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Challenging treatment of linear IgA dermatosis in a patient with dapsone-induced hypersensitivity syndrome
4386 Certolizumab pegol is associated with long-term improvements in extra-articular manifestations of psoriatic arthritis over 4years of treatment Oliver FitzGerald, Department of Rheumatology, St. Vincent’s University Hospital and Conway Institute; Roy Fleischmann, University of Texas SW Medical Center; Arthur Kavanaugh, Division of Rheumatology, Allergy & Immunology, UC San Diego; Bengt Hoepken, UCB Pharma; Luke Peterson, UCB Pharma; Dafna Gladman, Krembil Research Institute, Toronto Western Hospital Background: Previous reports have shown that patients (pts) with psoriatic arthritis (PsA) treated with certolizumab pegol (CZP) experience rapid improvements in extra-articular manifestations (EAMs), maintained over 96-weeks (wks) of treatment. Here we investigate the long-term effect of CZP treatment on EAMs over 4 years.
Conclusion: Analysis of these eight cases reveal an overall positive response to cetuximab. Only three patients experienced an acneiform rash as a side effect, which is minor compared to the toxicities of other chemotherapeutic drugs such as cisplatin. Given the success of cetuximab for this small sample of patients, we believe the field would greatly benefit from a multicenter prospective trial evaluating cetuximab use in high-risk cases of cSCC. Commercial support: None identified.
Methods: The RAPID-PsA trial (NCT01087788) was double-blind and placebocontrolled to Wk24, dose-blind to Wk48 and open-label (OL) to Wk216. Pts had active PsA and had failed $1 DMARD. Pts randomized to CZP (200mg Q2W or 400mg Q4W, following 400mg loading dose at Wks 0, 2, 4) continued their assigned dose in the OL period. We present EAM data for those pts randomized to CZP at Wk0 (combined 200mg Q2W and 400mg Q4W doses), with involvement of the respective EAM at baseline (BL). EAMs assessed included psoriasis (body surface area affected [BSA], BL involvement ¼ BL BSA $3%), nail psoriasis (modified nail psoriasis severity index [mNAPSI], BL involvement ¼ BL mNAPSI [ 0; for some pts the nail analyzed changed once or more following BL assessment), enthesitis (Leeds enthesitis index [LEI], BL involvement ¼ BL LEI [ 0) and dactylitis (Leeds dactylitis index [LDI], BL involvement ¼ $1 digit affected and with a difference in circumference $10%). Data are presented showing the proportion of pts with BL involvement of each EAM who achieve total resolution (ie, complete clearance) of the respective EAM on follow-up (a score of 0 for mNAPSI, LEI or LDI, or 0% BSA). Data shown are observed values. Results: 409 PsA pts were randomized, of whom 273 received CZP from Wk0. At baseline, 166 (60.8%) of these pts had psoriasis (mean BSA ¼ 24.2%), 197 (72.2%) nail psoriasis (mean mNAPSI ¼ 3.3), 172 (63.0%) enthesitis (mean LEI ¼ 3.0) and 73 (26.7%) dactylitis (mean LDI ¼ 51.3). A large proportion of CZP-randomized pts with baseline involvement achieved total resolution of the respective EAM by Wk24 (nail psoriasis: 38.5%, enthesitis: 65.2%, dactylitis: 73.8%, psoriasis: 26.8%). Mean scores in all EAMs assessed showed improvements by Wk12 (nail psoriasis: mean mNAPSI ¼ 2.1, enthesitis: mean LEI ¼ 1.2, dactylitis: mean LDI ¼ 13.6, psoriasis: mean BSA ¼ 10.0), with improvements maintained to Wk216 for those pts completing the study (nail psoriasis: mean mNAPSI ¼ 0.4, enthesitis: mean LEI ¼ 0.5, dactylitis: mean LDI ¼ 1.0, psoriasis: mean BSA ¼ 2.9%). Conclusion: Patients treated with CZP had improvements in all EAMs assessed, which were maintained over 4-years of the RAPID-PsA trial in those pts completing to Wk216. Commercial support: This study was sponsored by UCB Pharma. Editorial and Medical Writing support for the abstract was provided by Costello Medical Consulting, which was sponsored by UCB Pharma.
5048
5599 Cetuximab in high-risk cutaneous squamous cell carcinoma: A case series Cameron Trodello, Keck School of Medicine at the University of Southern California; Ashley Wysong, MD, Keck School of Medicine at the University of Southern California; Shauna Higgins, MD, Keck School of Medicine at the University of Southern California; Alexandre Ly, RN, Keck School of Medicine at the University of Southern California; Omeed Ahadiat, BS, Keck School of Medicine at the University of Southern California Purpose: Cutaneous squamous cell carcinoma (cSCC) is the second most common form of skin cancer, and has the potential for regional or distant metastasis. Most aggressive tumors that do spread are from a subset of cSCC with high-risk features, such as diameter greater than 2 cm, poorly differentiated histology, perineural invasion, and tumor invasion beyond subcutaneous fat. Despite the standardization of high-risk features associated with these tumors, there is no established consensus as to the proper management of high-risk cSCC. One systemic chemotherapeutic agent which has seen more recent use in these high-risk cases of cSCC is cetuximab; however, there is a paucity of data on its use in cSCC. We present a prospective series of eight cases of high-risk cSCC treated with cetuximab at our institution. The purpose is to evaluate the treatment strategies and effectiveness of cetuximab in hopes of learning more about how to optimally manage cases of advanced cSCC. Methods: Medical records were searched using CPT codes for cetuximab and cSCC. Demographic data and tumor characteristics were collected, as well as treatment regimens and follow-up times. A total of eight cases were examined. Results: Out of the eight patients, seven were male. Age ranged from 41 to 77 years, with a median of 68 years. Six tumors were located on the head and neck, one on the wrist, and one in multiple sites. Four patients were immunocompromised, and four tumors had a history of recurrence on presentation. Of the eight tumors, three were well-differentiated, three were moderately differentiated, one was spindle cell, and one was unspecified. Two demonstrated perineural invasion. Five of the tumors metastasized - three to the neck and two to lymph nodes. All eight patients were treated with cetuximab; surgery and radiation was given to seven patients. The median time of treatment to most recent follow up was 8.5 months. Three patients remain alive with disease and three patients alive without disease. Two patients died of other causes.
AB64
J AM ACAD DERMATOL
Challenging treatment of linear IgA dermatosis in a patient with dapsone-induced hypersensitivity syndrome Petra Cetkovska, MD, Department of Dermatovenereology, Faculty of Medicine, Charles University Hospital; Michaela Komorousova, MD, Department of Dermatovenereology, Faculty of Medicine, Charles University Hospital; Martina Kojanova, MD, Department of Dermatovenereology, 1st Faculty of Medicine and General Charles University Hospital; Iva Lomicova, MD, Department of Dermatovenereology, Faculty of Medicine, Charles University Hospital; Martin Novak, MD, Department of Dermatovenereology, Faculty of Medicine, Charles University Hospital; Jiri Stork, MD, Department of Dermatovenereology, 1st Faculty of Medicine and General Charles University Hospital We report a case of a 44-year-old woman, who was admitted to dermatovenereology department with a history of severe pruritic erythematous lesions for several months. Clinical picture consisted of erythematous and focally urticarial patches and plaques, with grouped vesicles in figurate arrangement resembling a cluster of jewels located on her scalp, trunk and extremities. Direct immunofluorescence confirmed diagnosis of linear IgA bullous dermatosis and the patient started treatment with oral dapsone in gradually increasing doses to 150 mg daily. Skin lesions healed promptly, nevertheless the patient developed dyspnea and acral cyanosis manifesting methemoglobinemia, on that account dose had to be lowered to 100 mg. Three weeks after dapsone introduction, the patient presented with malaise, high fever, morbilliform confluent rash, facial edema, cervical lymphadenopathy and painful oral mucosal lesions. Laboratory screening revealed elevated liver enzymes and serum bilirubin. Dapsone-induced hypersensitivity syndrome was highly suspected, therefore dapsone was stopped and the patient was treated by oral corticosteroids with slow tapering. Soon after dapsone withdrawal vesicular lesions on her extremities relapsed, consequently treatment with 1500 mg sulfasalazine was added into combination. To date, the patient is successfully treated with 1000 mg sulfasalazine along with low-dose prednisone, the blistering disorder resolved completely leaving milia and hyperpigmentation. The authors recommend sulfasalazine as beneficial treatment option in patients with linear IgA disease. Commercial support: None identified.
JUNE 2017
Conclusion: Analysis of these eight cases reveal an overall positive response to cetuximab. Only three patients experienced an acneiform rash as a side effect, which is minor compared to the toxicities of other chemotherapeutic drugs such as cisplatin. Given the success of cetuximab for this small sample of patients, we believe the field would greatly benefit from a multicenter prospective trial evaluating cetuximab use in high-risk cases of cSCC. Commercial support: None identified.
Methods: The RAPID-PsA trial (NCT01087788) was double-blind and placebocontrolled to Wk24, dose-blind to Wk48 and open-label (OL) to Wk216. Pts had active PsA and had failed $1 DMARD. Pts randomized to CZP (200mg Q2W or 400mg Q4W, following 400mg loading dose at Wks 0, 2, 4) continued their assigned dose in the OL period. We present EAM data for those pts randomized to CZP at Wk0 (combined 200mg Q2W and 400mg Q4W doses), with involvement of the respective EAM at baseline (BL). EAMs assessed included psoriasis (body surface area affected [BSA], BL involvement ¼ BL BSA $3%), nail psoriasis (modified nail psoriasis severity index [mNAPSI], BL involvement ¼ BL mNAPSI [ 0; for some pts the nail analyzed changed once or more following BL assessment), enthesitis (Leeds enthesitis index [LEI], BL involvement ¼ BL LEI [ 0) and dactylitis (Leeds dactylitis index [LDI], BL involvement ¼ $1 digit affected and with a difference in circumference $10%). Data are presented showing the proportion of pts with BL involvement of each EAM who achieve total resolution (ie, complete clearance) of the respective EAM on follow-up (a score of 0 for mNAPSI, LEI or LDI, or 0% BSA). Data shown are observed values. Results: 409 PsA pts were randomized, of whom 273 received CZP from Wk0. At baseline, 166 (60.8%) of these pts had psoriasis (mean BSA ¼ 24.2%), 197 (72.2%) nail psoriasis (mean mNAPSI ¼ 3.3), 172 (63.0%) enthesitis (mean LEI ¼ 3.0) and 73 (26.7%) dactylitis (mean LDI ¼ 51.3). A large proportion of CZP-randomized pts with baseline involvement achieved total resolution of the respective EAM by Wk24 (nail psoriasis: 38.5%, enthesitis: 65.2%, dactylitis: 73.8%, psoriasis: 26.8%). Mean scores in all EAMs assessed showed improvements by Wk12 (nail psoriasis: mean mNAPSI ¼ 2.1, enthesitis: mean LEI ¼ 1.2, dactylitis: mean LDI ¼ 13.6, psoriasis: mean BSA ¼ 10.0), with improvements maintained to Wk216 for those pts completing the study (nail psoriasis: mean mNAPSI ¼ 0.4, enthesitis: mean LEI ¼ 0.5, dactylitis: mean LDI ¼ 1.0, psoriasis: mean BSA ¼ 2.9%). Conclusion: Patients treated with CZP had improvements in all EAMs assessed, which were maintained over 4-years of the RAPID-PsA trial in those pts completing to Wk216. Commercial support: This study was sponsored by UCB Pharma. Editorial and Medical Writing support for the abstract was provided by Costello Medical Consulting, which was sponsored by UCB Pharma.
5048
5599 Cetuximab in high-risk cutaneous squamous cell carcinoma: A case series Cameron Trodello, Keck School of Medicine at the University of Southern California; Ashley Wysong, MD, Keck School of Medicine at the University of Southern California; Shauna Higgins, MD, Keck School of Medicine at the University of Southern California; Alexandre Ly, RN, Keck School of Medicine at the University of Southern California; Omeed Ahadiat, BS, Keck School of Medicine at the University of Southern California Purpose: Cutaneous squamous cell carcinoma (cSCC) is the second most common form of skin cancer, and has the potential for regional or distant metastasis. Most aggressive tumors that do spread are from a subset of cSCC with high-risk features, such as diameter greater than 2 cm, poorly differentiated histology, perineural invasion, and tumor invasion beyond subcutaneous fat. Despite the standardization of high-risk features associated with these tumors, there is no established consensus as to the proper management of high-risk cSCC. One systemic chemotherapeutic agent which has seen more recent use in these high-risk cases of cSCC is cetuximab; however, there is a paucity of data on its use in cSCC. We present a prospective series of eight cases of high-risk cSCC treated with cetuximab at our institution. The purpose is to evaluate the treatment strategies and effectiveness of cetuximab in hopes of learning more about how to optimally manage cases of advanced cSCC. Methods: Medical records were searched using CPT codes for cetuximab and cSCC. Demographic data and tumor characteristics were collected, as well as treatment regimens and follow-up times. A total of eight cases were examined. Results: Out of the eight patients, seven were male. Age ranged from 41 to 77 years, with a median of 68 years. Six tumors were located on the head and neck, one on the wrist, and one in multiple sites. Four patients were immunocompromised, and four tumors had a history of recurrence on presentation. Of the eight tumors, three were well-differentiated, three were moderately differentiated, one was spindle cell, and one was unspecified. Two demonstrated perineural invasion. Five of the tumors metastasized - three to the neck and two to lymph nodes. All eight patients were treated with cetuximab; surgery and radiation was given to seven patients. The median time of treatment to most recent follow up was 8.5 months. Three patients remain alive with disease and three patients alive without disease. Two patients died of other causes.
AB64
J AM ACAD DERMATOL
Challenging treatment of linear IgA dermatosis in a patient with dapsone-induced hypersensitivity syndrome Petra Cetkovska, MD, Department of Dermatovenereology, Faculty of Medicine, Charles University Hospital; Michaela Komorousova, MD, Department of Dermatovenereology, Faculty of Medicine, Charles University Hospital; Martina Kojanova, MD, Department of Dermatovenereology, 1st Faculty of Medicine and General Charles University Hospital; Iva Lomicova, MD, Department of Dermatovenereology, Faculty of Medicine, Charles University Hospital; Martin Novak, MD, Department of Dermatovenereology, Faculty of Medicine, Charles University Hospital; Jiri Stork, MD, Department of Dermatovenereology, 1st Faculty of Medicine and General Charles University Hospital We report a case of a 44-year-old woman, who was admitted to dermatovenereology department with a history of severe pruritic erythematous lesions for several months. Clinical picture consisted of erythematous and focally urticarial patches and plaques, with grouped vesicles in figurate arrangement resembling a cluster of jewels located on her scalp, trunk and extremities. Direct immunofluorescence confirmed diagnosis of linear IgA bullous dermatosis and the patient started treatment with oral dapsone in gradually increasing doses to 150 mg daily. Skin lesions healed promptly, nevertheless the patient developed dyspnea and acral cyanosis manifesting methemoglobinemia, on that account dose had to be lowered to 100 mg. Three weeks after dapsone introduction, the patient presented with malaise, high fever, morbilliform confluent rash, facial edema, cervical lymphadenopathy and painful oral mucosal lesions. Laboratory screening revealed elevated liver enzymes and serum bilirubin. Dapsone-induced hypersensitivity syndrome was highly suspected, therefore dapsone was stopped and the patient was treated by oral corticosteroids with slow tapering. Soon after dapsone withdrawal vesicular lesions on her extremities relapsed, consequently treatment with 1500 mg sulfasalazine was added into combination. To date, the patient is successfully treated with 1000 mg sulfasalazine along with low-dose prednisone, the blistering disorder resolved completely leaving milia and hyperpigmentation. The authors recommend sulfasalazine as beneficial treatment option in patients with linear IgA disease. Commercial support: None identified.
JUNE 2017