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Board Review

The Dermatologist’s Board Review - April 2017

April 2017

The contents of these questions are taken from the Galderma Pre-Board Webinar. The Pre-Board Webinar is now an online course. 

 

1. Autoantibodies in this disease react with:
a) Centromeres or nucleoli
b) SSA and SSB antigens
c) Single-stranded DNA
d) Sm and native DNA
e) Keratins

2. The lesion which is present near the canthus of this patient most often occurs in which of the following diseases?
a) Bullous pemphigoid
b) Cicatricial pemphigoid
c) Pemphigus vulgaris
d) Dermatitis herpetiformis
e) Epidermolysis bullosa acquisita

 

3. Which one of the following is true of this disease?
a) Cutaneous immune deposits containing autoantibodies to reticulin, gliadin, and endomysium are present within the dermal papillae
b) Cutaneous immune deposits may disappear if a strict gluten-free diet is used
c) Granular IgG immune deposits are present within the papillary tufts
d) This disease has the same targeted antigen as in linear IgA dermatosis
e) Prednisone is the treatment of choice

 

Find the answers on Page 2

1. Autoantibodies in this disease react with:
a) Centromeres or nucleoli

This patient has matted facial telangiectases, consistent with the diagnosis of CREST syndrome.

 

 

 

 

 

 

 

 

 

 

 

 

 

References
Sontheimer RD, Deng JS, Gilliam JN. Antinuclear and anticytoplasmic antibodies. Concepts and misconceptions. J Am Acad Dermatol. 1983;9(3):335-343.
Jarzabek-Chorzelska M, Blaszczyk M, Jablonska S, Chorzelski T, Kumar V, Beutner EH. Scl 70 antibody--a specific marker of systemic sclerosis. Br J Dermatol. 1986;115(4):393-401.
Sontheimer RD, McCauliffe DP, Zappi E, Targoff I. Antinuclear antibodies: clinical correlations and biologic significance. Adv Dermatol. 1992;7:3-52.
Mutasim DF, Adams BB. A practical guide for serologic evaluation of autoimmune connective tissue diseases. J Am Acad Dermatol. 2000;42(2 Pt 1):159-174.

----------

2. The lesion which is present near the canthus of this patient most often occurs in which of the following diseases?
b)  Cicatricial pemphigoid

The lesion depicted is a symblepharon—a fibrotic scar that connects the bulbar and tarsal conjunctival surfaces. It is a hallmark feature of cicatricial pemphigoid, occurring in at least 80% of patients at some point in their lives. Preceded by either frank vesiculation or subclinical disease activity, it typically causes no impairment in vision or movement of the eye. Rare patients with epidermolysis bullosa acquisita may present with clinical features most suggestive of cicatricial pemphigoid, to include blister formation within the oral cavity or along the external surfaces of the eyes.

References
Fitzpatrick TB, Eisen AZ, Wolff K, Freedberg IM, Austin KF, eds. Dermatology in General Medicine. 4th ed. New York, NY: McGraw-Hill Book Co; 1993:642-645.
Chan LS, Ahmed AR, Anhalt GJ, et al. The first international consensus on mucous membrane pemphigoid: definition, diagnostic criteria, pathogenic factors, medical treatment, and prognostic indicators. Arch Dermatol. 2002;138(3):370-379.
Egan CA, Yancey KB. The clinical and immunopathological manifestations of anti-epiligrin cicatricial pemphigoid, a recently defined subepithelial autoimmune blistering disease. Eur J Dermatol. 2000;10(8):585-589.
Fleming TE, Korman NJ. Cicatricial pemphigoid. J Am Acad Dermatol. 2000;43(4):571-591.
Nguyen QD, Foster CS. Cicatricial pemphigoid: diagnosis and treatment. Int Ophthalmol Clin. 1996;36(1):41-60.

----------

3. Which one of the following is true of this disease?
b)  Cutaneous immune deposits may disappear if a strict gluten-free diet is used

Dermatitis herpetiformis is an autoimmune disease characterized by pruritic vesicles arising in symmetrical array along the elbows, knees, buttocks, lumbosacral spine, and scalp. Granular deposits of IgA are present within the uppermost papillary dermis. Patients with dermatitis herpetiformis frequently have circulation autoantibodies within their sera against gliadin, reticulum, and smooth muscle endomysium, although cutaneous autoantibodies to these antigens have yet to be proven within lesional skin. Dermatitis herpetiformis does not share the same target for autoimmunity as occurs in linear IgA dermatosis. Recent data suggests that dermatitis herpetiformis patients may have autoimmunity to tissue transglutaminase as their underlying molecular basis.

References
Hall RP 3rd. Dermatitis herpetiformis. J Invest Dermatol. 1992;99(6):873-881.
Reunala TL. Dermatitis herpetiformis. Clin Dermatol. 2001;19(6):728-736.

 

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. 

 

1. Autoantibodies in this disease react with:
a) Centromeres or nucleoli
b) SSA and SSB antigens
c) Single-stranded DNA
d) Sm and native DNA
e) Keratins

2. The lesion which is present near the canthus of this patient most often occurs in which of the following diseases?
a) Bullous pemphigoid
b) Cicatricial pemphigoid
c) Pemphigus vulgaris
d) Dermatitis herpetiformis
e) Epidermolysis bullosa acquisita

 

3. Which one of the following is true of this disease?
a) Cutaneous immune deposits containing autoantibodies to reticulin, gliadin, and endomysium are present within the dermal papillae
b) Cutaneous immune deposits may disappear if a strict gluten-free diet is used
c) Granular IgG immune deposits are present within the papillary tufts
d) This disease has the same targeted antigen as in linear IgA dermatosis
e) Prednisone is the treatment of choice

 

 

1. Autoantibodies in this disease react with:
a) Centromeres or nucleoli

This patient has matted facial telangiectases, consistent with the diagnosis of CREST syndrome.

 

References
Sontheimer RD, Deng JS, Gilliam JN. Antinuclear and anticytoplasmic antibodies. Concepts and misconceptions. J Am Acad Dermatol. 1983;9(3):335-343.
Jarzabek-Chorzelska M, Blaszczyk M, Jablonska S, Chorzelski T, Kumar V, Beutner EH. Scl 70 antibody--a specific marker of systemic sclerosis. Br J Dermatol. 1986;115(4):393-401.
Sontheimer RD, McCauliffe DP, Zappi E, Targoff I. Antinuclear antibodies: clinical correlations and biologic significance. Adv Dermatol. 1992;7:3-52.
Mutasim DF, Adams BB. A practical guide for serologic evaluation of autoimmune connective tissue diseases. J Am Acad Dermatol. 2000;42(2 Pt 1):159-174.

----------

2. The lesion which is present near the canthus of this patient most often occurs in which of the following diseases?
b)  Cicatricial pemphigoid

The lesion depicted is a symblepharon—a fibrotic scar that connects the bulbar and tarsal conjunctival surfaces. It is a hallmark feature of cicatricial pemphigoid, occurring in at least 80% of patients at some point in their lives. Preceded by either frank vesiculation or subclinical disease activity, it typically causes no impairment in vision or movement of the eye. Rare patients with epidermolysis bullosa acquisita may present with clinical features most suggestive of cicatricial pemphigoid, to include blister formation within the oral cavity or along the external surfaces of the eyes.

References
Fitzpatrick TB, Eisen AZ, Wolff K, Freedberg IM, Austin KF, eds. Dermatology in General Medicine. 4th ed. New York, NY: McGraw-Hill Book Co; 1993:642-645.
Chan LS, Ahmed AR, Anhalt GJ, et al. The first international consensus on mucous membrane pemphigoid: definition, diagnostic criteria, pathogenic factors, medical treatment, and prognostic indicators. Arch Dermatol. 2002;138(3):370-379.
Egan CA, Yancey KB. The clinical and immunopathological manifestations of anti-epiligrin cicatricial pemphigoid, a recently defined subepithelial autoimmune blistering disease. Eur J Dermatol. 2000;10(8):585-589.
Fleming TE, Korman NJ. Cicatricial pemphigoid. J Am Acad Dermatol. 2000;43(4):571-591.
Nguyen QD, Foster CS. Cicatricial pemphigoid: diagnosis and treatment. Int Ophthalmol Clin. 1996;36(1):41-60.

----------

3. Which one of the following is true of this disease?
b)  Cutaneous immune deposits may disappear if a strict gluten-free diet is used

Dermatitis herpetiformis is an autoimmune disease characterized by pruritic vesicles arising in symmetrical array along the elbows, knees, buttocks, lumbosacral spine, and scalp. Granular deposits of IgA are present within the uppermost papillary dermis. Patients with dermatitis herpetiformis frequently have circulation autoantibodies within their sera against gliadin, reticulum, and smooth muscle endomysium, although cutaneous autoantibodies to these antigens have yet to be proven within lesional skin. Dermatitis herpetiformis does not share the same target for autoimmunity as occurs in linear IgA dermatosis. Recent data suggests that dermatitis herpetiformis patients may have autoimmunity to tissue transglutaminase as their underlying molecular basis.

References
Hall RP 3rd. Dermatitis herpetiformis. J Invest Dermatol. 1992;99(6):873-881.
Reunala TL. Dermatitis herpetiformis. Clin Dermatol. 2001;19(6):728-736.

 

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. 

 

1. Autoantibodies in this disease react with:
a) Centromeres or nucleoli
b) SSA and SSB antigens
c) Single-stranded DNA
d) Sm and native DNA
e) Keratins

2. The lesion which is present near the canthus of this patient most often occurs in which of the following diseases?
a) Bullous pemphigoid
b) Cicatricial pemphigoid
c) Pemphigus vulgaris
d) Dermatitis herpetiformis
e) Epidermolysis bullosa acquisita

 

3. Which one of the following is true of this disease?
a) Cutaneous immune deposits containing autoantibodies to reticulin, gliadin, and endomysium are present within the dermal papillae
b) Cutaneous immune deposits may disappear if a strict gluten-free diet is used
c) Granular IgG immune deposits are present within the papillary tufts
d) This disease has the same targeted antigen as in linear IgA dermatosis
e) Prednisone is the treatment of choice

 

,

The contents of these questions are taken from the Galderma Pre-Board Webinar. The Pre-Board Webinar is now an online course. 

 

1. Autoantibodies in this disease react with:
a) Centromeres or nucleoli
b) SSA and SSB antigens
c) Single-stranded DNA
d) Sm and native DNA
e) Keratins

2. The lesion which is present near the canthus of this patient most often occurs in which of the following diseases?
a) Bullous pemphigoid
b) Cicatricial pemphigoid
c) Pemphigus vulgaris
d) Dermatitis herpetiformis
e) Epidermolysis bullosa acquisita

 

3. Which one of the following is true of this disease?
a) Cutaneous immune deposits containing autoantibodies to reticulin, gliadin, and endomysium are present within the dermal papillae
b) Cutaneous immune deposits may disappear if a strict gluten-free diet is used
c) Granular IgG immune deposits are present within the papillary tufts
d) This disease has the same targeted antigen as in linear IgA dermatosis
e) Prednisone is the treatment of choice

 

Find the answers on Page 2

1. Autoantibodies in this disease react with:
a) Centromeres or nucleoli

This patient has matted facial telangiectases, consistent with the diagnosis of CREST syndrome.

 

 

 

 

 

 

 

 

 

 

 

 

 

References
Sontheimer RD, Deng JS, Gilliam JN. Antinuclear and anticytoplasmic antibodies. Concepts and misconceptions. J Am Acad Dermatol. 1983;9(3):335-343.
Jarzabek-Chorzelska M, Blaszczyk M, Jablonska S, Chorzelski T, Kumar V, Beutner EH. Scl 70 antibody--a specific marker of systemic sclerosis. Br J Dermatol. 1986;115(4):393-401.
Sontheimer RD, McCauliffe DP, Zappi E, Targoff I. Antinuclear antibodies: clinical correlations and biologic significance. Adv Dermatol. 1992;7:3-52.
Mutasim DF, Adams BB. A practical guide for serologic evaluation of autoimmune connective tissue diseases. J Am Acad Dermatol. 2000;42(2 Pt 1):159-174.

----------

2. The lesion which is present near the canthus of this patient most often occurs in which of the following diseases?
b)  Cicatricial pemphigoid

The lesion depicted is a symblepharon—a fibrotic scar that connects the bulbar and tarsal conjunctival surfaces. It is a hallmark feature of cicatricial pemphigoid, occurring in at least 80% of patients at some point in their lives. Preceded by either frank vesiculation or subclinical disease activity, it typically causes no impairment in vision or movement of the eye. Rare patients with epidermolysis bullosa acquisita may present with clinical features most suggestive of cicatricial pemphigoid, to include blister formation within the oral cavity or along the external surfaces of the eyes.

References
Fitzpatrick TB, Eisen AZ, Wolff K, Freedberg IM, Austin KF, eds. Dermatology in General Medicine. 4th ed. New York, NY: McGraw-Hill Book Co; 1993:642-645.
Chan LS, Ahmed AR, Anhalt GJ, et al. The first international consensus on mucous membrane pemphigoid: definition, diagnostic criteria, pathogenic factors, medical treatment, and prognostic indicators. Arch Dermatol. 2002;138(3):370-379.
Egan CA, Yancey KB. The clinical and immunopathological manifestations of anti-epiligrin cicatricial pemphigoid, a recently defined subepithelial autoimmune blistering disease. Eur J Dermatol. 2000;10(8):585-589.
Fleming TE, Korman NJ. Cicatricial pemphigoid. J Am Acad Dermatol. 2000;43(4):571-591.
Nguyen QD, Foster CS. Cicatricial pemphigoid: diagnosis and treatment. Int Ophthalmol Clin. 1996;36(1):41-60.

----------

3. Which one of the following is true of this disease?
b)  Cutaneous immune deposits may disappear if a strict gluten-free diet is used

Dermatitis herpetiformis is an autoimmune disease characterized by pruritic vesicles arising in symmetrical array along the elbows, knees, buttocks, lumbosacral spine, and scalp. Granular deposits of IgA are present within the uppermost papillary dermis. Patients with dermatitis herpetiformis frequently have circulation autoantibodies within their sera against gliadin, reticulum, and smooth muscle endomysium, although cutaneous autoantibodies to these antigens have yet to be proven within lesional skin. Dermatitis herpetiformis does not share the same target for autoimmunity as occurs in linear IgA dermatosis. Recent data suggests that dermatitis herpetiformis patients may have autoimmunity to tissue transglutaminase as their underlying molecular basis.

References
Hall RP 3rd. Dermatitis herpetiformis. J Invest Dermatol. 1992;99(6):873-881.
Reunala TL. Dermatitis herpetiformis. Clin Dermatol. 2001;19(6):728-736.

 

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. 

 

1. Autoantibodies in this disease react with:
a) Centromeres or nucleoli
b) SSA and SSB antigens
c) Single-stranded DNA
d) Sm and native DNA
e) Keratins

2. The lesion which is present near the canthus of this patient most often occurs in which of the following diseases?
a) Bullous pemphigoid
b) Cicatricial pemphigoid
c) Pemphigus vulgaris
d) Dermatitis herpetiformis
e) Epidermolysis bullosa acquisita

 

3. Which one of the following is true of this disease?
a) Cutaneous immune deposits containing autoantibodies to reticulin, gliadin, and endomysium are present within the dermal papillae
b) Cutaneous immune deposits may disappear if a strict gluten-free diet is used
c) Granular IgG immune deposits are present within the papillary tufts
d) This disease has the same targeted antigen as in linear IgA dermatosis
e) Prednisone is the treatment of choice

 

 

1. Autoantibodies in this disease react with:
a) Centromeres or nucleoli

This patient has matted facial telangiectases, consistent with the diagnosis of CREST syndrome.

 

References
Sontheimer RD, Deng JS, Gilliam JN. Antinuclear and anticytoplasmic antibodies. Concepts and misconceptions. J Am Acad Dermatol. 1983;9(3):335-343.
Jarzabek-Chorzelska M, Blaszczyk M, Jablonska S, Chorzelski T, Kumar V, Beutner EH. Scl 70 antibody--a specific marker of systemic sclerosis. Br J Dermatol. 1986;115(4):393-401.
Sontheimer RD, McCauliffe DP, Zappi E, Targoff I. Antinuclear antibodies: clinical correlations and biologic significance. Adv Dermatol. 1992;7:3-52.
Mutasim DF, Adams BB. A practical guide for serologic evaluation of autoimmune connective tissue diseases. J Am Acad Dermatol. 2000;42(2 Pt 1):159-174.

----------

2. The lesion which is present near the canthus of this patient most often occurs in which of the following diseases?
b)  Cicatricial pemphigoid

The lesion depicted is a symblepharon—a fibrotic scar that connects the bulbar and tarsal conjunctival surfaces. It is a hallmark feature of cicatricial pemphigoid, occurring in at least 80% of patients at some point in their lives. Preceded by either frank vesiculation or subclinical disease activity, it typically causes no impairment in vision or movement of the eye. Rare patients with epidermolysis bullosa acquisita may present with clinical features most suggestive of cicatricial pemphigoid, to include blister formation within the oral cavity or along the external surfaces of the eyes.

References
Fitzpatrick TB, Eisen AZ, Wolff K, Freedberg IM, Austin KF, eds. Dermatology in General Medicine. 4th ed. New York, NY: McGraw-Hill Book Co; 1993:642-645.
Chan LS, Ahmed AR, Anhalt GJ, et al. The first international consensus on mucous membrane pemphigoid: definition, diagnostic criteria, pathogenic factors, medical treatment, and prognostic indicators. Arch Dermatol. 2002;138(3):370-379.
Egan CA, Yancey KB. The clinical and immunopathological manifestations of anti-epiligrin cicatricial pemphigoid, a recently defined subepithelial autoimmune blistering disease. Eur J Dermatol. 2000;10(8):585-589.
Fleming TE, Korman NJ. Cicatricial pemphigoid. J Am Acad Dermatol. 2000;43(4):571-591.
Nguyen QD, Foster CS. Cicatricial pemphigoid: diagnosis and treatment. Int Ophthalmol Clin. 1996;36(1):41-60.

----------

3. Which one of the following is true of this disease?
b)  Cutaneous immune deposits may disappear if a strict gluten-free diet is used

Dermatitis herpetiformis is an autoimmune disease characterized by pruritic vesicles arising in symmetrical array along the elbows, knees, buttocks, lumbosacral spine, and scalp. Granular deposits of IgA are present within the uppermost papillary dermis. Patients with dermatitis herpetiformis frequently have circulation autoantibodies within their sera against gliadin, reticulum, and smooth muscle endomysium, although cutaneous autoantibodies to these antigens have yet to be proven within lesional skin. Dermatitis herpetiformis does not share the same target for autoimmunity as occurs in linear IgA dermatosis. Recent data suggests that dermatitis herpetiformis patients may have autoimmunity to tissue transglutaminase as their underlying molecular basis.

References
Hall RP 3rd. Dermatitis herpetiformis. J Invest Dermatol. 1992;99(6):873-881.
Reunala TL. Dermatitis herpetiformis. Clin Dermatol. 2001;19(6):728-736.

 

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.

 

1. Autoantibodies in this disease react with:
a) Centromeres or nucleoli

This patient has matted facial telangiectases, consistent with the diagnosis of CREST syndrome.

 

References
Sontheimer RD, Deng JS, Gilliam JN. Antinuclear and anticytoplasmic antibodies. Concepts and misconceptions. J Am Acad Dermatol. 1983;9(3):335-343.
Jarzabek-Chorzelska M, Blaszczyk M, Jablonska S, Chorzelski T, Kumar V, Beutner EH. Scl 70 antibody--a specific marker of systemic sclerosis. Br J Dermatol. 1986;115(4):393-401.
Sontheimer RD, McCauliffe DP, Zappi E, Targoff I. Antinuclear antibodies: clinical correlations and biologic significance. Adv Dermatol. 1992;7:3-52.
Mutasim DF, Adams BB. A practical guide for serologic evaluation of autoimmune connective tissue diseases. J Am Acad Dermatol. 2000;42(2 Pt 1):159-174.

----------

2. The lesion which is present near the canthus of this patient most often occurs in which of the following diseases?
b)  Cicatricial pemphigoid

The lesion depicted is a symblepharon—a fibrotic scar that connects the bulbar and tarsal conjunctival surfaces. It is a hallmark feature of cicatricial pemphigoid, occurring in at least 80% of patients at some point in their lives. Preceded by either frank vesiculation or subclinical disease activity, it typically causes no impairment in vision or movement of the eye. Rare patients with epidermolysis bullosa acquisita may present with clinical features most suggestive of cicatricial pemphigoid, to include blister formation within the oral cavity or along the external surfaces of the eyes.

References
Fitzpatrick TB, Eisen AZ, Wolff K, Freedberg IM, Austin KF, eds. Dermatology in General Medicine. 4th ed. New York, NY: McGraw-Hill Book Co; 1993:642-645.
Chan LS, Ahmed AR, Anhalt GJ, et al. The first international consensus on mucous membrane pemphigoid: definition, diagnostic criteria, pathogenic factors, medical treatment, and prognostic indicators. Arch Dermatol. 2002;138(3):370-379.
Egan CA, Yancey KB. The clinical and immunopathological manifestations of anti-epiligrin cicatricial pemphigoid, a recently defined subepithelial autoimmune blistering disease. Eur J Dermatol. 2000;10(8):585-589.
Fleming TE, Korman NJ. Cicatricial pemphigoid. J Am Acad Dermatol. 2000;43(4):571-591.
Nguyen QD, Foster CS. Cicatricial pemphigoid: diagnosis and treatment. Int Ophthalmol Clin. 1996;36(1):41-60.

----------

3. Which one of the following is true of this disease?
b)  Cutaneous immune deposits may disappear if a strict gluten-free diet is used

Dermatitis herpetiformis is an autoimmune disease characterized by pruritic vesicles arising in symmetrical array along the elbows, knees, buttocks, lumbosacral spine, and scalp. Granular deposits of IgA are present within the uppermost papillary dermis. Patients with dermatitis herpetiformis frequently have circulation autoantibodies within their sera against gliadin, reticulum, and smooth muscle endomysium, although cutaneous autoantibodies to these antigens have yet to be proven within lesional skin. Dermatitis herpetiformis does not share the same target for autoimmunity as occurs in linear IgA dermatosis. Recent data suggests that dermatitis herpetiformis patients may have autoimmunity to tissue transglutaminase as their underlying molecular basis.

References
Hall RP 3rd. Dermatitis herpetiformis. J Invest Dermatol. 1992;99(6):873-881.
Reunala TL. Dermatitis herpetiformis. Clin Dermatol. 2001;19(6):728-736.

 

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.