- Email: [email protected]
Benign cutaneous Degos disease
P8136
P8069
Benefiterisk trade-off preferences for severe chronic hand eczema treatments A. Brett Hauber, RTI Heath Solutions, Research Triangle Park, NC, United States; Ateesha F. Mohamed, RTI Health Solutions, Research Triangle Park, NC, United States; Juan Marcos Gonzalez, RTI Health Soutions, Research Triangle Park, NC, United States; Ole Graff, Stiefel, a GSK Company, Research Triangle Park, NC, United States; Susan C. Zelt, Stiefel, a GSK Company, Research Triangle Park, NC, United States Background: Hand eczema affects 10% to 15% of the US population. Long-term prognosis is poor, and 5% to 7% of patients experience severe chronic hand eczema (CHE) that interferes with daily activities. However, treatments for CHE may be associated with adverse events (AEs) or may be ineffective, which may dissuade patients from pursuing or continuing treatment.
Bevacizumab-induced pityriasis rubra pilariselike eruption Wesley Fletcher, MD, Scott & White Dermatology, Temple, TX, United States; Katherine Fiala, MD, Scott & White Dermatology, Temple, TX, United States; Shannon Brown, Texas A&M Health Science Center College of Medicine, Bryan, TX, United States
Objectives: To quantify patient benefit-risk trade-off preferences for the outcomes associated with CHE treatments, specifically between efficacy and AEs. Methods: Adult patients in the US with self-reported physician diagnosis of CHE and severe symptoms not resolved with topical agents that limited normal daily activities completed a Web-enabled, discrete-choice survey. Respondents were recruited from 2 sources: Harris Interactive’s (HI’s) online chronic illness panel and the National Eczema Association’s (NEA’s) member panel. Each respondent answered a series of 10 treatment-choice questions. Each question required evaluating a pair of hypothetical severe CHE medication profiles defined by efficacy (severity of CHE after treatment), severe headaches during the first month of taking the medication, risk of permanent vision or hearing problems, risk of permanent bone problems, and risk of suicidal ideation. Random-parameter logit models were used to explain respondents’ choices regarding medication characteristics and estimate the relative contribution of each treatment outcome to choice, also known as preference weights; these were used to determine the mean maximum acceptable risk (MAR) of each AE for improvements in efficacy. Results: Final samples consisted of 399 respondents from the HI sample and 200 respondents from the NEA sample. Statistical tests indicated that the 2 samples could not be pooled due to differences in variance (P \ .05). Improvement in clearing from 25% to 50% was rated as 1.5 times as important as eliminating a 5% risk of permanent bone problems in the HI sample and 3.1 times as important in the NEA sample. The mean MAR for permanent vision problems in exchange for an improvement in clearing from 25% to 50% was 3.4% (95% CI, 2.1%-4.6%) for the HI sample and 4.8% (95% CI, 2.3%-7.0%) for the NEA sample. Conclusions: Efficacy improvements associated with treatment of severe CHE were rated as more important than elimination of risks of specific AEs.
Pityriasis rubra pilaris (PRP) is a rare inflammatory disorder characterized by follicular papules on an erythematous base often exhibiting islands of unaffected skin, follicular plugging, and palmoplantar hyperkeratosis. While vitamin A deficiency and autoimmune reactions have been hypothesized as possible etiologies of pityriasis rubra pilaris, PRP-like eruptions secondary to medications are extremely rare. To the authors’ knowledge, only 3 other cases have been reported. PRP has never been reported in association with bevacizumab. We present a 70-year-old male who developed erythroderma, which was both clinically and histologically consistent with PRP, 10 days after an intravitreal injection of bevacizumab for agerelated macular degeneration. Our patient’s PRP-like eruption responded dramatically to oral corticosteroids, which is rarely helpful for PRP. As immunomodulating drugs grow in their application in various diseases, recognition of associated medication complications is helpful in dermatologic practice. Commercial support: None identified.
Supported by Stiefel, a GSK Company.
P8688 Benign cutaneous Degos disease Kwei-Lan Liu, MD, Taichung Veterans General Hospital, Taichung, Taiwan; Chii-Shuenn Yang, MD, Taichung Veterans General Hospital, Taichung, Taiwan; Jui-Lung Shen, MD, PhD, Taichung Veterans General Hospital, Taichung, Taiwan; Yi-Ju Chen, MD, PhD, Taichung Veterans General Hospital, Taichung, Taiwan Degos disease, a rare occlusive vasculopathy, is characterized by pathognomonic skin eruption with subsequent systemic involvement and skin-limited disease. The skin lesions usually precede systemic manifestations with gastrointestinal tract and central nervous system being most commonly affected. Degos disease has been classified into 2 forms: classical and benign cutaneous with the latter being the less common form. We reported a case of a 41-year-old woman with a 2-year history of multiple asymptomatic, pea-sized, atrophic porcelain-white papules with an erythematous, telangiectatic rim on the trunk and limbs. She had experienced abdominal fullness and vomiting for 1 year. Histopathology of a skin biopsy specimen showed epidermal atrophy and wedge-shaped degeneration of collagen in which obliterative vessels were present. Alcian blue staining demonstrated abundant mucin deposition outlining the wedge-shaped area. Direct immunofluorescence was negative. The clinicopathologic features were compatible with Degos disease. Laboratory tests were all within normal limits, including complete blood cell count, hepatorenal function, urinalysis and immunologic fecal occult blood test, as well as profiles of lupus erythematosus, systemic sclerosis, rheumatoid arthritis, dermatomyositis, antiphospholipid syndrome, coagulopathy, and vasculopathy. Magnetic resonance images of the brain and upper gastrointestinal and small bowel series showed no abnormalities. Panendoscopy revealed merely reflux esophagitis. A diagnosis of benign cutaneous Degos disease was made. Under treatment with aspirin and dipyridamole, the cutaneous lesions healed with atrophic scars. No intestinal perforation, cerebrovascular accident, or other life-threatening complications attacked during the following 6 months. To date, fewer than 40 cases of benign cutaneous Degos disease with characteristic histopathology and at least 6 months of follow-up have been reported. There is no definite clinical, laboratory, or histopathologic indicator to predict the clinical course and to distinguish the classical and benign cutaneous variants at the time of presentation. The combination of aspirin and dipyridamole or each alone has shown variable effectiveness, while corticosteroids may worsen skin eruptions and complications. Patients with benign cutaneous Degos disease may have a fair prognosis with long-term survival, but they require regular follow-up because of the possibility of future systemic involvement.
Bier spots: A case report Thaıs Martins Tonso, MD, Hospital E Maternidade Celso Pierro - PUC Campinas, Campinas, Brazil; Adilson Costa, MD, PhD, Hospital E Maternidade Celso Pierro PUC Campinas, Campinas, Brazil; Aline Siqueira Talarico, MD, Hospital E Maternidade Celso Pierro - PUC Campinas, Campinas, Brazil; Elisangela Samartin Pegas Pereira, MD, Hospital E Maternidade Celso Pierro - PUC Campinas, Campinas, Brazil; Lissa Sabino Matos Matos, MD, Hospital E Maternidade Celso Pierro - PUC Campinas, Campinas, Brazil; Thaisa Saddi Tannous, MD, Hospital E Maternidade Celso Pierro - PUC Campinas, Campinas, Brazil The objective of this study is to report a case of Bier spots in a female, 14 year-old, of Chinese descent, with a 2-year history of asymptomatic hypopigmented macules on her lower limbs. These macules became more evident when the patient remained in orthostasis, and vanish with the elevation of the limbs, or when a pressure was applied on the lesion. It showed no change with exposition to thermal variation. The patient underwent a clinical and laboratory research which led us to the diagnosis of Bier spots. Bier spots are a benign vascular abnormality and its recognition is important because of the similarity that it has with other vascular phenomena. Its incidence is unknown, and believed to be a poorly recognized entity in clinical practice. Clinically, it manifests as hypochromic macules, small and irregular, surrounded by erythematous areas in members. These characteristics give the skin a mottled appearance. The lesions are induced by gravity-dependent positions: they can be elicited while the individual remains in orthostasis and disappear when the affected member is raised, or with a hold pressure at the erythematous area that surrounds it. Patients with Bier spots are usually asymptomatic; cases reported in the medical literature shows no abnormalities on blood tests or skin biopsies. It is believed that such abnormality is caused by a venous hyperreactivity, manifesting itself through vasoconstriction of venules and venous dilation reflex of surrounding areas. Are considered as trigger, to such a nonmodulated response, the venous stasis, associated with hypoxia or failure venoarteriolar reflex of the descendants dermal arterioles, in response to venous filling. Despite showing macroscopic appearance similar to anemic nevus, Bier spots differs completely in other aspects. Regarding the treatment of Bier spots, there is 1 report in the literature, and it was unsuccessful. Because of the asymptomatic character of this condition, associated with no systemic repercussions, it is concluded that the indication for treatment is the aesthetic discomfort that brings injury to the patient. The treatment can be tried, but should be individualized, and it is important to note that, so far, satisfactory results were not obtained.
Commercial support: None identified.
Commercial support: None identified.
P7536
AB38
J AM ACAD DERMATOL
MAY 2014
P8069
Benefiterisk trade-off preferences for severe chronic hand eczema treatments A. Brett Hauber, RTI Heath Solutions, Research Triangle Park, NC, United States; Ateesha F. Mohamed, RTI Health Solutions, Research Triangle Park, NC, United States; Juan Marcos Gonzalez, RTI Health Soutions, Research Triangle Park, NC, United States; Ole Graff, Stiefel, a GSK Company, Research Triangle Park, NC, United States; Susan C. Zelt, Stiefel, a GSK Company, Research Triangle Park, NC, United States Background: Hand eczema affects 10% to 15% of the US population. Long-term prognosis is poor, and 5% to 7% of patients experience severe chronic hand eczema (CHE) that interferes with daily activities. However, treatments for CHE may be associated with adverse events (AEs) or may be ineffective, which may dissuade patients from pursuing or continuing treatment.
Bevacizumab-induced pityriasis rubra pilariselike eruption Wesley Fletcher, MD, Scott & White Dermatology, Temple, TX, United States; Katherine Fiala, MD, Scott & White Dermatology, Temple, TX, United States; Shannon Brown, Texas A&M Health Science Center College of Medicine, Bryan, TX, United States
Objectives: To quantify patient benefit-risk trade-off preferences for the outcomes associated with CHE treatments, specifically between efficacy and AEs. Methods: Adult patients in the US with self-reported physician diagnosis of CHE and severe symptoms not resolved with topical agents that limited normal daily activities completed a Web-enabled, discrete-choice survey. Respondents were recruited from 2 sources: Harris Interactive’s (HI’s) online chronic illness panel and the National Eczema Association’s (NEA’s) member panel. Each respondent answered a series of 10 treatment-choice questions. Each question required evaluating a pair of hypothetical severe CHE medication profiles defined by efficacy (severity of CHE after treatment), severe headaches during the first month of taking the medication, risk of permanent vision or hearing problems, risk of permanent bone problems, and risk of suicidal ideation. Random-parameter logit models were used to explain respondents’ choices regarding medication characteristics and estimate the relative contribution of each treatment outcome to choice, also known as preference weights; these were used to determine the mean maximum acceptable risk (MAR) of each AE for improvements in efficacy. Results: Final samples consisted of 399 respondents from the HI sample and 200 respondents from the NEA sample. Statistical tests indicated that the 2 samples could not be pooled due to differences in variance (P \ .05). Improvement in clearing from 25% to 50% was rated as 1.5 times as important as eliminating a 5% risk of permanent bone problems in the HI sample and 3.1 times as important in the NEA sample. The mean MAR for permanent vision problems in exchange for an improvement in clearing from 25% to 50% was 3.4% (95% CI, 2.1%-4.6%) for the HI sample and 4.8% (95% CI, 2.3%-7.0%) for the NEA sample. Conclusions: Efficacy improvements associated with treatment of severe CHE were rated as more important than elimination of risks of specific AEs.
Pityriasis rubra pilaris (PRP) is a rare inflammatory disorder characterized by follicular papules on an erythematous base often exhibiting islands of unaffected skin, follicular plugging, and palmoplantar hyperkeratosis. While vitamin A deficiency and autoimmune reactions have been hypothesized as possible etiologies of pityriasis rubra pilaris, PRP-like eruptions secondary to medications are extremely rare. To the authors’ knowledge, only 3 other cases have been reported. PRP has never been reported in association with bevacizumab. We present a 70-year-old male who developed erythroderma, which was both clinically and histologically consistent with PRP, 10 days after an intravitreal injection of bevacizumab for agerelated macular degeneration. Our patient’s PRP-like eruption responded dramatically to oral corticosteroids, which is rarely helpful for PRP. As immunomodulating drugs grow in their application in various diseases, recognition of associated medication complications is helpful in dermatologic practice. Commercial support: None identified.
Supported by Stiefel, a GSK Company.
P8688 Benign cutaneous Degos disease Kwei-Lan Liu, MD, Taichung Veterans General Hospital, Taichung, Taiwan; Chii-Shuenn Yang, MD, Taichung Veterans General Hospital, Taichung, Taiwan; Jui-Lung Shen, MD, PhD, Taichung Veterans General Hospital, Taichung, Taiwan; Yi-Ju Chen, MD, PhD, Taichung Veterans General Hospital, Taichung, Taiwan Degos disease, a rare occlusive vasculopathy, is characterized by pathognomonic skin eruption with subsequent systemic involvement and skin-limited disease. The skin lesions usually precede systemic manifestations with gastrointestinal tract and central nervous system being most commonly affected. Degos disease has been classified into 2 forms: classical and benign cutaneous with the latter being the less common form. We reported a case of a 41-year-old woman with a 2-year history of multiple asymptomatic, pea-sized, atrophic porcelain-white papules with an erythematous, telangiectatic rim on the trunk and limbs. She had experienced abdominal fullness and vomiting for 1 year. Histopathology of a skin biopsy specimen showed epidermal atrophy and wedge-shaped degeneration of collagen in which obliterative vessels were present. Alcian blue staining demonstrated abundant mucin deposition outlining the wedge-shaped area. Direct immunofluorescence was negative. The clinicopathologic features were compatible with Degos disease. Laboratory tests were all within normal limits, including complete blood cell count, hepatorenal function, urinalysis and immunologic fecal occult blood test, as well as profiles of lupus erythematosus, systemic sclerosis, rheumatoid arthritis, dermatomyositis, antiphospholipid syndrome, coagulopathy, and vasculopathy. Magnetic resonance images of the brain and upper gastrointestinal and small bowel series showed no abnormalities. Panendoscopy revealed merely reflux esophagitis. A diagnosis of benign cutaneous Degos disease was made. Under treatment with aspirin and dipyridamole, the cutaneous lesions healed with atrophic scars. No intestinal perforation, cerebrovascular accident, or other life-threatening complications attacked during the following 6 months. To date, fewer than 40 cases of benign cutaneous Degos disease with characteristic histopathology and at least 6 months of follow-up have been reported. There is no definite clinical, laboratory, or histopathologic indicator to predict the clinical course and to distinguish the classical and benign cutaneous variants at the time of presentation. The combination of aspirin and dipyridamole or each alone has shown variable effectiveness, while corticosteroids may worsen skin eruptions and complications. Patients with benign cutaneous Degos disease may have a fair prognosis with long-term survival, but they require regular follow-up because of the possibility of future systemic involvement.
Bier spots: A case report Thaıs Martins Tonso, MD, Hospital E Maternidade Celso Pierro - PUC Campinas, Campinas, Brazil; Adilson Costa, MD, PhD, Hospital E Maternidade Celso Pierro PUC Campinas, Campinas, Brazil; Aline Siqueira Talarico, MD, Hospital E Maternidade Celso Pierro - PUC Campinas, Campinas, Brazil; Elisangela Samartin Pegas Pereira, MD, Hospital E Maternidade Celso Pierro - PUC Campinas, Campinas, Brazil; Lissa Sabino Matos Matos, MD, Hospital E Maternidade Celso Pierro - PUC Campinas, Campinas, Brazil; Thaisa Saddi Tannous, MD, Hospital E Maternidade Celso Pierro - PUC Campinas, Campinas, Brazil The objective of this study is to report a case of Bier spots in a female, 14 year-old, of Chinese descent, with a 2-year history of asymptomatic hypopigmented macules on her lower limbs. These macules became more evident when the patient remained in orthostasis, and vanish with the elevation of the limbs, or when a pressure was applied on the lesion. It showed no change with exposition to thermal variation. The patient underwent a clinical and laboratory research which led us to the diagnosis of Bier spots. Bier spots are a benign vascular abnormality and its recognition is important because of the similarity that it has with other vascular phenomena. Its incidence is unknown, and believed to be a poorly recognized entity in clinical practice. Clinically, it manifests as hypochromic macules, small and irregular, surrounded by erythematous areas in members. These characteristics give the skin a mottled appearance. The lesions are induced by gravity-dependent positions: they can be elicited while the individual remains in orthostasis and disappear when the affected member is raised, or with a hold pressure at the erythematous area that surrounds it. Patients with Bier spots are usually asymptomatic; cases reported in the medical literature shows no abnormalities on blood tests or skin biopsies. It is believed that such abnormality is caused by a venous hyperreactivity, manifesting itself through vasoconstriction of venules and venous dilation reflex of surrounding areas. Are considered as trigger, to such a nonmodulated response, the venous stasis, associated with hypoxia or failure venoarteriolar reflex of the descendants dermal arterioles, in response to venous filling. Despite showing macroscopic appearance similar to anemic nevus, Bier spots differs completely in other aspects. Regarding the treatment of Bier spots, there is 1 report in the literature, and it was unsuccessful. Because of the asymptomatic character of this condition, associated with no systemic repercussions, it is concluded that the indication for treatment is the aesthetic discomfort that brings injury to the patient. The treatment can be tried, but should be individualized, and it is important to note that, so far, satisfactory results were not obtained.
Commercial support: None identified.
Commercial support: None identified.
P7536
AB38
J AM ACAD DERMATOL
MAY 2014