of moderate to severe chronic plaque psoriasis. No data exist on the safety or efficacy of apremilast as a component of combination therapy with either phototherapy or conventional systemic or biological therapies. Objective: To evaluate the short term-efficacy and safety of apremilast in combination with at least one form of photo-, systemic, or biologic therapy in the treatment of chronic plaque psoriasis. Methods: A retrospective chart review was conducted for patients who received apremilast in addition to systemic, biologic, or phototherapy. The primary outcome was the proportion of patients achieving at least 75% improvement in Psoriasis Area and Severity Index score (PASI-75). Results: A total of 81 patients with plaque psoriasis were treated with apremilast in combination with at least 1 other therapy (NB-UVB, methotrexate, acitretin, cyclosporin, etanercept, adalimumab, infliximab, or ustekinumab). Fourteen patients (17%) discontinued treatment before completion of 12 weeks of apremilast therapy. Sixty-seven patients continued on drug past 12 weeks. Of these patients, 81% achieved PASI-75 at week 12 after apremilast was added to an existing therapy. Nausea and/or diarrhea were reported in 25% of these patients, and weight loss was observed in 15%. Conclusion: Apremilast can be safely and effectively combined with phototherapy, systemic, and/or biological agents in patients with plaque psoriasis not responding adequately to these agents alone. Gastrointestinal side effects were manageable in the majority of patients.

Background: Cutaneous atypical mycobacterial infections have been increasingly described in association with cosmetic and alternative procedures. Objective: We report an outbreak of acupuncture-associated mycobacteriosis. Between April and December 2002, 32 patients developed cutaneous mycobacteriosis after visiting an acupuncture practice in Toronto, Canada. Results: Of 23 patients whose lesions were biopsied, 6 (26.1%) had culture-confirmed infection with Mycobacterium abscessus. These isolates were genetically indistinguishable by amplified fragment length polymorphism. The median incubation period was 1 month. Of 24 patients for whom clinical information was available, 23 (95.8%) had resolution of their infection. All patients developed residual scarring or hyperpigmentation. Conclusion: Nontuberculous mycobacteria should be recognized as an emerging, but preventable, cause of acupuncture-associated infections.

Background Pemphigus herpetiformis (PH) is a rare subtype of pemphigus that presents challenges in diagnosis. Objective To review the presentation, diagnosis, and management of PH. Methods We reviewed the charts of all patients diagnosed and treated for PH in an immunobullous referral center between September 2007 and June 2013. Results Eight patients were identified with a diagnosis of PH. All presented initially with pruritus. Clinical disease was manifest as either urticated erythematous plaques or a vesiculobullous eruption. Histological evaluation demonstrated eosinophilic spongiosis in all patients with acantholysis in half of cases (n = 4/8). Peripheral eosinophilia was noted in three of eight (37.5%) patients. In all cases, direct immunofluorescence showed intercellular deposition of immunoglobulin G in the epidermis. All patients required high-dose corticosteroid initially. All patients treated with dapsone or sulfasalazine (n = 4) achieved at least partial control. Other effective treatments included intravenous immunoglobulin (n = 2), azathioprine (n = 2), and leflunomide (n = 1). Rituximab was ineffective in two patients. Conclusion The clinical and histological features of PH develop over time and with treatment, making distinction between pemphigus subtypes challenging and delay in diagnosis common. Diagnosis of PH requires a high index of suspicion and is made on clinical grounds (urticated erythema) in the context of compatible histology and immunofluorescence findings. Treatment may be challenging, although efficacy of sulfonamide derivatives appears to offer a therapeutic effect.

SummaryBackground Pemphigus is a rare autoimmune blistering disease, reported to be associated with other coexisting and autoimmune diseases, including thyroid diseases, rheumatoid arthritis, alopecia areata, vitiligo, systemic lupus erythematosus, scleroderma and rare entities such as myasthenia gravis. Aim To identify and describe patients with pemphigus with a diagnosed comorbidity, and to quantify the risk of additional comorbidities. Methods This was a cross-sectional study of patients with pemphigus treated at a tertiary referral centre. Prevalence rates of 15 comorbid diseases were calculated. Age-standardized prevalence ratio (SPR) and 95% CI were calculated in comparison with prevalence rates in the general Canadian population using data from the Canadian Community Health Survey. Data were analysed using SAS software. Results In total, 295 patients were identified. An increased risk of hypothyroidism (n = 38, SPR = 1.53, 95% CI 1.08–2.10) and inflammatory bowel disease (IBD) (SPR = 1.48, 95% CI 0.40–3.80), and a two-fold increased risk of diabetes (SPR = 2.20, 95% CI 1.64–2.87) were observed. Conclusions Patients with pemphigus have a higher incidence of hypothyroidism, IBD and diabetes compared with the general population. As part of pemphigus investigations and surveillance, investigating for these conditions may be considered.

Background: Burning mouth syndrome (BMS) is a burning or sore mouth in the absence of changes in the oral mucosa. It is often difficult to diagnose and treat. Numerous theories of the etiology have been suggested, including contact allergy. Objective: To determine the clinical utility of patch testing in patients with BMS. Methods: We retrospectively reviewed the charts of patients diagnosed with BMS who had patch testing performed between January 1, 2008, and July 31, 2012. Results: Of 142 consecutive patients with BMS, 132 consented to patch testing; 89 (67%) had allergic patch test reactions. Of the patients with positive results, 66 (74%) had results that were deemed to have possible relevance. The most common allergens detected were nickel sulfate 2.5%, dodecyl gallate 0.3%, octyl gallate 0.3%, fragrance mix 8%, benzoyl peroxide 1%, and cinnamic alcohol 1%. Conclusions: Our findings suggest that contact allergy may be an etiologic factor in some patients with BMS. Patch testing is a useful investigation for BMS patients.

Background: Lymphomatoid papulosis is a rare CD30+ lymphoproliferative T-cell disorder with limited effective treatments. Objective: We describe the case of a 50-year-old woman diagnosed with lymphomatoid papulosis who was unable to access phototherapy and who failed to clear while on systemic treatment with methotrexate. Methods: The patient was initiated on mycophenolate mofetil (MMF), a prodrug of mycophenolic acid, at a dose of 2 g divided twice daily. Results: MMF produced a rapid response with complete clearing within 8 weeks, and the patient has been successfully maintained for 2 years at the same dose with no noted side effects. Other patients in our clinic have had similar success. Conclusions: Mycophenolic acid is a safe and well-tolerated therapy for lymphomatoid papulosis.

Background: Recent basic science research has revealed a decreased tissue expression of peroxisome proliferator-activated receptor (PPAR) γ in lichen planopilaris (LPP). Therefore, thiazolidinediones, being PPARγ agonists, could be used to treat LPP. Methods: We followed 24 patients with LPP who were treated with oral pioglitazone hydrochloride. Improvement in LPP was defined as a decrease in or disappearance of symptoms and perifollicular erythema in the context of halted spread of old patches. Results: Twenty of 24 patients were females. The average age was 52.5 years, and ages ranged from 22 to 70 years. Five of 24 patients have achieved remission; improvement was noted in half of the patients; there was no change in 3 patients; and 4 patients discontinued treatment due to side effects. Side effects were mild and included left calf pain, lightheadedness and nausea, dizziness, and hives. Conclusion: Use of thiazolidinediones might be a new promising venue of LPP treatment.

Background There is increasing evidence that a single cycle of rituximab (375 mg per square meter of body surface area once weekly for 4 weeks) is efficacious in patients with severe pemphigus. The approved protocol in rheumatoid arthritis is 1 g on days 1 and 15. We report herein on the efficacy and safety of this latter protocol for rituximab in 9 patients with pemphigus. Observations Nine patients with recalcitrant pemphigus were treated with prednisone, immunosuppressive agents, and/or intravenous immunoglobulin. Rituximab, 1 g, was infused on days 1 and 15. Each patient was observed for a minimum of 6 months. Reepithelialization of at least 50% of the affected areas occurred in all patients within 16 weeks. Three of 6 patients (50%) discontinued intravenous immunoglobulin therapy. A significant decrease in the pemphigus severity score and the mean dosage of prednisone was observed at 3 and 6 months. Relapses were observed in 4 patients between 5 and 13 months after rituximab treatment; these patients completed a second cycle of rituximab. There were no serious adverse effects observed during the follow-up period. Conclusions A single cycle of rituximab, 1 g on days 1 and 15, is an effective treatment for pemphigus. Further studies are needed to determine the efficacy and safety of repeated treatment courses in patients who experience recurrences.

Background: There are two rare variants of cutaneous T-cell lymphoma (CTCL) that primarily involve the subcutis. These include subcutaneous panniculitis-like T-cell lymphoma (SPTL) and cutaneous γ/δ T-cell lymphoma. Objective: This case report describes the clinical presentation, diagnosis, and treatment of a young man with probable SPTL. A review of recent literature outlining the differences between SPTL and cutaneous γ/δ T-cell lymphoma is discussed. Conclusion: The differential diagnosis in patients presenting with subcutaneous nodules and constitutional symptoms should include CTCL.

Importance Rituximab induces B-lymphocyte apoptosis by targeting CD20 antigen and has shown efficacy in antibody-mediated autoimmune disease. Rituximab is increasingly being acknowledged as an effective and safe treatment option for pemphigus. Objective To assess the clinical response of patients with pemphigus to rituximab using a modified fixed-dose rheumatoid arthritis protocol (1 g intravenously on days 1 and 15, followed by 500 mg intravenously if clinically warranted at 6-month intervals or repeated full dosing). Design, Setting, and Participants A retrospective cohort study was conducted using records from a tertiary referral center for autoimmune bullous disorders. Participants included 92 patients (pemphigus vulgaris, 84 [91%], and pemphigus foliaceus, 8 [9%]) who received rituximab treatment between May 1, 2006, and August 30, 2012. Main Outcomes and Measures The primary outcomes were time to relapse and achievement of a complete response with or without treatment at the end of the study. Results Median time to relapse after the first treatment cycle was 15 months (95% CI, 10.3-19.7). All patients experienced improvement. Complete remission rates with or without adjuvant treatment at final follow-up were 89% (56 patients [61%] were in complete remission without treatment and 26 patients [28%] were in complete remission during adjuvant treatment). No serious infectious adverse events occurred. Conclusions and Relevance The fixed-dose, modified rheumatoid arthritis protocol for rituximab was efficacious and well tolerated in patients with pemphigus. Patients who do not achieve remission after 1 cycle or patients who experience relapse benefit from further cycles of rituximab. Our results need to be confirmed in larger and controlled trials.

Use of rituximab in patients with chronic viral hepatitis can worsen pre-existing hepatitis or reactivate occult infection. There are no reports of use of rituximab in pemphigus patients with co-existing viral hepatitis. Herein, we report three pemphigus patients with co-existing chronic viral hepatitis (hepatitis C (n = 2), hepatitis B (n = 1)), who were treated successfully with rituximab under close supervision and concurrent antiviral drug administration. There was no derangement of the liver function tests or increase in viral load in any of the patients. By incorporating good collaboration with a hepatologist and close follow-up, such patients can be managed successfully with biologic therapies when the conventional treatment modalities have failed.