1. This patient had mild arthralgias, alopecia, Raynaud disease, photosensitivity, and these skin lesions. Which one of the following is true of this disease?
a) A significant number of these patients (>20%) fail to meet American College of Rheumatology criteria for the diagnosis of systemic lupus erythematosus
b) All of these cases are positive for antinuclear antibodies
c) There is a low incidence of antibodies to the cytoplasmic antigens Ro (SSA) and La (SSB)
d) The lupus band test is positive in 80% of lesional skin
e) These patients have a low incidence of the HLA antigens A1, B8, and Dw3
2. IgA autoantibodies producing this pattern of immunofluorescence usually react with:
a) A 180 kD glycoprotein in the lamina densa
b) A 97-120 kD epitope of the 180 kD glycoprotein in the lamina lucida
c) A 250-280 kD glycoprotein on and beneath the lamina densa
d) A 250-280 kD glycoprotein in the lamina lucida
e) Unidentified antigens in dermal papillae
To learn the answers, go to page 2
BOARD REVIEW ANSWERS
1. This patient had mild arthralgias, alopecia, Raynaud disease, photosensitivity, and these skin lesions. Which one of the following is true of this disease?
a) A significant number of these patients (>20%) fail to meet American College of Rheumatology criteria for the diagnosis of systemic lupus erythematosus
A characteristic of subacute cutaneous lupus erythematosus (SCLE) is annular or polycyclic lesions which occur in about one-third of patients. Most patients have papulosquamous lesions. SCLE is characterized by mild systemic disease, a high incidence of photosensitivity and arthralgias, anti-Ro antibodies, and an HLA A1, B8, and Dw3 serotype. The lupus band test is positive in about 50% of lesional and 30% of nonlesional skin specimens in SCLE.
References
Sontheimer RD, Thomas JR, Gilliam JN. Subacute cutaneous lupus erythematosus: a cutaneous marker for a distinct lupus erythematosus subset. Arch Dermatol. 1979;115(12):1409-1415.
Sontheimer RD. Subacute cutaneous lupus erythematosus: a decade’s perspective. Med Clin North Am. 1989;73(5):1073-1090.
Sontheimer RD. The lexicon of cutaneous lupus erythematosus--a review and personal perspective on the nomenclature and classification of the cutaneous manifestations of lupus erythematosus. Lupus. 1997;6(2):84-95.
Patel P, Werth V. Cutaneous lupus erythematosus: a review. Dermatol Clin. 2002;20(3):373-385, v.
Kuhn A, Sontheimer RD. Cutaneous lupus erythematosus: molecular and cellular basis of clinical findings. Curr Dir Autoimmun. 2008;10:119-140.
2. IgA autoantibodies producing this pattern of immunofluorescence usually react with:
b) A 97-120 kD epitope of the 180 kD glycoprotein in the lamina lucida
IgA staining in this direct immunofluorescence photomicrograph shows a linear homogenous pattern along the dermoepidermal junction, consistent with (but not restricted to) linear IgA dermatosis. Most of these autoantibodies react against the 120-kD proteolytic fragment of type XVII collagen. Others react against a 97-kD epitope of the same protein. Direct immunoelectron microscopy demonstrates that this protein is present within the lamina lucida.
References
Egan CA, Taylor TB, Meyer LJ, Petersen MJ, Zone JJ. Bullous pemphigoid sera that contain antibodies to BPAg2 also contain antibodies to LABD97 that recognize epitopes distal to the NC16A domain. J Invest Dermatol. 1999;112(2):148-152.
Zillikens D, Herzele K, Georgi M, et al. Autoantibodies in a subgroup of patients with linear IgA disease react with the NC16A domain of BP1801. J Invest Dermatol. 1999;113(6):947-953.
Metz BJ, Ruggeri SY, Hsu S, et al. Linear IgA dermatosis with IgA and IgG autoantibodies to the 180 kDa bullous pemphigoid antigen (BP180): evidence for a distinct subtype. Int J Dermatol. 2004;43(6):443-446.
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. This patient had mild arthralgias, alopecia, Raynaud disease, photosensitivity, and these skin lesions. Which one of the following is true of this disease?
a) A significant number of these patients (>20%) fail to meet American College of Rheumatology criteria for the diagnosis of systemic lupus erythematosus
b) All of these cases are positive for antinuclear antibodies
c) There is a low incidence of antibodies to the cytoplasmic antigens Ro (SSA) and La (SSB)
d) The lupus band test is positive in 80% of lesional skin
e) These patients have a low incidence of the HLA antigens A1, B8, and Dw3
2. IgA autoantibodies producing this pattern of immunofluorescence usually react with:
a) A 180 kD glycoprotein in the lamina densa
b) A 97-120 kD epitope of the 180 kD glycoprotein in the lamina lucida
c) A 250-280 kD glycoprotein on and beneath the lamina densa
d) A 250-280 kD glycoprotein in the lamina lucida
e) Unidentified antigens in dermal papillae
1. This patient had mild arthralgias, alopecia, Raynaud disease, photosensitivity, and these skin lesions. Which one of the following is true of this disease?
a) A significant number of these patients (>20%) fail to meet American College of Rheumatology criteria for the diagnosis of systemic lupus erythematosus
A characteristic of subacute cutaneous lupus erythematosus (SCLE) is annular or polycyclic lesions which occur in about one-third of patients. Most patients have papulosquamous lesions. SCLE is characterized by mild systemic disease, a high incidence of photosensitivity and arthralgias, anti-Ro antibodies, and an HLA A1, B8, and Dw3 serotype. The lupus band test is positive in about 50% of lesional and 30% of nonlesional skin specimens in SCLE.
References
Sontheimer RD, Thomas JR, Gilliam JN. Subacute cutaneous lupus erythematosus: a cutaneous marker for a distinct lupus erythematosus subset. Arch Dermatol. 1979;115(12):1409-1415.
Sontheimer RD. Subacute cutaneous lupus erythematosus: a decade’s perspective. Med Clin North Am. 1989;73(5):1073-1090.
Sontheimer RD. The lexicon of cutaneous lupus erythematosus--a review and personal perspective on the nomenclature and classification of the cutaneous manifestations of lupus erythematosus. Lupus. 1997;6(2):84-95.
Patel P, Werth V. Cutaneous lupus erythematosus: a review. Dermatol Clin. 2002;20(3):373-385, v.
Kuhn A, Sontheimer RD. Cutaneous lupus erythematosus: molecular and cellular basis of clinical findings. Curr Dir Autoimmun. 2008;10:119-140.
2. IgA autoantibodies producing this pattern of immunofluorescence usually react with:
b) A 97-120 kD epitope of the 180 kD glycoprotein in the lamina lucida
IgA staining in this direct immunofluorescence photomicrograph shows a linear homogenous pattern along the dermoepidermal junction, consistent with (but not restricted to) linear IgA dermatosis. Most of these autoantibodies react against the 120-kD proteolytic fragment of type XVII collagen. Others react against a 97-kD epitope of the same protein. Direct immunoelectron microscopy demonstrates that this protein is present within the lamina lucida.
References
Egan CA, Taylor TB, Meyer LJ, Petersen MJ, Zone JJ. Bullous pemphigoid sera that contain antibodies to BPAg2 also contain antibodies to LABD97 that recognize epitopes distal to the NC16A domain. J Invest Dermatol. 1999;112(2):148-152.
Zillikens D, Herzele K, Georgi M, et al. Autoantibodies in a subgroup of patients with linear IgA disease react with the NC16A domain of BP1801. J Invest Dermatol. 1999;113(6):947-953.
Metz BJ, Ruggeri SY, Hsu S, et al. Linear IgA dermatosis with IgA and IgG autoantibodies to the 180 kDa bullous pemphigoid antigen (BP180): evidence for a distinct subtype. Int J Dermatol. 2004;43(6):443-446.
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. This patient had mild arthralgias, alopecia, Raynaud disease, photosensitivity, and these skin lesions. Which one of the following is true of this disease?
a) A significant number of these patients (>20%) fail to meet American College of Rheumatology criteria for the diagnosis of systemic lupus erythematosus
b) All of these cases are positive for antinuclear antibodies
c) There is a low incidence of antibodies to the cytoplasmic antigens Ro (SSA) and La (SSB)
d) The lupus band test is positive in 80% of lesional skin
e) These patients have a low incidence of the HLA antigens A1, B8, and Dw3
2. IgA autoantibodies producing this pattern of immunofluorescence usually react with:
a) A 180 kD glycoprotein in the lamina densa
b) A 97-120 kD epitope of the 180 kD glycoprotein in the lamina lucida
c) A 250-280 kD glycoprotein on and beneath the lamina densa
d) A 250-280 kD glycoprotein in the lamina lucida
e) Unidentified antigens in dermal papillae
,
1. This patient had mild arthralgias, alopecia, Raynaud disease, photosensitivity, and these skin lesions. Which one of the following is true of this disease?
a) A significant number of these patients (>20%) fail to meet American College of Rheumatology criteria for the diagnosis of systemic lupus erythematosus
b) All of these cases are positive for antinuclear antibodies
c) There is a low incidence of antibodies to the cytoplasmic antigens Ro (SSA) and La (SSB)
d) The lupus band test is positive in 80% of lesional skin
e) These patients have a low incidence of the HLA antigens A1, B8, and Dw3
2. IgA autoantibodies producing this pattern of immunofluorescence usually react with:
a) A 180 kD glycoprotein in the lamina densa
b) A 97-120 kD epitope of the 180 kD glycoprotein in the lamina lucida
c) A 250-280 kD glycoprotein on and beneath the lamina densa
d) A 250-280 kD glycoprotein in the lamina lucida
e) Unidentified antigens in dermal papillae
To learn the answers, go to page 2
BOARD REVIEW ANSWERS
1. This patient had mild arthralgias, alopecia, Raynaud disease, photosensitivity, and these skin lesions. Which one of the following is true of this disease?
a) A significant number of these patients (>20%) fail to meet American College of Rheumatology criteria for the diagnosis of systemic lupus erythematosus
A characteristic of subacute cutaneous lupus erythematosus (SCLE) is annular or polycyclic lesions which occur in about one-third of patients. Most patients have papulosquamous lesions. SCLE is characterized by mild systemic disease, a high incidence of photosensitivity and arthralgias, anti-Ro antibodies, and an HLA A1, B8, and Dw3 serotype. The lupus band test is positive in about 50% of lesional and 30% of nonlesional skin specimens in SCLE.
References
Sontheimer RD, Thomas JR, Gilliam JN. Subacute cutaneous lupus erythematosus: a cutaneous marker for a distinct lupus erythematosus subset. Arch Dermatol. 1979;115(12):1409-1415.
Sontheimer RD. Subacute cutaneous lupus erythematosus: a decade’s perspective. Med Clin North Am. 1989;73(5):1073-1090.
Sontheimer RD. The lexicon of cutaneous lupus erythematosus--a review and personal perspective on the nomenclature and classification of the cutaneous manifestations of lupus erythematosus. Lupus. 1997;6(2):84-95.
Patel P, Werth V. Cutaneous lupus erythematosus: a review. Dermatol Clin. 2002;20(3):373-385, v.
Kuhn A, Sontheimer RD. Cutaneous lupus erythematosus: molecular and cellular basis of clinical findings. Curr Dir Autoimmun. 2008;10:119-140.
2. IgA autoantibodies producing this pattern of immunofluorescence usually react with:
b) A 97-120 kD epitope of the 180 kD glycoprotein in the lamina lucida
IgA staining in this direct immunofluorescence photomicrograph shows a linear homogenous pattern along the dermoepidermal junction, consistent with (but not restricted to) linear IgA dermatosis. Most of these autoantibodies react against the 120-kD proteolytic fragment of type XVII collagen. Others react against a 97-kD epitope of the same protein. Direct immunoelectron microscopy demonstrates that this protein is present within the lamina lucida.
References
Egan CA, Taylor TB, Meyer LJ, Petersen MJ, Zone JJ. Bullous pemphigoid sera that contain antibodies to BPAg2 also contain antibodies to LABD97 that recognize epitopes distal to the NC16A domain. J Invest Dermatol. 1999;112(2):148-152.
Zillikens D, Herzele K, Georgi M, et al. Autoantibodies in a subgroup of patients with linear IgA disease react with the NC16A domain of BP1801. J Invest Dermatol. 1999;113(6):947-953.
Metz BJ, Ruggeri SY, Hsu S, et al. Linear IgA dermatosis with IgA and IgG autoantibodies to the 180 kDa bullous pemphigoid antigen (BP180): evidence for a distinct subtype. Int J Dermatol. 2004;43(6):443-446.
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. This patient had mild arthralgias, alopecia, Raynaud disease, photosensitivity, and these skin lesions. Which one of the following is true of this disease?
a) A significant number of these patients (>20%) fail to meet American College of Rheumatology criteria for the diagnosis of systemic lupus erythematosus
b) All of these cases are positive for antinuclear antibodies
c) There is a low incidence of antibodies to the cytoplasmic antigens Ro (SSA) and La (SSB)
d) The lupus band test is positive in 80% of lesional skin
e) These patients have a low incidence of the HLA antigens A1, B8, and Dw3
2. IgA autoantibodies producing this pattern of immunofluorescence usually react with:
a) A 180 kD glycoprotein in the lamina densa
b) A 97-120 kD epitope of the 180 kD glycoprotein in the lamina lucida
c) A 250-280 kD glycoprotein on and beneath the lamina densa
d) A 250-280 kD glycoprotein in the lamina lucida
e) Unidentified antigens in dermal papillae
1. This patient had mild arthralgias, alopecia, Raynaud disease, photosensitivity, and these skin lesions. Which one of the following is true of this disease?
a) A significant number of these patients (>20%) fail to meet American College of Rheumatology criteria for the diagnosis of systemic lupus erythematosus
A characteristic of subacute cutaneous lupus erythematosus (SCLE) is annular or polycyclic lesions which occur in about one-third of patients. Most patients have papulosquamous lesions. SCLE is characterized by mild systemic disease, a high incidence of photosensitivity and arthralgias, anti-Ro antibodies, and an HLA A1, B8, and Dw3 serotype. The lupus band test is positive in about 50% of lesional and 30% of nonlesional skin specimens in SCLE.
References
Sontheimer RD, Thomas JR, Gilliam JN. Subacute cutaneous lupus erythematosus: a cutaneous marker for a distinct lupus erythematosus subset. Arch Dermatol. 1979;115(12):1409-1415.
Sontheimer RD. Subacute cutaneous lupus erythematosus: a decade’s perspective. Med Clin North Am. 1989;73(5):1073-1090.
Sontheimer RD. The lexicon of cutaneous lupus erythematosus--a review and personal perspective on the nomenclature and classification of the cutaneous manifestations of lupus erythematosus. Lupus. 1997;6(2):84-95.
Patel P, Werth V. Cutaneous lupus erythematosus: a review. Dermatol Clin. 2002;20(3):373-385, v.
Kuhn A, Sontheimer RD. Cutaneous lupus erythematosus: molecular and cellular basis of clinical findings. Curr Dir Autoimmun. 2008;10:119-140.
2. IgA autoantibodies producing this pattern of immunofluorescence usually react with:
b) A 97-120 kD epitope of the 180 kD glycoprotein in the lamina lucida
IgA staining in this direct immunofluorescence photomicrograph shows a linear homogenous pattern along the dermoepidermal junction, consistent with (but not restricted to) linear IgA dermatosis. Most of these autoantibodies react against the 120-kD proteolytic fragment of type XVII collagen. Others react against a 97-kD epitope of the same protein. Direct immunoelectron microscopy demonstrates that this protein is present within the lamina lucida.
References
Egan CA, Taylor TB, Meyer LJ, Petersen MJ, Zone JJ. Bullous pemphigoid sera that contain antibodies to BPAg2 also contain antibodies to LABD97 that recognize epitopes distal to the NC16A domain. J Invest Dermatol. 1999;112(2):148-152.
Zillikens D, Herzele K, Georgi M, et al. Autoantibodies in a subgroup of patients with linear IgA disease react with the NC16A domain of BP1801. J Invest Dermatol. 1999;113(6):947-953.
Metz BJ, Ruggeri SY, Hsu S, et al. Linear IgA dermatosis with IgA and IgG autoantibodies to the 180 kDa bullous pemphigoid antigen (BP180): evidence for a distinct subtype. Int J Dermatol. 2004;43(6):443-446.
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. This patient had mild arthralgias, alopecia, Raynaud disease, photosensitivity, and these skin lesions. Which one of the following is true of this disease?
a) A significant number of these patients (>20%) fail to meet American College of Rheumatology criteria for the diagnosis of systemic lupus erythematosus
A characteristic of subacute cutaneous lupus erythematosus (SCLE) is annular or polycyclic lesions which occur in about one-third of patients. Most patients have papulosquamous lesions. SCLE is characterized by mild systemic disease, a high incidence of photosensitivity and arthralgias, anti-Ro antibodies, and an HLA A1, B8, and Dw3 serotype. The lupus band test is positive in about 50% of lesional and 30% of nonlesional skin specimens in SCLE.
References
Sontheimer RD, Thomas JR, Gilliam JN. Subacute cutaneous lupus erythematosus: a cutaneous marker for a distinct lupus erythematosus subset. Arch Dermatol. 1979;115(12):1409-1415.
Sontheimer RD. Subacute cutaneous lupus erythematosus: a decade’s perspective. Med Clin North Am. 1989;73(5):1073-1090.
Sontheimer RD. The lexicon of cutaneous lupus erythematosus--a review and personal perspective on the nomenclature and classification of the cutaneous manifestations of lupus erythematosus. Lupus. 1997;6(2):84-95.
Patel P, Werth V. Cutaneous lupus erythematosus: a review. Dermatol Clin. 2002;20(3):373-385, v.
Kuhn A, Sontheimer RD. Cutaneous lupus erythematosus: molecular and cellular basis of clinical findings. Curr Dir Autoimmun. 2008;10:119-140.
2. IgA autoantibodies producing this pattern of immunofluorescence usually react with:
b) A 97-120 kD epitope of the 180 kD glycoprotein in the lamina lucida
IgA staining in this direct immunofluorescence photomicrograph shows a linear homogenous pattern along the dermoepidermal junction, consistent with (but not restricted to) linear IgA dermatosis. Most of these autoantibodies react against the 120-kD proteolytic fragment of type XVII collagen. Others react against a 97-kD epitope of the same protein. Direct immunoelectron microscopy demonstrates that this protein is present within the lamina lucida.
References
Egan CA, Taylor TB, Meyer LJ, Petersen MJ, Zone JJ. Bullous pemphigoid sera that contain antibodies to BPAg2 also contain antibodies to LABD97 that recognize epitopes distal to the NC16A domain. J Invest Dermatol. 1999;112(2):148-152.
Zillikens D, Herzele K, Georgi M, et al. Autoantibodies in a subgroup of patients with linear IgA disease react with the NC16A domain of BP1801. J Invest Dermatol. 1999;113(6):947-953.
Metz BJ, Ruggeri SY, Hsu S, et al. Linear IgA dermatosis with IgA and IgG autoantibodies to the 180 kDa bullous pemphigoid antigen (BP180): evidence for a distinct subtype. Int J Dermatol. 2004;43(6):443-446.
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.
