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Case Report and Brief Review

The Dermatologist’s Board Review - November 2017

November 2017

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.

board review eye1. Patients with an autoimmune blistering disease limited to this location most likely have autoantibodies to which adhesion protein?  
 

a) Type VII collagen

b) Bullous pemphigoid 230

c) Type IV collagen

d) β4 integrin

e) Desmoglein 1

 

board review lesions 2. These lesions:
 

a) Are present in most patients with systemic lupus erythematosus (SLE)

b) Are associated with an increased incidence of severe renal disease in patients with SLE

c) Usually heal without scars

d) Do not occur in SLE

e) Are associated with an increased incidence of SLE if present both above and below the neck (generalized)

To learn the answers, go to page 2

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Board Review Answers

board review 11. Patients with an autoimmune blistering disease limited to this location most likely have autoantibodies to which adhesion protein?
 

d) β4 integrin 

Patients with autoimmune blister formation limited to the external ocular mucosa usually represent a variant of cicatricial pemphigoid, although similar findings may rarely occur in pemphigus vulgaris. Several adhesion proteins may be targeted for autoimmunity in cicatricial pemphigoid, most commonly laminin 332 (laminin 5, epiligrin, kalinin) and type XVII collagen (bullous pemphigoid antigen 2). Rare patients with cicatricial pemphigoid confined to the eyes may have autoantibodies to β4 integrin. 

Reference

Tyagi S, Bhol K, Natarajan K, Livir-Rallatos C, Foster CS, Ahmend AR. Ocular cicatricial pemphigoid antigen: partial sequence and biochemical characterization. Proc Natl Acad Sci U S A. 1996;93(25):14714-14719.

 

board review lesions 2. These lesions:
 

e) Are associated with an increased incidence of SLE if present both above and below the neck (generalized) 

Discoid lupus erythematosus skin lesions occur in 15% to 30% of patients with SLE. However, patients with SLE and discoid lesions appear to have a less severe clinical course than patients with SLE without the discoid lesions. They have a relatively decreased incidence of severe renal disease, particularly proliferative glomerulonephritis, and a lower incidence of hypocomplementemia, and antibodies to native DNA. Several studies have shown that patients with chronic cutaneous lupus erythematosus and discoid lesions in a generalized distribution are at greater risk of developing SLE compared with patients with discoid lesions affecting only the head. 

References

Gilliam JN, Sontheimer RD. Subacute cutaneous lupus erythematosus. Clin Rheumatic Dis. 1982;8(2):343-352.

Gilliam JN, Sontheimer RD. Skin manifestations of SLE. Clin Rheumatic Dis. 1982;8(1):207-218.

Callen JP. Systemic lupus erythematosus in patients with chronic cutaneous (discoid) lupus erythematosus. Clinical and laboratory findings in seventeen patients. J Am Acad Dermatol. 1985;12(2 Pt 1):278-288.

McCauliffe DP, Sontheimer RD. Dermatologic manifestations of rheumatic disorders. Prim Care. 1993;20(4):925-941.

McCauliffe DP. Cutaneous lupus erythematosus. Semin Cutan Med Surg. 2001;20(1):14-26.

Patel P, Werth V. Cutaneous lupus erythematosus: a review. Dermatol Clin. 2002;20(3):373-385, v.

 

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.

board review eye1. Patients with an autoimmune blistering disease limited to this location most likely have autoantibodies to which adhesion protein?  
 

a) Type VII collagen

b) Bullous pemphigoid 230

c) Type IV collagen

d) β4 integrin

e) Desmoglein 1

 

board review lesions 2. These lesions:
 

a) Are present in most patients with systemic lupus erythematosus (SLE)

b) Are associated with an increased incidence of severe renal disease in patients with SLE

c) Usually heal without scars

d) Do not occur in SLE

e) Are associated with an increased incidence of SLE if present both above and below the neck (generalized)

To learn the answers, go to page 2

{{pagebreak}}

Board Review Answers

board review 11. Patients with an autoimmune blistering disease limited to this location most likely have autoantibodies to which adhesion protein?
 

d) β4 integrin 

Patients with autoimmune blister formation limited to the external ocular mucosa usually represent a variant of cicatricial pemphigoid, although similar findings may rarely occur in pemphigus vulgaris. Several adhesion proteins may be targeted for autoimmunity in cicatricial pemphigoid, most commonly laminin 332 (laminin 5, epiligrin, kalinin) and type XVII collagen (bullous pemphigoid antigen 2). Rare patients with cicatricial pemphigoid confined to the eyes may have autoantibodies to β4 integrin. 

Reference

Tyagi S, Bhol K, Natarajan K, Livir-Rallatos C, Foster CS, Ahmend AR. Ocular cicatricial pemphigoid antigen: partial sequence and biochemical characterization. Proc Natl Acad Sci U S A. 1996;93(25):14714-14719.

 

board review lesions 2. These lesions:
 

e) Are associated with an increased incidence of SLE if present both above and below the neck (generalized) 

Discoid lupus erythematosus skin lesions occur in 15% to 30% of patients with SLE. However, patients with SLE and discoid lesions appear to have a less severe clinical course than patients with SLE without the discoid lesions. They have a relatively decreased incidence of severe renal disease, particularly proliferative glomerulonephritis, and a lower incidence of hypocomplementemia, and antibodies to native DNA. Several studies have shown that patients with chronic cutaneous lupus erythematosus and discoid lesions in a generalized distribution are at greater risk of developing SLE compared with patients with discoid lesions affecting only the head. 

References

Gilliam JN, Sontheimer RD. Subacute cutaneous lupus erythematosus. Clin Rheumatic Dis. 1982;8(2):343-352.

Gilliam JN, Sontheimer RD. Skin manifestations of SLE. Clin Rheumatic Dis. 1982;8(1):207-218.

Callen JP. Systemic lupus erythematosus in patients with chronic cutaneous (discoid) lupus erythematosus. Clinical and laboratory findings in seventeen patients. J Am Acad Dermatol. 1985;12(2 Pt 1):278-288.

McCauliffe DP, Sontheimer RD. Dermatologic manifestations of rheumatic disorders. Prim Care. 1993;20(4):925-941.

McCauliffe DP. Cutaneous lupus erythematosus. Semin Cutan Med Surg. 2001;20(1):14-26.

Patel P, Werth V. Cutaneous lupus erythematosus: a review. Dermatol Clin. 2002;20(3):373-385, v.

 

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.

board review eye1. Patients with an autoimmune blistering disease limited to this location most likely have autoantibodies to which adhesion protein?  
 

a) Type VII collagen

b) Bullous pemphigoid 230

c) Type IV collagen

d) β4 integrin

e) Desmoglein 1

 

board review lesions 2. These lesions:
 

a) Are present in most patients with systemic lupus erythematosus (SLE)

b) Are associated with an increased incidence of severe renal disease in patients with SLE

c) Usually heal without scars

d) Do not occur in SLE

e) Are associated with an increased incidence of SLE if present both above and below the neck (generalized)

To learn the answers, go to page 2

{{pagebreak}}

Board Review Answers

board review 11. Patients with an autoimmune blistering disease limited to this location most likely have autoantibodies to which adhesion protein?
 

d) β4 integrin 

Patients with autoimmune blister formation limited to the external ocular mucosa usually represent a variant of cicatricial pemphigoid, although similar findings may rarely occur in pemphigus vulgaris. Several adhesion proteins may be targeted for autoimmunity in cicatricial pemphigoid, most commonly laminin 332 (laminin 5, epiligrin, kalinin) and type XVII collagen (bullous pemphigoid antigen 2). Rare patients with cicatricial pemphigoid confined to the eyes may have autoantibodies to β4 integrin. 

Reference

Tyagi S, Bhol K, Natarajan K, Livir-Rallatos C, Foster CS, Ahmend AR. Ocular cicatricial pemphigoid antigen: partial sequence and biochemical characterization. Proc Natl Acad Sci U S A. 1996;93(25):14714-14719.

 

board review lesions 2. These lesions:
 

e) Are associated with an increased incidence of SLE if present both above and below the neck (generalized) 

Discoid lupus erythematosus skin lesions occur in 15% to 30% of patients with SLE. However, patients with SLE and discoid lesions appear to have a less severe clinical course than patients with SLE without the discoid lesions. They have a relatively decreased incidence of severe renal disease, particularly proliferative glomerulonephritis, and a lower incidence of hypocomplementemia, and antibodies to native DNA. Several studies have shown that patients with chronic cutaneous lupus erythematosus and discoid lesions in a generalized distribution are at greater risk of developing SLE compared with patients with discoid lesions affecting only the head. 

References

Gilliam JN, Sontheimer RD. Subacute cutaneous lupus erythematosus. Clin Rheumatic Dis. 1982;8(2):343-352.

Gilliam JN, Sontheimer RD. Skin manifestations of SLE. Clin Rheumatic Dis. 1982;8(1):207-218.

Callen JP. Systemic lupus erythematosus in patients with chronic cutaneous (discoid) lupus erythematosus. Clinical and laboratory findings in seventeen patients. J Am Acad Dermatol. 1985;12(2 Pt 1):278-288.

McCauliffe DP, Sontheimer RD. Dermatologic manifestations of rheumatic disorders. Prim Care. 1993;20(4):925-941.

McCauliffe DP. Cutaneous lupus erythematosus. Semin Cutan Med Surg. 2001;20(1):14-26.

Patel P, Werth V. Cutaneous lupus erythematosus: a review. Dermatol Clin. 2002;20(3):373-385, v.

 

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.