Practice questions vesiculobullous disorders

Practice questions

Vesiculobullous disorders

Updated: 03/01/2019

© Jun Wang, MD, PhD

1. Use this image and this case for the next four question. A 44-year-old man presents with painful blistering lesions involving his chest, back and oral mucosa. These blisters start from his oral mucosa and spread to his chest and back. His past medical history is unremarkable. Physical examination reveals blisters from a few millimeters to 5 cm in greatest dimension. Some have ruptures with purulent exudates. An image of the biopsy is shown. Detached clusters of round to polygonal keratinocytes are seen. There is no evidence of significant cytological atypia. What is the diagnosis?

(Image credit: Nephron [CC BY-SA 3.0 (https:\creativecommons.org\licenses\by-sa\3.0)])

A. Bullous pemphigoid

B. Dermatitis herpetiformis

C. Epidermolysis bullosa

D. Herpes

E. Pemphigus vulgaris

2. A 44-year-old man presents with painful blistering lesions involving his chest, back and oral mucosa. These blisters start from his oral mucosa and spread to his chest and back. His past medical history is unremarkable. Physical examination reveals blisters from a few millimeters to 5 cm in greatest dimension. Some have ruptures with purulent exudates. An image of the biopsy is shown. Detached clusters of round to polygonal keratinocytes are seen. There is no evidence of significant cytological atypia. What is directly causing these changes?

(Image credit: Nephron [CC BY-SA 3.0 (https:\creativecommons.org\licenses\by-sa\3.0)])

A. Autoimmune process

B. Congenital defect

C. Trauma

D. UV light

E. Viral infection

3. A 44-year-old man presents with painful blistering lesions involving his chest, back and oral mucosa. These blisters start from his oral mucosa and spread to his chest and back. His past medical history is unremarkable. Physical examination reveals blisters from a few millimeters to 5 cm in greatest dimension. Some have ruptures with purulent exudates. An image of the biopsy is shown. Detached clusters of round to polygonal keratinocytes are seen. There is no evidence of significant cytological atypia. What immunofluoscence test results is most likely seen?

(Image credit: Nephron [CC BY-SA 3.0 (https:\creativecommons.org\licenses\by-sa\3.0)])

A. Clumped deposit of IgM at dermoepidermal junction

B. Deposit of IgG along epidermal intercellular junctions

C. Granular deposit of IgA at dermal papillae

D. Linear deposit of IgG along dermoepidermal junction

4. A 44-year-old man presents with painful blistering lesions involving his chest, back and oral mucosa. These blisters start from his oral mucosa and spread to his chest and back. His past medical history is unremarkable. Physical examination reveals blisters from a few millimeters to 5 cm in greatest dimension. Some have ruptures with purulent exudates. An image of the biopsy is shown. Detached clusters of round to polygonal keratinocytes are seen. There is no evidence of significant cytological atypia. Abnormality of what molecule is most likely associated with these findings?

(Image credit: Nephron [CC BY-SA 3.0 (https:\creativecommons.org\licenses\by-sa\3.0)])

A. BP180

B. Collagen VII

C. Desmoglein 1

D. Keratin 5

E. Laminin 5

5. A 55-year-old woman presents with erosions on her chest and back for 4 months. She has a history of type 2 diabetes, but denies any history of allergy. Physical examinations reveals small erosions up to 1.5 cm with an erythematous base, located on her trunk. No other abnormalities are noted. Biopsy reveals skin with mild spongiosis. Intraepidermal vesicles are noted at the granular layer. There is no significant acanthosis, hyperplasia or cytological atypia. Immunofluorescence studies reveal deposit of IgG along keratinocytic borders. What is the diagnosis?

A. Bullous pemphigoid

B. Epidermolysis bullosa

C. Pemphigus erythematosus

D. Pemphigus foliaceus

E. Pemphigus vegetans

6. Use this case for the next 2 cases. A 79-year-old man presents with thickened skin at his arms. The lesions developed from clusters of small pustules. He has a history of type 2 diabetes and hypertension. His family history is significant for skin cancers. Physical examination reveals rubbery grayish plaques at both arms. A few pustules are noted in adjacent skin. Biopsy of the lesions reveals marked acanthosis, focal spongiosis and intraepidermal microabscesses. No significant cytological atypia is noted. Immunofluorescent studies reveal diffuse IgG deposit along keratinocyte borders. What is the diagnosis?

A. Bullous pemphigoid

B. Pemphigus erythematosus

C. Pemphigus foliaceus

D. Pemphigus vegetans

E. Squamous cell carcinoma

7. A 79-year-old man presents with thickened skin at his arms. The lesions developed from clusters of small pustules. He has a history of type 2 diabetes and hypertension. His family history is significant for skin cancers. Physical examination reveals rubbery grayish plaques at both arms. A few pustules are noted in adjacent skin. Biopsy of the lesions reveals marked acanthosis, focal spongiosis and intraepidermal microabscesses. No significant cytological atypia is noted. Immunofluorescent studies reveal diffuse IgG deposit along keratinocyte borders. What is likely the cause of these findings?

A. Autoimmune antibody against desmoglein 1

B. Complement deposit at dermoepidermal junction

C. Congenital defect of keratin 5

D. UV-induced malignant transformation of keratinocytes

E. Viral infection

8. Use this image and this case for the next four question. A 65-year-old man presents with large blisters at his left shoulder and chest for a month. His past medical history is unremarkable. These blisters appear to be tense and contain clear fluid. An image of the biopsy is shown. There is no evidence of significant cytological atypia. What is the diagnosis?

(Image credit: Sarahkayb [CC BY-SA 4.0 (https:\creativecommons.org\licenses\by-sa\4.0)])

A. Bullous pemphigoid

B. Dermatitis herpetiformis

C. Epidermolysis bullosa

D. Herpes

E. Pemphigus vulgaris

9. A 65-year-old man presents with large blisters at his left shoulder and chest for a month. His past medical history is unremarkable. These blisters appear to be tense and contain clear fluid. An image of the biopsy is shown. There is no evidence of significant cytological atypia. What is the cause of these findings?

(Image credit: Sarahkayb [CC BY-SA 4.0 (https:\creativecommons.org\licenses\by-sa\4.0)])

A. Autoimmune process

B. Congenital defect

C. Trauma

D. UV light

E. Viral infection

10. A 65-year-old man presents with large blisters at his left shoulder and chest for a month. His past medical history is unremarkable. These blisters appear to be tense and contain clear fluid. An image of the biopsy is shown. There is no evidence of significant cytological atypia. Abnormality of what molecule is most likely associated with these findings?

(Image credit: Sarahkayb [CC BY-SA 4.0 (https:\creativecommons.org\licenses\by-sa\4.0)])

A. BP180

B. Collagen VII

C. Desmoglein 1

D. Keratin 5

E. Laminin 5

11. A 65-year-old man presents with large blisters at his left shoulder and chest for a month. His past medical history is unremarkable. These blisters appear to be tense and contain clear fluid. An image of the biopsy is shown. There is no evidence of significant cytological atypia. What immunofluoscence test results is most likely seen?

(Image credit: Sarahkayb [CC BY-SA 4.0 (https:\creativecommons.org\licenses\by-sa\4.0)])

A. Clumped deposit of IgM at dermoepidermal junction

B. Deposit of IgG along epidermal intercellular junctions

C. Granular deposit of IgA at dermal papillae

D. Linear deposit of IgG along dermoepidermal junction

12. Use this case for the next 3 questions. A 12-year-old boy presents with pruritic skin lesions at his back. He has a history of intermittent bloating and diarrhea. He denies history of abdominal pain, cough, fever, and other symptoms. Physical examination reveals small pustules in clusters at his back. No other abnormalities are noted. Biopsy of the vesicle reveals separation along dermoepidermal junction with neutrophilic aggregates. The keratinocytes have mild atypia but no multinucleated cells nor nuclear abnormalities are seen. Special stains reveals no evidence of microorganisms. What is most likely the diagnosis?

A. Bullous pemphigoid

B. Dermatitis herpetiformis

C. Epidermolysis bullosa

D. Herpes

E. Pemphigus vulgaris

13. A 12-year-old boy presents with pruritic skin lesions at his back. He has a history of intermittent bloating and diarrhea. He denies history of abdominal pain, cough, fever, and other symptoms. Physical examination reveals small pustules in clusters at his back. No other abnormalities are noted. Biopsy of the vesicle reveals separation along dermoepidermal junction with neutrophilic aggregates. The keratinocytes have mild atypia but no multinucleated cells nor nuclear abnormalities are seen. Special stains reveals no evidence of microorganisms. What pattern of immunofluorescence study is expected?

A. Clumped deposit of IgM at dermoepidermal junction

B. Deposit of IgG along epidermal intercellular junctions

C. Granular deposit of IgA at dermal papillae

D. Linear deposit of IgA along dermoepidermal junction

E. Linear deposit of IgG along dermoepidermal junction

14. A 12-year-old boy presents with pruritic skin lesions at his back. He has a history of intermittent bloating and diarrhea. He denies history of abdominal pain, cough, fever, and other symptoms. Physical examination reveals small pustules in clusters at his back. No other abnormalities are noted. Biopsy of the vesicle reveals separation along dermoepidermal junction with neutrophilic aggregates. The keratinocytes have mild atypia but no multinucleated cells nor nuclear abnormalities are seen. Special stains reveals no evidence of microorganisms. What is most likely associated with his bloating and diarrhea?

A. Absence of enteric ganglion

B. Autoimmune gastritis and duodenitis

C. Gluten hypersensitivity

D. Herpes gastritis

E. Pyloric smooth muscle hyperplasia

15. Use this case for the next 3 questions. A 1-day-old boy develops skin sloughing and appears to be in pain. New blisters and sloughing appear during the initial management. The skin around joints is extremely easy to be peeled off. He was born at term. The prenatal course is unremarkable. Biopsy reveals separation between basal and upper layer epidermis. Both dermoepidermal junction and dermis are morphologically unremarkable. There is no spongiosis, acantholysis, nor inflammation. What is likely the diagnosis?

A. Bullous pemphigoid

B. Dermatitis herpetiformis

C. Epidermolysis bullosa simplex

D. Herpes

E. Pemphigus vulgaris

16. A 1-day-old boy develops skin sloughing and appears to be in pain. New blisters and sloughing appear during the initial management. The skin around joints is extremely easy to be peeled off. He was born at term. The prenatal course is unremarkable. Biopsy reveals separation between basal and upper layer epidermis. Both dermoepidermal junction and dermis are morphologically unremarkable. There is no spongiosis, acantholysis, nor inflammation. Defect of what molecule is likely to causing these presentations?

A. BP180 or BP230

B. Collagen VII

C. Desmoglein 1 or desmoglein 3

D. Keratin 5

E. Laminin 5

17. A 1-day-old boy develops skin sloughing and appears to be in pain. New blisters and sloughing appear during the initial management. The skin around joints is extremely easy to be peeled off. He was born at term. The prenatal course is unremarkable. Biopsy reveals separation between basal and upper layer epidermis. Both dermoepidermal junction and dermis are morphologically unremarkable. There is no spongiosis, acantholysis, nor inflammation. What test is likely to confirm the diagnosis?

A. Gram stain and culture

B. Immunofluorescence mapping

C. Lymphocyte phenotyping

D. Quantitative serum immunoglobulin levels

E. Viral DNA analysis

18. Use this image and this case for the next 2 questions. A 7-year-old boy presents with deformation of feet. He has had painful skin erosions since birth. He has a few family members with easy peeled skin. An image of his feet is shown. What is likely the diagnosis?

(Image credit: Master Sgt. Keith A. Milks [Public domain], via Wikimedia Commons [Public domain])

A. Bullous pemphigoid

B. Epidermolysis bullosa dystrophic type

C. Epidermolysis bullosa junctional type

D. Epidermolysis bullosa simplex

E. Kindler syndrome

19. A 7-year-old boy presents with deformation of feet. He has had painful skin erosions since birth. He has a few family members with easy peeled skin. An image of his feet is shown. What defect is likely to be associated with these presentations?

(Image credit: Master Sgt. Keith A. Milks [Public domain], via Wikimedia Commons [Public domain])

A. Actin-ECM linkage

B. Collagen VII

C. Desmoglein 1 or desmoglein 3

D. Keratin 5

E. Laminin 5

Answers

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