The contents of these questions are taken from the Galderma Pre-Board Webinar. The Pre-Board Webinar is now an online course.
For details, go to https://www.galdermausa.com/Our-Commitment/PreBoard-Webinar.aspx.
1. Which one of the following is true of this disease?
a) May be caused by the topical application of silver sulfadiazine in a patient with a history of severe allergy to sulfa drugs
b) Skin lesions are asymptomatic
c) Usually associated with low risk of mortality
d) Prednisone is universally accepted as the treatment of choice
e) Mucous membranes are rarely involved
2. The mother of this 4-month-old child had clinical and serologic evidence of systemic lupus erythematosus at the time of the child’s birth. Which of the following is true of the child’s disease?
a) It is likely to be lifelong
b) The child may have atrioventricular conduction defects
c) Permanent organ damage does not occur
d) Nephritis is common
e) Scarring skin lesions are the most common cutaneous manifestations
of the disease
To learn the answers, go to page 2
{{pagebreak}}
BOARD REVIEW ANSWERS
1. Which one of the following is true of this disease?
a) May be caused by the topical application of silver sulfadiazine in a patient with a history of severe allergy to sulfa drugs
The clinical findings of toxic epidermal necrolysis (TEN) include rapid-onset of tender, red skin which is markedly Nikolsky sign-positive, resulting in mechanical fragility of skin, widespread blistering, and denudation. Full-thickness epidermal necrosis also occurs, resulting in wounds identical to those seen in second-degree thermal and electrical burns. Most patients experience severe involvement of numerous mucosal surfaces, to include the oral cavity, external eye, esophagus, upper airway, and lower genitourinary tract. Additionally, patients with greater than 25% surface area involvement have a very high risk of mortality, usually as a result of overwhelming secondary sepsis.
A number of drugs may cause TEN (most notably sulfa drugs and anticonvulsants), including topically administered drugs in patients with histories of preceding drug allergies. Treatment invariably involves inpatient care within a burn unit or an equivalent intensive care facility. Preliminary data suggest that higher dosage intravenous immunoglobulin or cyclophosphamide may be therapeutically beneficial. Although still controversial, some experts believe that high-dose prednisone may be equally beneficial, if given at or shortly after disease onset and if used for no more than 5 to 7 days.
References
Fine JD. Management of acquired bullous skin diseases. N Engl J Med. 1995;333(22):1475-1484. stein P, Revuz J. Toxic epidermal necrolysis. Dermatol Clin. 2000;18(3):485-495, ix.
Ringheanu M, Laude TA. Toxic epidermal necrolysis in children—an update. Clin Pediatr (Phila). 2000;39(12):687-694.
2. The mother of this 4-month-old child had clinical and serologic evidence of systemic lupus erythematosus at the time of the child’s birth. Which of the following is true of the child’s disease?
b) The child may have atrioventricular conduction defects
Neonatal lupus erythematosus usually manifests itself at or shortly after birth in infants born to women with obvious clinical evidence of active systemic lupus erythematosus. Skin lesions usually occur on the head, trunk, and upper extremities and are usually scaly, erythematous, telangiectatic, and hypopigmented. Atrophic discoid lupus erythematosus lesions seldom occur, but disfiguring telangiectasias may persist. Passively transferred antibodies to DNA, SSA, platelets, etc, have been described and evidence suggests that passive transfer of SSA (Ro-) antibodies is pathogenic in the expression of the disease. The lupus band test is often positive in these children and may contain anti-SSA antibodies. Atrioventricular heart block is common; however, severe renal and central nervous system disease activity are not. The disease is self-limited (<1 year) in most cases. Although self-limited, the disease may cause inflammatory injury to organs, resulting in fibrosis and chronic organ dysfunction.
References
Lee LA, Roberts CM, Frank MB, McCubbin VR, Reichlin M. The autoantibody response to Ro/SSA in cutaneous lupus erythematosus. Arch Dermatol. 1994;130(10):1262-1268.
Lee LA. Neonatal lupus erythematosus. J Invest Dermatol. 1993;100(1)(suppl):S9-S13.
Thornton CM, Eichenfield LF, Shinall EA, et al. Cutaneous telangiectases in neonatal lupus erythematosus. J Am Acad Dermatol. 1995;33(1):19-25.
Lee LA. Neonatal lupus: clinical features, therapy, and pathogenesis. Curr Rheumatol Rep. 2001;3(5):391-395.
Brucato A, Cimaz R, Stramba-Badiale M. Neonatal lupus. Clin Rev Allergy Immunol. 2002;23(3):279-299.
Jo-David Fine, MD, MPH, FRCP, is board certified in internal medicine, dermatology, and diagnostic and laboratory immunodermatology. Dr Fine is currently professor of medicine (dermatology) and pediatrics at Vanderbilt University School of Medicine in Nashville, TN.
Ron J. Feldman, MD, PhD, is assistant professor in the department of dermatology at Emory University School of Medicine in Atlanta, GA.
The contents of these questions are taken from the Galderma Pre-Board Webinar. The Pre-Board Webinar is now an online course.For details, go to https://www.galdermausa.com/Our-Commitment/PreBoard-Webinar.aspx.
1. Which one of the following is true of this disease?
a) May be caused by the topical application of silver sulfadiazine in a patient with a history of severe allergy to sulfa drugs
b) Skin lesions are asymptomatic
c) Usually associated with low risk of mortality
d) Prednisone is universally accepted as the treatment of choice
e) Mucous membranes are rarely involved
2. The mother of this 4-month-old child had clinical and serologic evidence of systemic lupus erythematosus at the time of the child’s birth. Which of the following is true of the child’s disease?
a) It is likely to be lifelong
b) The child may have atrioventricular conduction defects
c) Permanent organ damage does not occur
d) Nephritis is common
e) Scarring skin lesions are the most common cutaneous manifestations
of the disease
BOARD REVIEW ANSWERS
1. Which one of the following is true of this disease?
a) May be caused by the topical application of silver sulfadiazine in a patient with a history of severe allergy to sulfa drugs
The clinical findings of toxic epidermal necrolysis (TEN) include rapid-onset of tender, red skin which is markedly Nikolsky sign-positive, resulting in mechanical fragility of skin, widespread blistering, and denudation. Full-thickness epidermal necrosis also occurs, resulting in wounds identical to those seen in second-degree thermal and electrical burns. Most patients experience severe involvement of numerous mucosal surfaces, to include the oral cavity, external eye, esophagus, upper airway, and lower genitourinary tract. Additionally, patients with greater than 25% surface area involvement have a very high risk of mortality, usually as a result of overwhelming secondary sepsis.
A number of drugs may cause TEN (most notably sulfa drugs and anticonvulsants), including topically administered drugs in patients with histories of preceding drug allergies. Treatment invariably involves inpatient care within a burn unit or an equivalent intensive care facility. Preliminary data suggest that higher dosage intravenous immunoglobulin or cyclophosphamide may be therapeutically beneficial. Although still controversial, some experts believe that high-dose prednisone may be equally beneficial, if given at or shortly after disease onset and if used for no more than 5 to 7 days.
References
Fine JD. Management of acquired bullous skin diseases. N Engl J Med. 1995;333(22):1475-1484. stein P, Revuz J. Toxic epidermal necrolysis. Dermatol Clin. 2000;18(3):485-495, ix.
Ringheanu M, Laude TA. Toxic epidermal necrolysis in children—an update. Clin Pediatr (Phila). 2000;39(12):687-694.
2. The mother of this 4-month-old child had clinical and serologic evidence of systemic lupus erythematosus at the time of the child’s birth. Which of the following is true of the child’s disease?
b) The child may have atrioventricular conduction defects
Neonatal lupus erythematosus usually manifests itself at or shortly after birth in infants born to women with obvious clinical evidence of active systemic lupus erythematosus. Skin lesions usually occur on the head, trunk, and upper extremities and are usually scaly, erythematous, telangiectatic, and hypopigmented. Atrophic discoid lupus erythematosus lesions seldom occur, but disfiguring telangiectasias may persist. Passively transferred antibodies to DNA, SSA, platelets, etc, have been described and evidence suggests that passive transfer of SSA (Ro-) antibodies is pathogenic in the expression of the disease. The lupus band test is often positive in these children and may contain anti-SSA antibodies. Atrioventricular heart block is common; however, severe renal and central nervous system disease activity are not. The disease is self-limited (<1 year) in most cases. Although self-limited, the disease may cause inflammatory injury to organs, resulting in fibrosis and chronic organ dysfunction.
References
Lee LA, Roberts CM, Frank MB, McCubbin VR, Reichlin M. The autoantibody response to Ro/SSA in cutaneous lupus erythematosus. Arch Dermatol. 1994;130(10):1262-1268.
Lee LA. Neonatal lupus erythematosus. J Invest Dermatol. 1993;100(1)(suppl):S9-S13.
Thornton CM, Eichenfield LF, Shinall EA, et al. Cutaneous telangiectases in neonatal lupus erythematosus. J Am Acad Dermatol. 1995;33(1):19-25.
Lee LA. Neonatal lupus: clinical features, therapy, and pathogenesis. Curr Rheumatol Rep. 2001;3(5):391-395.
Brucato A, Cimaz R, Stramba-Badiale M. Neonatal lupus. Clin Rev Allergy Immunol. 2002;23(3):279-299.
Jo-David Fine, MD, MPH, FRCP, is board certified in internal medicine, dermatology, and diagnostic and laboratory immunodermatology. Dr Fine is currently professor of medicine (dermatology) and pediatrics at Vanderbilt University School of Medicine in Nashville, TN.
Ron J. Feldman, MD, PhD, is assistant professor in the department of dermatology at Emory University School of Medicine in Atlanta, GA.
The contents of these questions are taken from the Galderma Pre-Board Webinar. The Pre-Board Webinar is now an online course.
For details, go to https://www.galdermausa.com/Our-Commitment/PreBoard-Webinar.aspx.
1. Which one of the following is true of this disease?
a) May be caused by the topical application of silver sulfadiazine in a patient with a history of severe allergy to sulfa drugs
b) Skin lesions are asymptomatic
c) Usually associated with low risk of mortality
d) Prednisone is universally accepted as the treatment of choice
e) Mucous membranes are rarely involved
2. The mother of this 4-month-old child had clinical and serologic evidence of systemic lupus erythematosus at the time of the child’s birth. Which of the following is true of the child’s disease?
a) It is likely to be lifelong
b) The child may have atrioventricular conduction defects
c) Permanent organ damage does not occur
d) Nephritis is common
e) Scarring skin lesions are the most common cutaneous manifestations
of the disease
,
The contents of these questions are taken from the Galderma Pre-Board Webinar. The Pre-Board Webinar is now an online course.
For details, go to https://www.galdermausa.com/Our-Commitment/PreBoard-Webinar.aspx.
1. Which one of the following is true of this disease?
a) May be caused by the topical application of silver sulfadiazine in a patient with a history of severe allergy to sulfa drugs
b) Skin lesions are asymptomatic
c) Usually associated with low risk of mortality
d) Prednisone is universally accepted as the treatment of choice
e) Mucous membranes are rarely involved
2. The mother of this 4-month-old child had clinical and serologic evidence of systemic lupus erythematosus at the time of the child’s birth. Which of the following is true of the child’s disease?
a) It is likely to be lifelong
b) The child may have atrioventricular conduction defects
c) Permanent organ damage does not occur
d) Nephritis is common
e) Scarring skin lesions are the most common cutaneous manifestations
of the disease
To learn the answers, go to page 2
{{pagebreak}}
BOARD REVIEW ANSWERS
1. Which one of the following is true of this disease?
a) May be caused by the topical application of silver sulfadiazine in a patient with a history of severe allergy to sulfa drugs
The clinical findings of toxic epidermal necrolysis (TEN) include rapid-onset of tender, red skin which is markedly Nikolsky sign-positive, resulting in mechanical fragility of skin, widespread blistering, and denudation. Full-thickness epidermal necrosis also occurs, resulting in wounds identical to those seen in second-degree thermal and electrical burns. Most patients experience severe involvement of numerous mucosal surfaces, to include the oral cavity, external eye, esophagus, upper airway, and lower genitourinary tract. Additionally, patients with greater than 25% surface area involvement have a very high risk of mortality, usually as a result of overwhelming secondary sepsis.
A number of drugs may cause TEN (most notably sulfa drugs and anticonvulsants), including topically administered drugs in patients with histories of preceding drug allergies. Treatment invariably involves inpatient care within a burn unit or an equivalent intensive care facility. Preliminary data suggest that higher dosage intravenous immunoglobulin or cyclophosphamide may be therapeutically beneficial. Although still controversial, some experts believe that high-dose prednisone may be equally beneficial, if given at or shortly after disease onset and if used for no more than 5 to 7 days.
References
Fine JD. Management of acquired bullous skin diseases. N Engl J Med. 1995;333(22):1475-1484. stein P, Revuz J. Toxic epidermal necrolysis. Dermatol Clin. 2000;18(3):485-495, ix.
Ringheanu M, Laude TA. Toxic epidermal necrolysis in children—an update. Clin Pediatr (Phila). 2000;39(12):687-694.
2. The mother of this 4-month-old child had clinical and serologic evidence of systemic lupus erythematosus at the time of the child’s birth. Which of the following is true of the child’s disease?
b) The child may have atrioventricular conduction defects
Neonatal lupus erythematosus usually manifests itself at or shortly after birth in infants born to women with obvious clinical evidence of active systemic lupus erythematosus. Skin lesions usually occur on the head, trunk, and upper extremities and are usually scaly, erythematous, telangiectatic, and hypopigmented. Atrophic discoid lupus erythematosus lesions seldom occur, but disfiguring telangiectasias may persist. Passively transferred antibodies to DNA, SSA, platelets, etc, have been described and evidence suggests that passive transfer of SSA (Ro-) antibodies is pathogenic in the expression of the disease. The lupus band test is often positive in these children and may contain anti-SSA antibodies. Atrioventricular heart block is common; however, severe renal and central nervous system disease activity are not. The disease is self-limited (<1 year) in most cases. Although self-limited, the disease may cause inflammatory injury to organs, resulting in fibrosis and chronic organ dysfunction.
References
Lee LA, Roberts CM, Frank MB, McCubbin VR, Reichlin M. The autoantibody response to Ro/SSA in cutaneous lupus erythematosus. Arch Dermatol. 1994;130(10):1262-1268.
Lee LA. Neonatal lupus erythematosus. J Invest Dermatol. 1993;100(1)(suppl):S9-S13.
Thornton CM, Eichenfield LF, Shinall EA, et al. Cutaneous telangiectases in neonatal lupus erythematosus. J Am Acad Dermatol. 1995;33(1):19-25.
Lee LA. Neonatal lupus: clinical features, therapy, and pathogenesis. Curr Rheumatol Rep. 2001;3(5):391-395.
Brucato A, Cimaz R, Stramba-Badiale M. Neonatal lupus. Clin Rev Allergy Immunol. 2002;23(3):279-299.
Jo-David Fine, MD, MPH, FRCP, is board certified in internal medicine, dermatology, and diagnostic and laboratory immunodermatology. Dr Fine is currently professor of medicine (dermatology) and pediatrics at Vanderbilt University School of Medicine in Nashville, TN.
Ron J. Feldman, MD, PhD, is assistant professor in the department of dermatology at Emory University School of Medicine in Atlanta, GA.
The contents of these questions are taken from the Galderma Pre-Board Webinar. The Pre-Board Webinar is now an online course.
For details, go to https://www.galdermausa.com/Our-Commitment/PreBoard-Webinar.aspx.
1. Which one of the following is true of this disease?
a) May be caused by the topical application of silver sulfadiazine in a patient with a history of severe allergy to sulfa drugs
b) Skin lesions are asymptomatic
c) Usually associated with low risk of mortality
d) Prednisone is universally accepted as the treatment of choice
e) Mucous membranes are rarely involved
2. The mother of this 4-month-old child had clinical and serologic evidence of systemic lupus erythematosus at the time of the child’s birth. Which of the following is true of the child’s disease?
a) It is likely to be lifelong
b) The child may have atrioventricular conduction defects
c) Permanent organ damage does not occur
d) Nephritis is common
e) Scarring skin lesions are the most common cutaneous manifestations
of the disease
BOARD REVIEW ANSWERS
1. Which one of the following is true of this disease?
a) May be caused by the topical application of silver sulfadiazine in a patient with a history of severe allergy to sulfa drugs
The clinical findings of toxic epidermal necrolysis (TEN) include rapid-onset of tender, red skin which is markedly Nikolsky sign-positive, resulting in mechanical fragility of skin, widespread blistering, and denudation. Full-thickness epidermal necrosis also occurs, resulting in wounds identical to those seen in second-degree thermal and electrical burns. Most patients experience severe involvement of numerous mucosal surfaces, to include the oral cavity, external eye, esophagus, upper airway, and lower genitourinary tract. Additionally, patients with greater than 25% surface area involvement have a very high risk of mortality, usually as a result of overwhelming secondary sepsis.
A number of drugs may cause TEN (most notably sulfa drugs and anticonvulsants), including topically administered drugs in patients with histories of preceding drug allergies. Treatment invariably involves inpatient care within a burn unit or an equivalent intensive care facility. Preliminary data suggest that higher dosage intravenous immunoglobulin or cyclophosphamide may be therapeutically beneficial. Although still controversial, some experts believe that high-dose prednisone may be equally beneficial, if given at or shortly after disease onset and if used for no more than 5 to 7 days.
References
Fine JD. Management of acquired bullous skin diseases. N Engl J Med. 1995;333(22):1475-1484. stein P, Revuz J. Toxic epidermal necrolysis. Dermatol Clin. 2000;18(3):485-495, ix.
Ringheanu M, Laude TA. Toxic epidermal necrolysis in children—an update. Clin Pediatr (Phila). 2000;39(12):687-694.
2. The mother of this 4-month-old child had clinical and serologic evidence of systemic lupus erythematosus at the time of the child’s birth. Which of the following is true of the child’s disease?
b) The child may have atrioventricular conduction defects
Neonatal lupus erythematosus usually manifests itself at or shortly after birth in infants born to women with obvious clinical evidence of active systemic lupus erythematosus. Skin lesions usually occur on the head, trunk, and upper extremities and are usually scaly, erythematous, telangiectatic, and hypopigmented. Atrophic discoid lupus erythematosus lesions seldom occur, but disfiguring telangiectasias may persist. Passively transferred antibodies to DNA, SSA, platelets, etc, have been described and evidence suggests that passive transfer of SSA (Ro-) antibodies is pathogenic in the expression of the disease. The lupus band test is often positive in these children and may contain anti-SSA antibodies. Atrioventricular heart block is common; however, severe renal and central nervous system disease activity are not. The disease is self-limited (<1 year) in most cases. Although self-limited, the disease may cause inflammatory injury to organs, resulting in fibrosis and chronic organ dysfunction.
References
Lee LA, Roberts CM, Frank MB, McCubbin VR, Reichlin M. The autoantibody response to Ro/SSA in cutaneous lupus erythematosus. Arch Dermatol. 1994;130(10):1262-1268.
Lee LA. Neonatal lupus erythematosus. J Invest Dermatol. 1993;100(1)(suppl):S9-S13.
Thornton CM, Eichenfield LF, Shinall EA, et al. Cutaneous telangiectases in neonatal lupus erythematosus. J Am Acad Dermatol. 1995;33(1):19-25.
Lee LA. Neonatal lupus: clinical features, therapy, and pathogenesis. Curr Rheumatol Rep. 2001;3(5):391-395.
Brucato A, Cimaz R, Stramba-Badiale M. Neonatal lupus. Clin Rev Allergy Immunol. 2002;23(3):279-299.
Jo-David Fine, MD, MPH, FRCP, is board certified in internal medicine, dermatology, and diagnostic and laboratory immunodermatology. Dr Fine is currently professor of medicine (dermatology) and pediatrics at Vanderbilt University School of Medicine in Nashville, TN.
Ron J. Feldman, MD, PhD, is assistant professor in the department of dermatology at Emory University School of Medicine in Atlanta, GA.
BOARD REVIEW ANSWERS
1. Which one of the following is true of this disease?
a) May be caused by the topical application of silver sulfadiazine in a patient with a history of severe allergy to sulfa drugs
The clinical findings of toxic epidermal necrolysis (TEN) include rapid-onset of tender, red skin which is markedly Nikolsky sign-positive, resulting in mechanical fragility of skin, widespread blistering, and denudation. Full-thickness epidermal necrosis also occurs, resulting in wounds identical to those seen in second-degree thermal and electrical burns. Most patients experience severe involvement of numerous mucosal surfaces, to include the oral cavity, external eye, esophagus, upper airway, and lower genitourinary tract. Additionally, patients with greater than 25% surface area involvement have a very high risk of mortality, usually as a result of overwhelming secondary sepsis.
A number of drugs may cause TEN (most notably sulfa drugs and anticonvulsants), including topically administered drugs in patients with histories of preceding drug allergies. Treatment invariably involves inpatient care within a burn unit or an equivalent intensive care facility. Preliminary data suggest that higher dosage intravenous immunoglobulin or cyclophosphamide may be therapeutically beneficial. Although still controversial, some experts believe that high-dose prednisone may be equally beneficial, if given at or shortly after disease onset and if used for no more than 5 to 7 days.
References
Fine JD. Management of acquired bullous skin diseases. N Engl J Med. 1995;333(22):1475-1484. stein P, Revuz J. Toxic epidermal necrolysis. Dermatol Clin. 2000;18(3):485-495, ix.
Ringheanu M, Laude TA. Toxic epidermal necrolysis in children—an update. Clin Pediatr (Phila). 2000;39(12):687-694.
2. The mother of this 4-month-old child had clinical and serologic evidence of systemic lupus erythematosus at the time of the child’s birth. Which of the following is true of the child’s disease?
b) The child may have atrioventricular conduction defects
Neonatal lupus erythematosus usually manifests itself at or shortly after birth in infants born to women with obvious clinical evidence of active systemic lupus erythematosus. Skin lesions usually occur on the head, trunk, and upper extremities and are usually scaly, erythematous, telangiectatic, and hypopigmented. Atrophic discoid lupus erythematosus lesions seldom occur, but disfiguring telangiectasias may persist. Passively transferred antibodies to DNA, SSA, platelets, etc, have been described and evidence suggests that passive transfer of SSA (Ro-) antibodies is pathogenic in the expression of the disease. The lupus band test is often positive in these children and may contain anti-SSA antibodies. Atrioventricular heart block is common; however, severe renal and central nervous system disease activity are not. The disease is self-limited (<1 year) in most cases. Although self-limited, the disease may cause inflammatory injury to organs, resulting in fibrosis and chronic organ dysfunction.
References
Lee LA, Roberts CM, Frank MB, McCubbin VR, Reichlin M. The autoantibody response to Ro/SSA in cutaneous lupus erythematosus. Arch Dermatol. 1994;130(10):1262-1268.
Lee LA. Neonatal lupus erythematosus. J Invest Dermatol. 1993;100(1)(suppl):S9-S13.
Thornton CM, Eichenfield LF, Shinall EA, et al. Cutaneous telangiectases in neonatal lupus erythematosus. J Am Acad Dermatol. 1995;33(1):19-25.
Lee LA. Neonatal lupus: clinical features, therapy, and pathogenesis. Curr Rheumatol Rep. 2001;3(5):391-395.
Brucato A, Cimaz R, Stramba-Badiale M. Neonatal lupus. Clin Rev Allergy Immunol. 2002;23(3):279-299.
Jo-David Fine, MD, MPH, FRCP, is board certified in internal medicine, dermatology, and diagnostic and laboratory immunodermatology. Dr Fine is currently professor of medicine (dermatology) and pediatrics at Vanderbilt University School of Medicine in Nashville, TN.
Ron J. Feldman, MD, PhD, is assistant professor in the department of dermatology at Emory University School of Medicine in Atlanta, GA.
